19100006004 CASE PRESENTATION

LONG CASE :

CHEIF COMPLAINTS :

29/F came to OPD with C/o Loss of appetite , weight since 2 years

Generalised weakness and easy fatiguability since 2 years .

C/o Fever since 6 months

C/o Multiple joint pains and swelling since 6 months

Difficulty in movements and walking since 6 months.

C/o Hyper-pigmented leisons over cheeck bones and chin ,ears , b/l fore arm since 4 months .

C/o Severe hair loss since 4 months .

HOPI AND PAST HISTORY :

NO H/o decreased urine output or frothy urine, red color urine.

NO H/o chest pain, shortness of breath ,PND.

NO H/o bluish ,white discoloration of limbs on exposure to cold .

No h/o dry eyes and dry mouth .

FAMILY HISTORY :

No family history of joint pains/ other auto-immune diseases.

Younger sister SCD .

EDUCATIONAL STATUS :

Patient completed her BA .

MARITAL HISTORY :

Married at 15 years of age . But divorced at 20 years of age.

SOCIAL HISTORY :

Poor socio-economic status .

PROVISIONAL DIAGNOSIS :

PROBLEM REPRESENTATION :

- H/o recurrent abortions , and young onset CVA , Seizures suggests ? HYPER-COAGULABLE STATE

-29/F with recurrent history of fever and multiple joint pains , rash and hair loss , loss of appetite and weight ,fatigue - all point towards auto immune etiology ,especially in a young female .

2-3 year history of bilaterally symmetrical ,chronic progressive inflammatory polyarthritis predominantly involving small joints .

Features favouring an inflammatory pathology are -

Features of inflammation such as severe pain associated with edema of the joints and limitation of range of active movements
Early morning stiffness, lasting for more than 30 mins (for 1 hour in this patient)
Pain and edema of joints improving with activity and worsening with rest
Features of uncontrolled systemic inflammation such as fever, involuntary loss of weight associated with loss of appetite.
Swellings at joints and deformation of normal joint posture

Provisional Diagnosis - Bilaterally Symmetric Chronic Progressive Inflammatory Peripheral Polyarthritis

GENERAL EXAMINATION :

Patient was conscious, coherent and co-operative .

In Supine Position

Pulse - 94 bpm, regular, normal volume, condition of vessel wall - normal, no radio-radial or radio-femoral delay. All peripheral pulses were normal.

Blood Pressure - 110/80 mmHg - RIGHT ARM , supine posture .

110/70 mmhg -Left arm , supine posture .

Temperature - 99 F

Respiratory Rate - 18 cycles per minute , thoraco-abdominal type , no usuage of accesory muscles .

BMI - 18.3

JVP - Not elevated .

Pallor - present

No icterus ,clubbing ,cyanosis , lymphadenopathy ,edema .

Head to Toe General Examination

GENERAL CONDITION - Lean and thin built

HAIR - Thinning of hair noted on scalp . Non scarring alopecia present .

EYES - No conjunctival chemosis or injection, No redness or corneal lesions. Palpebral conjunctival pallor +. No icterus.

SKIN - Multiple, hyper-pigmented, well defined plaque/macules noted over face ,ears and fore-arm.

ORAL CAVITY - No ulcers currently

FINGERS AND NAILS - No clubbing or cyanosis , pallor -

GENERAL HEAD NECK AND ENT EXAMINATION - No abnormalities. No lymph node enlargement.

AXIAL - No apparent spinal deformities

Systemic Examination

Musculo-Skeletal System :

Axial Skeleton

Inspection - No visibly apparent spinal deformities;

Palpation - Inspectory findings confirmed. No spine tenderness.

Movements -

CERVICAL SPINE - Atlanto-occipital - Flexion, extension and lateral flexion normal .

Atlanto-axial - Rotation of head normal

FLESCHE TEST - Occiput to wall distance is zero - normal

THORACIC SPINE - Flexion , extension ,lateral bending , rotation - normal

SCHOBER'S TEST - normal

STRAIGHT LEG RAISING TEST (SLRT) - Pain present in hip joint . Not in back

SACRO-ILIAC JOINT - DIRECT PRESSURE - No pain

PATRICK'S TEST - no pain

GAENSLEN'S TEST - no pain

APPENDICULAR SKELETON :

UPPER LIMBS :

JOINTS

RIGHT

LEFT

1)SHOULDER JOINT

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

Slightly flexed and internally rotated; Contour normal

NO ERYTHEMA

All inspiratory findings confirmed

YES

Slight limitation of active and passive extension and overhead abduction

Slightly flexed and internally rotated; Contour normal

NO ERYTHEMA

All inspiratory findings confirmed

YES

Slight limitation of active and passive extension and overhead abduction

2)ELBOW JOINT

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

Attitude - mid-flexion; alignment of elbow and forearm - normal;

YES

NO erythema

YES

SLIGHT

No restriction

Attitude - mid-flexion; alignment of elbow and forearm - normal;

NO erythema

YES

No restriction

3)WRIST JOINT:

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

MILD EXTENSION

Yes

Limitation of active and passive ulnar deviation

MILD EXTENSION

Yes

Limitation of active and passive ulnar deviation

4)HANDS (small joints)

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

Fingers flexed at MCP and PIP

MILD

NO ERYTHEMA

Present at MCP,PIP

Slight Limitation of passive flexion at MCP

Fingers flexed at MCP and PIP

MILD

NO ERYTHEMA

Present at MCP,PIP

Slight Limitation of passive flexion at MCP

5)HIP JOINT :

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

Slight flexed and internal rotated

NO erythema

Yes

Limitation of passive and active movements of flexion and extension. (towards the end of range of motion);

Slight flexed and internal rotated

NO erythema

Yes

Limitation of passive and active movements of flexion and extension( towards the end of range of motion);

