LONG CASE - 19100006004

 

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CASE PRESENTATION :

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 -

1. Features of inflammation such as severe pain associated with edema of the joints and limitation of range of active movements
2.  Early morning stiffness, lasting for more than 30 mins (for 1 hour in this patient)
3. Pain and edema of joints improving with activity and worsening with rest
4. Features of uncontrolled systemic inflammation such as fever, involuntary loss of weight associated with loss of appetite.
5. 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 NO ERYTHEMA NO NO   All inspiratory findings confirmed YES NO NO NO   Slight limitation of active and passive extension and overhead abduction	Slightly flexed and internally rotated; Contour normal NO NO ERYTHEMA NO NO   All inspiratory findings confirmed YES NO NO NO   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 No No       YES SLIGHT NO NO   No restriction	Attitude - mid-flexion;  alignment of elbow and forearm - normal; NO NO erythema No No       YES NO NO NO     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   No No No No     Yes No No No   Limitation of active and passive  ulnar deviation	MILD  EXTENSION   No No No No     Yes No No No   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 NO NO     Present at MCP,PIP NO NO NO     Slight Limitation of passive flexion at MCP	Fingers flexed at MCP and PIP MILD NO ERYTHEMA NO NO     Present at MCP,PIP NO NO NO       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 NO erythema No No     Yes   No No No    Limitation of passive and active movements of flexion and extension. (towards the end of range of motion);	Slight flexed and internal rotated NO NO erythema No No   Yes No No No   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 No No     Yes No No No   No restriction	MILD NO erythema No No     Yes No No No   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) No No No     Yes Slight No No   Limitation of passive and active plantar flexion	Dorsiflexed Yes No No No     Yes Slight No No   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   No No No No     Yes at MTP,PIP NO No No   Limitation of passive movements of flexion and extension of MTP joints	Foot hanging on side of bed   No No No No     Yes at MTP ,PIP NO No No    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