Case presentation

case presentation of SLE with Nephritis

1. Welcome to Clinical Meeting Presented by DR. Dhiraj Chandra Biswas DR. Amlendra K. Yadav Resident (phase-A)

2. Particulars of the Patient Name : NUPUR Age : 10 years Sex : Female Address : Noaokhali Reg. no. : 550/02 Date of Admission :18/06/2014 Date of examination :18/06/2014 Informant : Mother

3. Chief Complaints Fever for 4 months. Pain over multiple Joints for same duration. Rash all over body for same duration.

4. History of Present illness According to the statement of informant mother, her child was reasonably well 4 months back, then she developed fever which was high grade, intermittent in nature, highest recorded temperature was 103 F, not associated with chills and rigors but subsided after taking antipyretics.

5. Contd…………… She also developed pain over multiple joints which first appeared both knee joint followed by both ankle, both wrist, elbow and small joints of hand and feet. Pain was non-migratory in nature, associated with morning stiffness lasting for 10- 20 minutes.

6. Contd…………… She also developed rash all over the body for last 4 months which appeared first over cheeks, nasal bridge but spared the nasolabial fold, then on both upper and lower extremities, chest and abdomen which were non pruritic in nature. On query, mother gave h/o of red color urine and painless oral ulceration.

7. Contd…………… She had no history of joint swelling, photosensitivity, hair loss, respiratory distress, abdominal discomfort, headache or convulsion. For these above mentioned complain she was treated in Dhaka sishu hospital with NSAIDs, antibiotics, hydroxychloroquine and 2 unit blood transfusion 2 months back but condition did not improved.

8. Contd…………… As the condition of child did not improve she was referred to BSMMU for further evaluation and management .

9. Birth History Antenatal History : Mother was on regular Ante natal check up. Natal history : Delivered at term at home by NVD. Postnatal History : Uneventful. Cried immediately after birth . Immunization History Immunized as per EPI schedule.

10. Milestones of Development Age appropriate. Feeding History On normal family diet .

11. Family History She is the 4 th issue of her parents. other family members are healthy . Socio-Economical History Belongs to low socio-economical background, father is a farmer, stay in kacha house and drinks tub-well water.

12. Physical Examination General Appearance : Ill-looking. Pallor : Moderately pale Edema Jaundice Cyanosis Clubbing Absent Koilonychias Dehydration Neck vein : Not engorged

13. Lymph Node :- Not enlarged Skin survey : – BCG scar mark present. Erythematous rash present over cheeks and nasal bridge sparing nasolabial fold and some blackish rash on both upper and lower extremities, chest and abdomen which are non palpable and does not blanch on pressure. Signs of meningeal irritation : – Absent . Bony tenderness : – Absent Bed side urine for albumin : Nil

14. Vital Signs Temperature – 101 o F H.R – 110 beats /min B.P – 120/90 mmHg (lies above 95 th centile) R.R – 28 breaths /min ANTHROPOMETRY : Weight - 22 kg ( 3 rd to 5 th centile) Height - 126cm ( 3 rd to 5 th centile)

15. Systemic Examination

16. Locomotor system Look: No swelling , no deformity or muscle wasting, position of the limb normal. Feel: Tenderness present (grade 2/4) over B/L knee joint ,ankle joint , elbow joint and meta- tarsophalengeal joint. Move: Movement of all joint are restricted. Other Systemic examination reveals normal

17. Gastrointestinal System Oral cavity : – Multiple oral ulceration present. Abdomen :- Soft , not distended , non-tender, umbilicus centrally placed & inverted, Liver and spleen not palpable. Shifting dullness absent. Bowel sound present.