6) KNEE JOINT :

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

MILD

NO erythema

Yes

No restriction

MILD

NO erythema

Yes

No restriction

7) ANKLE JOINT:

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

Dorsiflexed

Yes (minimal)

Yes

Slight

Limitation of passive and active plantar flexion

Dorsiflexed

Yes

Slight

Limitation of passive and active plantar flexion

8) FEET EXAMINATION :

a) Inspection :

Attitude of limbs –

Swelling –

Skin -

Deformity –

Muscle wasting –

b)Palpation :

Tenderness –

Warmth –

Synovial thickening –

Crepitus –

c) Range of movements :

Active

Passive

Foot hanging on side of bed

Yes at MTP,PIP

Limitation of passive movements of flexion and extension of MTP joints

Foot hanging on side of bed

Yes at MTP ,PIP

Limitation of passive movements of flexion and extension of MTP joints

OTHER SYSTEM EXAMINATION :

Cardiovascular System - No abnormalities detected

Respiratory System - No abnormalities detected

Abdominal Exam - No abnormalities detected

Nervous System -NO abnormalities detected.

SCHOBERS TEST :

Examination Video links :

https://youtu.be/zJc5JiX25Xc

INVESTIGATIONS :

Serum LDH:- 212 IU/L

Random blood sugar :- 85mg/dl

COMPLETE BLOOD PICTURE :-

Hb:- 7.3%

TLC:- 6,400

Neutrophils :- 67%

Lymphocytes :- 30%

Monocytes :- 2%

Eosinophils:- 1%

PCV:-22.4%

MCV:- 80.3

MCH:- 26.2

MCHC:- 32.6

RBC:- 2.79 million/cumm

PLATELETS:- 1.16 lakhs

NC/NC - PICTURE .

DCT- POSITIVE

COMPLETE URINE EXAMINATION:-

Pus cells- 4-5

Epithelial cells :- 2-3

Albumin :- 2+

Sugars :- Nil

No RBC

Urine protein/ Creatinine ratio:-

Spot urine protein:- 10 mg/dl

Spot urine creatinine:- 171.2 mg/dl

Ratio:- 0.05

RFT :-

Urea:- 34 mg/dl

Creatinine:- 0.9 mg/dl

Uric acid :- 5.3 mg/dl

Calcium:- 10.1 mg/dl

Phosphorus :- 3.7 mg/dl

Sodium:- 139 mEq/L

Potassium:- 3.5 mEq/L

Chloride :- 98 mEq/L

LFT:-

Total Bilirubin :- 0.51 mg/dl

Direct Bilirubin:- 0.16 mg/dl

AST:- 18 IU/L

ALT:- 10 IU/L

Alkaline phosphatase:- 98 IU/L

Total proteins :- 5.6 gm/dl

Albumin :- 3.2 gm/dl

A/G ratio:- 1.17

Serology :- NEGATIVE

ULTRASOUND

Impression :-

1) Mild splenomegaly

2) Mild Ascites

3) Hemorrhagic cysts in right ovary

2D ECHO:-

Impression:-

EF:- 56%

RVSP:- 30mmhg

TRIVIAL TR+/ AR+, No MR

NO RWMA , NO AS/MS

Good LV Systolic Functions

No diastolic Dysfunctions ,

NO PAH/PE

Attachments area

Previous admission fever chart :

FINAL DIAGNOSIS :

The patient has Fever , fatigue ,hyper-pigmented rash on malar surface ,loss of appetite ,non scarring alopecia and

Bilaterally Symmetrical Chronic , Non erosive peripheral Polyarthritis, predominantly involving small joints with pain ,tenderness , early morning stiffness and swelling ,and restricted movemets .

Differential diagnosis for such conditions include - 1) SLE

2) MCTD

3) Polymyositis and dermatomyositis

4) Rheumatoid arthritis - but its erosive

5) Systemic sclerosis

In background of strong ANA positivity, anti- ds-DNA ,anti- smith , anti ro antibodies

, SLICC criteria more than 4

SLE is likely diagnosis- with skin ,joint , CNS lupus , anemia of chronic disease and thrombocytopenia.

? Associated with APLA - in view of recurrent abortion history and CVA

? Moderate Depression ( Score 18 - according to Hamilton-depression score )

TREATMENT :

1) TAB PREDNISOLONE 20 MG BD

2) TAB HCQ 200MG BD

3) TAB ACECLOFENAC 100MG BD

4) TAB DOLO 650 MG SOS

5) TAB PANTOPRAZOLE 40 MG OD

6) FUSIDIC CREAM LOCAL APPLICATION

FURTHER PLAN -

SLE is a life long disease ,and balance has to be maintained always between infections and immunosuppression .

FLARE has to be prevented .

1) Screen for APLA

2) 24 HOUR URINE PROTEIN EXCRETION - If >1g/day - Plan for renal biopsy .

3) Start patient on immunosupressants and ecosprin and anti- epileptics back

4) Improve her quality of life by reducing pain and able to get back to work and earn.

5) Psychiatric evaulation and counselling for suicidal thoughts and excessive thoughts .

6) Repeat screening of anti-ds DNA ,C3,C4 to know disease progression .

DISCUSSION :

1) SLE PATHOGENESIS ?

2) JOINT INVOLVEMENT :

3) SLICC CRITERIA :

4) ANTIBODIES IN SLE :

5) Sensitivity and specificity of ANA and anti-dsDNA in the diagnosis of systemic lupus erythematosus:

P- Serum samples from HC, MMP and SLE patients, 100 in each group, were analyzed for the presence of ANA and anti-dsDNA, by indirect immunofluorescent assay .

I - Antibody profiles assesment

C - Comparison of anti ds DNA and ANA in sera of SLE patients and sera from healthy controls and multiple medical conditions.

