Case Discussion Dr. Raid Jastania

19 year old female presents with fever and generalized lymphadenopathy for one month. What are the causes of Fever? What are the causes of lymphadenopathy (lymph node enlargement)?

She has cough and mild to moderate chest pain for one week. There is no shortness of breath. The chest pain is sharp and felt the most on the lateral sides bilaterally. The pain increases with breathing but not severe to interrupt the respiration. The pain increases with coughing as well. The pain is improved with “Panadol”. What are the causes of Chest pain?

She has no vomiting, or diarrhea She has no palpitation She has no urinary symptoms She has no joints pain What is your differential diagnosis?

Examination: The patient looks ill but not in distress. Normal vital signs The systemic examination is normal except for generalized lymph node enlargement. She has generalized skin rash, maculopapular type The patient was admitted to the hospital for investigations. The ER physician considered the following possibilities: –Infection: ? pneumonia, ? TB, viral infection –Malignant neoplasm: ? Lymphoma

CBC shows anemia, leukopenia and thrombocytopenia She has formal Na, and K Urinalysis is positive for protein and RBC casts She has high urea and creatinine Chest x-ray shows bilateral pleural effusion with pleural thickening. Both lungs are normal with no consolidation. Blood culture is negative Mantoux (TB skin test) is negative Would you change your differential diagnosis?

The treating physician thinks that: –Pneumonia is unlikely since there is no consolidation –But –TB is still possible (even with negative skin test) –Lymphoma should be ruled out So he books the patient for lymph node biopsy.

What do you expect to see in the lymph node if the patient has –TB infection –Lymphoma

The lymph node biopsy shows: –Reactive lymph node with follicular hyperplasia and prominent population of plasma cells –There is no granuloma –There is no lymphoma

It is now one week already in hospital. There is no improvement of the patient condition. In fact, the patient becomes sick with deterioration of the renal function. The skin rash is more now What other things should be considered in the differential diagnosis?

Everyone thinks that an autoimmune disease is missed What investigations should be ordered now?

ANA positive Anti-DNA positive Anti-Smith positive Rheumatoid factor positive Skin and Kidney biopsies are ordered What would you expect to find in –Skin biopsy –Kidney biopsy

The skin biopsy shows: –Chronic inflammation: infiltrate of lymphocytes and plasma cells around the superficial and deep blood vessels. The inflammation is also found at the dermoepidermal junction with vacuolar change (cell injury) of the basal squamous cells. –Vasculitis: with fibrinoid necrosis of the vessels and infiltrates of neutrophils How do you explain the findings in the skin biopsy?

The kidney biopsy shows: –Focal proliferative glomerulonephritis There is chronic inflammation with infiltration of lymphocytes in the glomeruli There is focal fibrinoid necrosis of the glomeruli There is proliferation of the mesangeal and endothelial cells How do you explain the findings in the kidney biopsy?