18. RESPIRATORY SYSTEM Inspection Shape of the chest : normal R/R : 28 breaths/min Visible vein & Pulsation : absent Palpation Trachea : centrally placed Apex beat : left 5 th ICS, medial to midclavicular line Percussion note Resonant all over the lung fields Auscultation Breath sound : Vesicular Added sound : Absent Vocal resonance : Normal & symmetrical

19. CARDIOVASCULAR SYSTEM Inspection : No visible pulsation Palpation: Thrill : Absent P 2 : Not palpable Apex beat : left 5 th ICS medial to midclavicular line Lt. parasternal heave : Absent Auscultation: 1 st & 2 nd heart sounds audible in all 4 areas Murmur : Absent

20. Genito urinary system Kidney – Both kidney not ballot able renal angle – non-tender Bladder – Not palpable

21. Salient Features Nupur , 10yrs old female child 4 th issue of non-consanguineous parent got admitted with a complaints of high grade intermittent fever and pain over multiple joints with Haematuria and oral ulceration for last 4 months . She also developed non- pruritic rash over face, trunk and extremities.

22. Salient Features contd……….. For that she had treated with NSAIDs , antibiotics, hydroxychloroquine and 2 unit blood transfusion but condition did not improved. She had no history of joint swelling, photosensitivity, hair loss, respiratory distress, abdominal discomfort, headache or convulsion

23. Salient Features contd……….. O/G/E- Patient was ill llooking, febrile, moderately pale, multiple painless ulceration present . Skin survey : Erythematous rash present over cheeks and nasal bridge sparing nasolabial fold and some blackish rash on both upper and lower extremities, chest and abdomen which are non palpable and does not blanch on pressure.

24. Salient Features contd……….. Bed side urine for albumin= nil. Vitals: temperature 101 o F, She is hypertensive. Systemic examination: Locomotors system examination reveals arthalgia present. Other systemic examination- normal.

25. Provisional Diagnosis ???

26. Provisional Diagnosis Systemic Lupus Erythematous (SLE) Differential diagnosis Systemic onset JIA

27. Point in favor Point against SLE Female child Fever Arthralgia Typical rash Oral Ulcer Hematuria Hypertension SOJIA Age less than 16 years Intermittent fever Arthralgia No characteristics rash

28. INVESTIGATION

29. CBC Test Date 19/06/2014 Hb % 6.8 g/dl ESR 10 mm in 1 st hour Total count of WBC 4500 cumm Neutrophil 70% Lymphocyte 27% Platelet count 3,50,000 cumm CRP < 6 mg/dl Blood Group AB Positive CBC

30. Test Date 19/06/2014 ANA Positive Anti- ds -DNA 154.5 U/ml ( positive ) Coomb’s Test Positive C3 level 0.093 g/l ( decreased ) C4 level 0.317 g/l ( decreased ) SGPT 24 U/L S. Creatinine 0.6 mg/dl Chest X-ray Normal Findings

31. On 21/06/2014 MT 02 mm Blood culture No growth of bacteria Urine R/M/E Pus cell – (6-7)/ hpf RBC - (10-15)/ hpf Urine Culture No bacterial growth UTP 0.94 gm/day UTV 1200 ml/day HBsAG Negative PT 12.6 sec APTT 41.6 sec

32. Final diagnosis SLE with Lupus Nephritis

33. Management

34. Counseling : About the nature and future of the disease Renal Biopsy Diet

35. Drug therapy: IVF – 10% dextrose IV antibiotics ( Inj. Ceftriaxone ) Tab. Hydroxychloroquine Tab. Naproxen Tab. Ranitidine Tab. Paracetamol Tab. Calcium with Vitamin D Tab. Folic acid Tab. Captopril

36. Follow up

37. F/U On25.6.2014 Subjective Objective Assessment Plan Fever still persist Oral mucosal ulcer present Ill- looking, mildly pale, Febrile RR – 24/min HR – 96/min BP – 90/60mmhg BSUA - Nil Not improving Continue antibiotics & plan to start inj. Methylprednisolone and Inj. Amikacin

38. Subjective Objective Assessment Plan No new complain (Fever subsided) Well, allert , afebrile Vitals within normal limit BSUA – nil Improving Started oral prednisolone and cyclophosphamide F/U On 29.6.2014

39. Thank You ..