O - When using HC sera for the diagnosis of SLE, the sensitivity of ANA at a titer of ≥ 1:80 and ≥ 1:160 was 98% and 90%, respectively, with specificity of 92% and 96%, respectively. The specificity decreased to 88% and 94%, respectively, when using sera from MMP patients. The specificity of anti-dsDNA was 100% and 97%, when using sera from HC and MMP patients, respectively.

ANA and anti-dsDNA gave high sensitivity and high specificity in patients with SLE, even when using MMP patient's sera as controls.

https://pubmed.ncbi.nlm.nih.gov/24383972/

6) Efficacy of cognitive behavioural therapy for the treatment of chronic stress in patients with lupus erythematosus: a randomized controlled trial

P - 45 patients with lupus and high levels of daily stress were randomly assigned to a control group (CG) or a therapy group (TG)

I - Received cognitive behavioural therapy (CBT) which consisted of ten consecutive weekly sessions.

C - Control sle patients

O - We found a significant reduction in the level of depression, anxiety and daily stress in the TG compared to the CG and a significant improvement in QOL and somatic symptoms in the TG throughout the entire follow-up period.

7)Thrombocytopenia in SLE ?

Is associated with a worse prognosis and higher mortality from the disease.12,16,17 It has been linked with a severe disease course including neuropsychiatric disorders, renal involvement, hemolytic anemia and antiphospholipid syndrome.
The leading cause of death in the Reveille et al.20 study was infection.
In two large studies thrombocytopenia was the only independent predictor for mortality in SLE.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7045921/#:~:text=Systemic%20Lupus%20Erythematosus%20(SLE)%20is,with%20worse%20prognosis%20in%20SLE.

8)Differentiating infection vs lupus flare ?

In our recent study on Korean patients with lupus, CRP levels higher than 1.35 mg/dL indicated the presence of an infection with 100% sensitivity and 90% specificity

. Leukocyte count is elevated in SLE with infection, and remains unchanged or decreased in SLE with flare

Complement levels and anti-dsDNA antibody titer are changed only in SLE with flare, but the changes are usually not considerable at already abnormal levels.

Overall, CRP is the most critical marker in differentiating between infection and disease flare up in SLE. In a recent study, fever duration, anti-dsDNA antibody titers, and CRP were regarded as the most reliable markers to distinguish between infection and SLE activity

Jung JY, Suh CH. Infection in systemic lupus erythematosus, similarities, and differences with lupus flare. Korean J Intern Med. 2017;32(3):429-438. doi:10.3904/kjim.2016.234

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SHORT CASE 1:

19 year old male resident of Nalgonda and currently studying intermediate ,came to opd with complaints of :

-Itchy Ring leisons over arms ,abdomen ,thigh and groin since 1 and half year .

-Purple stretch marks all over abdomen ,lower back ,upper limbs ,thighs since 1 year .

-Abdominal distension and facial puffiness since 6 months.

- Pedal edema since 3 months.

- Low back ache since 3 months .

- Feeling low , not feeling to talk to anyone.

- Weight gain and decreased libido since 3months.

- Loss of libido and erectile dysfunction since 2 months .

Pt was apparently alright one and half year ago , when he slowly developed erythematous round leisons which are annular shaped and itchy all over abdomen , upper limb ,groin and inner thigh region .

No history of fever back then. No other complaints apart from skin lesions.

Pt visited local RMP where he prescribed auyurvedic medications and other creams ( unknown composition as pt don't have them currently ). He also prescribed tablets (unknown composition) . Patient started using all these medications for 1-2 months .

Leisons reduced a bit after using medications .

Later after 2 months he developed multiple hyper pigmented plaques over lower limbs ,abdomen , for which he again visited same place and used ayurvedic oils over the leisons.

He also used clobetasol ointment over the leisons.(for approximately 1 year all over the body)

He started noticing pink striae over his abdomen first 1 year ago and later on back and over arms,which were gradually increasing in size .

Later he visited a hospital and used miconazole and luliconazole ointments also.

He used clobetasol ointment all over the leisons for long time .

He started noticing abdominal distension and facial puffiness ,weight gain, but never visited any hospital.

Later he developed pedal edema and low back ache since 3 months .

His consulted a dermatologist at this point of time who advised to consult physician and prescribed monteleukast , itraconazole tablets ,luliconazole ointment for tenia corporis.

He stopped all medications one month ago and visited our opd with complaints of pink striae and easy fatigue ,weakness and low back ache .

His brother also gave history of pt being moody and feeling of low self esteem due to multiple leisons.

He even complaints pt wouldn't step out of house and always stays indoor and wouldn't interact with others .

No complaints of chest pain ,sob , palpitations .

No complaints of decreased or frothy urine.

No other negative history.

No h/o DM,HTN,TB,ASTHMA,CAD.

ALLERGIC HISTORY - pt gives h/o allergy to eggs ,brinjal .

O/E : Pt was c/c/c

BP - 160/100 mmHg

Pr - 96 BPM ,regular ,normovolemic .

Rr - 18/min

Spo2- 98% on ra.

Weight - 63 kg.

Height - 175 cm.

GENERAL EXAMINATION :

NO pallor ,icterus ,cyanosis , clubbing, lymphadenopathy.

Pedal edema present - pitting type extending upto knee.

Abdominal distension present.

Moon face present

Pink striae noted over anterior abdominal wall and on low back and on upper arms and thighs.

Thin skin present .

Poor healing noticed over leg ulcers and easy bruising noted .

Acne present over face .

Acanthosis nigrans noted over neck.

GYNECOMASTIA PRESENT .

neck pad of fat present .

Sparse scalp hair .

Skin examination - Multiple itchy erythematous annular leisons noted all over abdomen , upper limb ,groin and inner thigh region .

Multiple hyperpigmented plaques noted over bilateral lower limbs .

SYSTEMIC EXAMINATION :

CVS - S1S2 heard .No murmurs

RS - BAE present .

No adventitious sounds .

P/A - Soft , distended .

No organomegaly .

Bowel sounds present .

CNS - HMF - INTACT R. L

MOTOR SYSTEM - POWER - UL 5/5 5/5

LL 5/5 5/5

Proximal muscles lower limb - power is 4/5 .

TONE - NORMAL.

REFLEXES - B. T. S. K. A. P

R. +2 +2. +1. +2. +1. FELXOR

L. +2. +2. +2. +2. +1. FLEXOR.

CRANIAL NERVES - NORMAL .

Difficulty in getting up from chair was noticed.

PROVISIONAL DIAGNOSIS -

? IATROGENIC CUSHINGS SYNDROME .

TINEA CORPORIS .

DENOVO HTN .

INVESTIGATIONS :

CBP - HB - 13.4 g/dl

TLC - 6,800

PLT - 1.5 lakhs.

RBS - 139 mg/dl

CUE - ALBUMIN - +1

SUGARS - NIL .

PUS CELLS - 3-4

RBC - NIL .

LFT - TB -1.03

DB-0.21

ALBUMIN - 3.9

RFT - UREA - 22

SERUM CREATININE -0.6

ELECTROLYTES - NA - 136

K- 4

CL-98

USG ABDOMEN - NORMAL.

ECG - SINUS TACHYCARDIA

LVH PRESENT.

This was picture of striae one year ago when it gradually started :

On presentation to opd pictures : 28/05/21

We took dermatologist opinion for tenia corporis where they advised

Ointment AMLORFINE

FUSIDIC ACID CREAM.

SALINE COMPRESS OVER LEISONS.

Plan to start anti fungals on next visit once dose of steroids is reduced .

OPTHAL opinion Was taken to look for visual acuity and cataract .

No features of lens opacities noted .

BUT IOP was high ,where they advised to follow up .

We advised pt to get fasting 8am serum cortisol levels and was planned to start on low dose steroids to avoid adrenal crisis.

8AM S CORTISOL LEVELS (30/5/21)

- 0.46 mcg/dl ( very low) .

( normal range - 4.3-22.4 mcg/dl).

In view of lvh pt was started on tab telma 20 mg od .

On 3/6/21 - ACTH STIMULATION TEST WAS DONE .

BY INJECTING 0.4 ML OF ACTOM PROLONGATUM INJECTION (ACTH) INTRA MUSCULAR @ 7am

1 HR LATER FASTING SERUM CORTISOL SAMPLE WAS SENT .

VALUE - 0.73 mcg/dl

Indicating there was HPA AXIS suppression and pt was started on TAB HIZONE 15 mg per day in three divided doses @ 8am ,12 pm and 4 pm.

Pt was asked to follow up after one month .

FINAL DIAGNOSIS :

IATROGENIC CUSHINGS SYNDROME SECONDARY TO TOPICAL CLOBETASOL APPLICATION ALL OVER BODY FOR APPROXIMATELY ONE YEAR.

TINEA CORPORIS

DENOVO HTN .

FOLLOW UP -

ON NEXT VISIT : ( 25/6/21).

Pt was symptomatically better , pedal edema subsided.

Striae were pale in color and we're subsiding.

Weight - 67kg

Ht -175 cm.

Bp- 160/100 mmHg.

Pr -88bpm.

Dose of Tab hizone was reduced to 10 mg per day in divided doses for one month.

In view of low back ache Xray LS spine was done which was normal and pt was advised.:

Tab Shelcal 500 OD and Tab Vit D 3 Od.

Tab ULTRACET /PO/SOS.

Psychiatry opinion was taken and he was diagnosed with moderate depression .

ON JULY 5 - Patient came for follow up in dermatology opd ,

IMPROVEMENT SEEN . PT COMPLAINING OF NEW RING LEISONS OVER LEGS . PUS DISCHARGE NOTED OVER ABDOMINAL STRIAE WITH ERYTHEMATIZATION .

THEY ADVISED ,

- TRETIN GEL 0.05 % L/A OD X 2 weeks

- TAB ATARAX 10MG OD

-AMOROLFINE CREAM L/A BD X 5 DAYS

-CAP AMOXICLAV 625 MG BD X 5 DAYS

FUSIDIC CREAM L/A BD X 5 DAYS.

PLAN - TO START ANTIFUNGALS ONCE HIS TOTAL DOSAGE OF HISONE IS 5 MG/DAY.

In July 2021 pt was complaining of fever ,sore throat and dry cough since 3 days and he was tested positive for COVID 19 , we advised him home isolation and PCM 650 Mg /po /tid x 5 days .

He was advised to continue tab hizone tablets as it was advised. ( 10mg/day in divided doses.)

-INJ HYDROCORTISONE 100mg sos if pt is in adrenal shock.

He recovered from COVID within one week .

Next visit : ( 6/8/21).

BP- 170/100 - TELMA DOSE WAS INCREASED TO 40 MG OD.

PR - 88bpm.regular , normovolemic.

Wt- 69 kg

Height -

Abdominal girth - 96cm

Pt complaints of excoriation over striae and appearance of erythematous macules over abdomen whenever he takes food he is allergic to.

Took dermatologist opinion for it . They started him on Tab Itraconazole 100 mg bd for 2 weeks. And lulifin cream and tab levocitrixine 5mg od.

His brother complaints of depressed mood , pt not going out due to social stigma. Psychiatric counselling was given .

He still complaints of low back ache..othropedics opinion was taken and advised to continue Ultracet and tab Shelcal .

Cbp , cue and electoltes were repeated which were all in normal range .

USG ABDOMEN was done - Normal kidney size bilateral and CMD maintained. No other sonological abnormality noted.

As his lesions dint subside we reduced dose of hisone to 7.5 mg per day ,to see response.

Review psychiatry and opthal opinion was taken , where his lens was clear - no e/o cataract .

no retinopathy changes and no raised IOP and his visual acuity was 6/6.

0N 20/8/21 - IN view of constant low back ache , MRI LS SPINE WAS done with whole soine screening .

which showed lumbar epidural lipomatosis .

Mild depression of superior endplates of D12 and L4 - Likely subacute /chronic compression .

L3-L4 disc degeneration with mild diffuse disc bulge causing no significant neural foramen stenosis .

ON 24/8/21 - Pt developed painful swelling and redness of right lower limb along with intermittent fever - DIAGNOSED TO HAVE RIGHT LOWE LIMB CELLULITIS .

Pt was admitted and was given IV AUGMENTIN for 5 days and MGSO4 dressings were done .

TAB chymerol forte was given for 5 days.

Tab hisone 7.5 mg was continued in divided doses .

-INJ HYDROCORTISONE 100mg sos if pt is in adrenal shock.

PT again came for follow up in september , complaints of weight gain , but striae reduced and patient attender was giving history of patient having low mood and constantly being at home and not doing any exercise .

He was having social inhibition and was avoiding contact with other people.

We sent him for psychiatry where brief counselling was given .

TAB HISONE dose was reduced to 5 mg /day.

Next follow up was in october (22/10/11) ,pt improved and dose was further reduced to 2.5 mg/day .

PT again visited to our OPD on 23/ 11/21 , again with

c/o increasing striae and abdominal distension.since one month .( Striae were same as before ,but pt was feeling that way )

Abdominal distension could be due to fat accumulation,as pt was hardly exercising and is only staying at home and eating food.

weight gain present .( 76kg ).

c/o diminished vision

BP-160/100 mmhg

PR - 110 bpm

CVS- S1 S2 PRESENT

RS - BAE present and clear .

Pt was giving history of good compliance to medications and was using tab hisone 2.5mg only since past one month .No history of other medications ( ayurvedic or homeopathic ) usuage .

He is still having social inhibiton and is not going out of house or doing regular exercise .

P is having lot of anxiety and psychiatric issues.

As his blood pressure is uncontrolled and ECG having LVH - we increased the dose of anti-htn to TAB TELMA AM 40/5 mg OD.

HIS ROUTINE investigations were repeated , cbp,s. electrolytes ,s.creat was normal .

HIS RBS was 178 mg/dl

HBA1C was 6.5 %

Review Dermat opinion was taken ,

PT dint use his cream and itraconazole tablets regularly last month .

Review psychiatry opinion taken - Adjustment issues due to underlying illness.

Brief psychotherapy given .

REVIEW ENDOCRINE OPINION TAKEN (26/11/21) : Pt apprehensive .

Stopped hisone 2.5 mg and observe patient.

Only stress dose - Inj hydrocortisone 100 mg IV IF PT IS IN Adrenal shock .

Adv -8am serum cortsiol

Inspite of using the minimal dose of tapering steroids , pt has all these complaints , so a diagnostic uncertainity was prevailing .

1) COULD THIS PT HAVE UNDERLYING ENDOGENOUS CUSHINGS ?

A) In this patient ,it is not possible as his basal cortisol and post ACTH stimulation ,his serum cortisol levels were very low .(so ruling out possibility of endogenous cushings).

-( It is very rare to have this co-occurence of endogenous and exogenous cushings syndrome , although one such case was reported from malasiya .

But in this patient ,her basacl cortisol levels were not available/done .So hence they missed diagnosis .

We present a case of a 66-year-old lady who was noted to have typical features of Cushing's syndrome. As she gave a very clear history of ingesting exogenous GC for a year, no further work up was undertaken. Despite cessation of GC for a year, she continued to have thin skin and easy bruising. Upon admission for hypertensive emergency, her clinician took note of her changes and investigated her for endogenous Cushing's syndrome. Her cortisol post overnight dexamethasone suppression test was 707 nmol/l. Post low dose dexamethasone suppression test yielded a cortisol of 1133.2 nmol/l. 24 hours urine cortisol was 432.2 nmol/l. Plasma ACTH was 1.1 pmol/l, indicating an ACTH independent Cushing's syndrome. We proceeded with Computed tomography scan (CT scan) of adrenals which revealed a right adrenal adenoma measuring 4.4 × 3.4 × 4.0 cm. Right retroperitoneoscopic adrenalectomy was done. Histopathology examination was consistent with adrenal cortical adenoma with foci of myelolipoma. Post adrenalectomy she developed hypocorticolism secondary to contralateral adrenal suppression which lasted up to the present date. Her cutaneous and musculoskeletal manifestations improved substantially. Co-occurrence of endogenous and exogenous Cushing's syndromes is uncommon but should be considered in patients whose Cushingoid features do not resolve after cessation of exogenous GC. )

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6815529/

2) OR is patient using other steroids/ ayurvedic/homeo medications ,which he is concealing the history.

is he doing it for medical attention ?

is he using the medications as prescribed or using double doses ?? although pt and his attender denies any such history .

all these questions remain un-answerwed .

Current plan - to admit the patient and stop tab hisone 2.5 mg and monitor him for any adrenal crisis.

fasting 8 am serum cortisol to be sent again .

Hisone 2.5 mg tablet was stopped for 2 days. No symptoms as of now.

26/11/21 - Repeat 8am fasting serum cortisol is 0.54 mcg/dl

ACTH stimulation test was done . Report awaited .

1/12/21 -Pt was observed for one week and had no episodes of hypotension .

So after taking patient and his attenders consent ,and explaining them about condition and course and prognosis of disease , tapering dose of steroids was stopped .

Pt was explained to take stress dose of steroid INJ HYDROCORTISONE 100mg IV STAT ,in case of fever,acute illnesses, syncope and hypotension .

Pt again followed up with us in December . Had no complaints and was still having low mood and was not having any physical activity .

His vitals were stable . Good appetite .

On 21/1/22 -

Pt visited our hospital again with complaints of giddiness and vomiitings since 3 days and loss of appetite since 1 week .

His sbp on presentation was 40 mmHg and ot was rushed to casualty and immediately IV HYDROCORTISONE 100 mg was given and fluid resuscitation was done . Following which his blood pressure improved .

He was admitted into ICU and intense monitoring was done along with IV HYDROCORTISONE 100 MG 6 th hourly for 2 days. Plan to shift to oral hydrocortisone 15 mg later and send 8 am Serum cortisol levels and ACTH stimulation test to be done .

He started of with Exogenous cushings syndrome, but a more pressing problem right now is HPA -axis suppression and secondary adrenal insufficiency .

Inspite of using the minimal dose of tapering steroids , his HPA- Axis wasn’t improving .

Possible reasons could be :

1)Obesity

2)Concomitant usage of Anti-fungal drugs ,which can reduce metabolism of exogenous steroid (Tab.Hisone) by inhibiting CYP 3A 4 . So this causes prolonged HPA-Axis suppression and thereby reduced serum cortisol levels.

3)Is Patient using other steroids/ ayurvedic/homeo medications ,without our knowledge and is concealing the history. Is he doing it for medical attention ?

4) Is he using the medications as prescribed or using double doses ?? although pt and his attender denies any such history .

All these questions remain un-answered .

DISCUSSION :

Sequence of images of our current patient :

MAY -2022 :

NO complaints. Weight reduced .

CUSHING'S SYNDROME :

CLASSIFICATION OF CUSHINGS SYNDROME :

APPROACH TO A CASE OF CUSHINGS SYNDROME :

NIEMAN LK,BILLER BM et al. THE DIAGNOSIS OF CUSHINGS SYNDROME,J CLIN ENDOCRINE METABOLISM.2008;93:1526-1540.

POST –LDDST SERUM CORTISOL VALUE >1.8 mcg/dl – SUGGESTS CUSHINGS SYNDROME.

Source : WILLIAM TEXTBOOK OF ENDOCRINOLOGY 14TH EDITION .

MANAGEMENT OF EXOGENOUS CUSHINGS SYNDROME :

Source : Hopkins, Rachel L.; Leinung, Matthew C. (2005). Exogenous Cushing's Syndrome and Glucocorticoid Withdrawal. , 34(2), 371–384.

MANAGEMENT OF ENDOGENOUS CUSHING'S SYNDROME:

Medical management:

R.PIVONELLO et al-Treatment of cushings disease. Endocrine Reviews, Volume 36, Issue 4, 10 August 2015

Supporting evidences :

CASE REPORT : Janiel Pimentel; Chirag Kapadia et al-ADRENAL SUPPRESSION SECONDARY TO INTERACTION OF COMBINED INHALED CORTICOSTEROID AND ANTIFUNGAL AGENT .AACE CLINICAL CASE REPORTS Vol 4 No. 4 July/August 2018 .

Inhaled corticosteroids (ICS) are exogenous glucocorticoids that typically have minimal systemic effects at standard doses. They are metabolized by the cytochrome P450 (CYP450) enzyme pathway in the liver.

Posaconazole, is a known CYP450 inhibitor.

P- 9-year-old Caucasian female with history of hyper IgE syndrome. On chronic fluticasone and posaconazole for Aspergillus infection, she presented with fatigue and “facial puffiness” (cushingoid features).

8AM cortisol was (0.3 μg/dL) and Post (ACTH)- stimulation test cortisol level < 1 μg/dl.

I- Started on physiologic dose of hydrocortisone (10 mg/day) and fluticasone and antifungals discontinued. Hydrocortisone supplementation was slowly weaned over a 7-month period.

O- After weaning hydrocortisone, repeat low-dose ACTH-stimulation test with peak cortisol of 21.8 μg/dL, consistent with resolution of AS.

2) Skov, K.M main et al -Iatrogenic adrenal insufficiency as a side-effect of combined treatment of Itraconazole and Budesonide. European Respiratory Journal 2002 20: 127-133;

P-

CASES -37 cystic fibrosis patients with ABPA , out of which 25 CF patients treated with both itraconazole and budesonide, and in 12 patients treated with itraconazole alone .

Controls -30 cystic fibrosis patients.

I – ACTH –stimulation test was done in both cases and controls and cortisol levels were measured .

C- Eleven of the 25 patients treated with both itraconazole and budesonide had adrenal insufficiency. None of the patients on itraconazole therapy alone nor the control CF patients had a pathological ACTH test. Mineralocorticoid and gonadal insufficiency was not observed in any of the patients.

These patients improved but had not achieved normalized adrenal function 2–10 months after itraconazole treatment had been discontinued.

Tempark T, Wananukul S et al - Exogenous Cushing's syndrome due to topical corticosteroid application: case report and review literature. Endocrine. 2010 Dec;38(3):328-34

Prolonged use of topical corticosteroids causes systemic adverse effects including Cushing's syndrome and hypothalamic-pituitary-adrenal (HPA) axis suppression, which is less common than that of the oral or parenteral route.

P- 43 cases with iatrogenic Cushing syndrome from very potent topical steroid usage (Clobetasol) in children and adult have been studied.

In children group (n = 22), most are infants with diaper dermatitis. For the adult group(n = 21), the most common purpose of steroid use was for treatment of Psoriasis.

The recovery period of HPA axis suppression was 3.49 ± 2.92 and 3.84 ± 2.51 months in children and adult, respectively.

4) Varshney I, Amin SS, Adil M, Mohtashim M, Bansal R, Khan HQ. Topical Coticosteroid Abuse- Risk factors and Consequences. JDA Indian Journal of Clinical Dermtology. 2019;2:72-77.

Topical corticosteroids are one of the most widely prescribed class of topical drugs. They have been abused in developing countries as they provide rapid symptomatic relief in inflammatory dermatoses.

Aim: This study was done to find the risk factors related to topical corticosteroids abuse and consequences thereof.

Methods : A hospital-based cross-sectional study was conducted in the dermatology OPD from July 2017 to June 2018.

P - 2032 patients abusing topical steroids, 1365 (67.2%) patients were recorded with adverse effects of topical corticosteroids.

The majority (60.78%) were from urban areas. 20.9% were abusing topical steroids for more than 12 months

O- The most common abused steroid formulation was mixed combination consisting of steroids, anti-fungals and anti-bacterials (47.9%).

Fungal infection (59.5%) and acne (15.3%) were the most common indications of steroid abuse. Quacks (31.9%) and pharmacists (26.8%) were the most common prescribers of these topicals .

Most common adverse effects of topical corticosteroid abuse were tinea incognito (41.1%), steroid-induced acne/ aggravation of acne vulgaris (18.2%) and telangiectasia (14.1%).

Most common source of abuse was non-prescriptional and it was mainly due to easy availability of mixed combinations of steroid containing creams and lack of public education regarding its adverse effects.

So final take home message is STOP steroid abuse and public health education and awareness regarding the lethal effects of usuage of non -prescriptional medications and long term usuage of topical applications .

Only awarness would save one's life .

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SHORT CASE 2:

65 year old male patient r/o Nakrekal presented to casualty with complaints of

C/O Shortness of breath grade 3 since 4 months .

C/O B/L pedal edema since 4 months .

C/O abdominal distension since 5 days

C/O Oliguria since 2 days

HISTORY OF PRESENTING ILLNESS :

Patient is toddy climber by occupation , was apparently asymptomatic 4 months ago .

-Intially he noticed b/l swelling of lower limbs , gradual onset and progressive . Pitting type and extending upto knees .

Associated with shortness of breath grade 2 , progressed to grade 3 over 4 months .

H/O orthopnea and PND present .

No h/o chest pain , palpitations

In view of sob , patient visited local hospital and was told ,he had a stone in one of his kidneys and both his kidneys failed .

He was advised maintainace hemodialysis,but patient denied and was discharged on medications .

His pedal edema subsided after using medications .

He continued taking medications , but noticed loss of appetite, weight , fatigue and generalized weakness .

His urine output was adequate previously . H/o hematuria present occasionally

No h/o pus in urine , burning micturition , frothy urine .

As he had generalized fatigue ,loss of appetite and ,sob ,elevated urea and s. creatinine he visited our hospital and was initiated on hemodialysis by placing central venous catheter in right internal jugular vein .

Patient had 4 sessions of hemodialysis .

He went to Hyderabad and got A-V fistula on his left hand .

C/o Low back ache and body pains .

C/O abdominal distension since 5 days , sudden onset and progressed gradually . Associated with increased sob on lying down and abdominal tightness.

Pedal edema is mild extending upto ankle joint.

No h/o yellowish discoloration of eyes . No h/o binge alcohol intake .

Past history - K/c/o HTN since 10 years and is not on regular medication .

NOT a k/c/o DM, TB , ASTHMA,CAD , EPILEPSY,CVA .

No surgical history and past Medical history

No h/ o NSAID abuse .

Personal history - Regular bowel and bladder movements

Adequate sleep

Loss of appetite present

Mixed diet

Social & Educational History :

Married for 27 years with 2 children. Not educated

Family history - Not significant

Addictions - Toddy drinker occasionally -3 times /week . 90 ml

Non -Smoker

PROVISIONAL DIAGNOSIS :

65 year old male with acute history of oliguria and abdominal distension ,on a background of Sob and pedal edema and HTN

?Acute decompensated Heart failure in view of anasarca and orthopnea and PND .

? Renal failure in view of decreased urine output and Anasarca and h/o renal calculi and HTN

General examination :

Pt C/C/C

Pallor - present

No icterus , clubbing, cyanosis,koilonychia , lymphadenopathy

B/L pedal edema - pitting type present. extending upto ankle .

Jvp - couldn't be assessed due to central line .

Skin - Dry ,scaly , itching present .

Eyes - Grade 2 HTN retinopathy changes noted on fundoscopy .

Vitals :

Bp - 140/90 mmhg - Right arm supine posture

Pulse - 130 bpm ,regular ,normal volume, condition of vessel wall - normal, no radio-radial or radio-femoral delay.

Resp rate - 26/ min

Spo2 - 97% on RA

Grbs - 110 mg/dl

Temp -99 F

SYSTEMIC EXAMINATION :

GIT EXAMINATION :

INSPECTION :

Shape of abdomen - Distended-uniform

Flanks – Full

Umbilicus – Everted

Skin – Stretched, shiny

No scars, sinuses, striae, nodules , discoloration.

Dilated veins – on front present

Movements of the abdominal wall - All quadrants equally moving with respiration .

Abdomino - Thoracic type of breathing

NO visible intestinal peristalsis

Hernial Orifices normal

Cough impulse - Negative

External genitalia - Scrotal swelling present

PALPATION :

Measurements - Abdominal Girth - 108 cm

Flanks - full

Superficial Palpation – Tenderness present in epigastrium

No local rise of temperature

Direction of Blood Flow in Veins - away from umbilicus

Deep Palpation :

Liver Span - Couldn't be palpalted due to gross distension .

Spleen - Couldn't be palpalted due to gross distension

Kidney - Couldn't be palpalted due to gross distension

Any other Palpable swelling - No

Hernial Orifices - normal

Murphy’s Punch/Renal angle tenderness - no tenderness

External Genitalia - scrotal edema present . non tender and trans -luminant

PERCUSSION:

Fluid Thrill - Present

Shifting dullness - Present

AUSCULTATION:

Bowel sounds – Present

Aortic, Renal Bruit - Absent

CARDIOVASCULAR EXAMINATION :

INSPECTION:

Chest wall shape - Ellipsoid and b/l symmetrical

No Precordial bulge, Pectus carinatum/excavatum

No Kyphoscoliosis

No Dilated veins, scars, sinuses

Apical impulse - Visible in left 5 ICS 1 cm lateral to MCL .

Pulsations – epigastric, parasternal - absent

PALPATION:

Apical impulse – Tapping type , felt in left 5 ICS 1 cm lateral to MCL .

Pulsations – No Epigastric pulsations

Parasternal Heave – Present - Grade 2

No Thrills and palpable heart sounds .

Auscultation :

S1 S2 heard in Aortic , pulmonary,tricuspid and mitral areas .

No added sounds

No murmurs

Respiratory system -B/L NVBS

B/L fine crepitations present IAA ,ISA .

CNS - NO abnormality detected .

INVESTIGATIONS :

BGT:- A positive

Serum iron :- 83

CBP :

HB - 7 g/dl

TLC - 12,400 cells /mm3

Platelets -1.3 lakhs .

RFT:-

Urea :- 92 mg/dl

Creatinine :- 5.7 mg/dl

Uric acid :- 6.6 mg/dl

Calcium :- 9.6 mg/dl

Phosphorus :- 3.4 mg/dl

Sodium :- 132 mEq /L

Potassium :- 3.7 mEq/L

Chloride :- 98 mEq/L

RBS:- 79mg/dl

LFT:-

Total bilirubin :- 2.12 mg/dl

Direct bilirubin:- 0.64 mg/dl

AST :- 17 IU/L

ALT :- 10 IU/L

Alkaline phosphatase:- 203 IU/L

Total proteins :-6.1 gm/dl

Albumin:- 2.2 gm/dl

A/G ratio:- 0.56

USG SCANNING OF WHOLE ABDOMEN:-

Impression:-

1) Bilateral GRADE 3 RENAL PARENCHYMAL CHANGES

2) MULTIPLE LARGE LEFT RENAL CALCULI .

3) Moderate to Gross ascitis

COMPLETE URINE EXAMINATION:-

Colour:- pale yellow

Appearance :- slightly hazy

Specific gravity:- 1.010

PH:- Acidic (6.0)

Proteins:- +++

Glucose :- Nil

Urobilinogen:- Negative

Bilirubin:- Negative

Ketones:- Negative

Nitrates :- Negative

Pus cells:- 5-6 /HPF

Rbc:- 5-7 /HPF

Epithelial cells:- 1-2/HPF

Casts:- Granular casts present

Crystals :- Nil

2D echo :-

Impression -

EF:- 48%

Dilated LA

Conc LVH

Moderate MR/ Mild AR / Mild TR

No PAH/ No PE/ No CLOT

No RWMA

Sclerotic AV .

CT KUB : showing left kidney 7-8 cm Staghorn calculus causing mild hydronephrosis and thining of parenchyma

Ascitic fluid analysis showed - HIGH SAAG

Examination videos links :

https://youtu.be/FDq1rKdkIzQ

https://youtu.be/SXvrJfDQzig

https://youtu.be/p_W68yJ8Yu8

Final diagnosis -

Ascitis secondary to portal hypertension - ? Post hepatic -

Secondary to Congestive cardiac failure (HFPEF)

Post renal AKI on CKD - Left staghorn calculus

CKD - Stage 5 - Native kidney disease - ? Htn nephropathy

Cardio- renal syndrome type 4

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CRITICAL APPRAISAL:

Dapagliflozin in Patients with Heart Failure and Reduced Ejection Fraction .

RCT - placebo control trial .

https://www.nejm.org/doi/full/10.1056/NEJMoa1911303.

P- Total 4744 patients with HFref .

2373- Dapagliflozin 10mg od .

2371 - placebo

I- Dapagliflozin vs placebo

C- Placebo

O - The primary outcome was a composite of worsening heart failure (hospitalization or an urgent visit resulting in intravenous therapy for heart failure) or cardiovascular death.

1) 237 - In dapaglifozin grp had worsening heart failure and 227- Died.

2) 326- placebo had worsening HF

273- DIED .

CONCLUSION :

Among patients with heart failure and a reduced ejection fraction, the risk of worsening heart failure or death from cardiovascular causes was lower among those who received dapagliflozin than among those who received placebo, regardless of the presence or absence of diabetes.

But pts who were included , dapagliflozin had more patients with NYHA class 2 and placebo had more pts with NYHA class 3,4 HF . Which can explain frequent hospital admissions in them .

More larger data is required and they dint mention about what happened to other remaining patients .

And also all the patients received ARNI/ARB and beta blockers along with SGLT2 OR Placebo.

So individual benefits couldn't be estimated .

But according to numbers they showed ,lesser hosp admissions and mortality was there for SLGT-2 group .

LIMITATIONS :

This trial has some limitations. We used specific inclusion and exclusion criteria, which may have limited the generalizability of our findings.

The baseline use of sacubitril–valsartan, which is more effective than renin–angiotensin system blockade alone at reducing the incidence of hospitalization for heart failure and death from cardiovascular causes, was low.

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