A 25-year-old recently married female presented to a tertiary care hospital in Anuradhapura, Sri Lanka, with a 3-week history of fever, malaise, cough, shortness of breath, and loss of appetite with mild hair loss for a few months. Her past medical history was unremarkable although one of her monozygotic triplet sisters had been diagnosed with SLE. On examination, she was febrile, and pale, with multiple lymphadenopathies involving the cervical, axillary, and inguinal groups along with facial puffiness and bilateral lower limb edema. Bi-basal fine end-inspiratory crepitation with hepatosplenomegaly was also noted.
Investigations revealed a low hemoglobin level and thrombocytopenia, elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and lactate dehydrogenase levels (LDH) (Table 1).
Urine analysis showed proteinuria with red blood cell (RBC) casts. The 24-h urine protein quantification confirmed nephrotic range proteinuria and the urine protein:creatinine ratio (U:PCR) was 5.76 mg/dL. Bilateral pleural effusions and a left lower zone consolidation were noted in the chest x-ray. Ultrasound abdomen confirmed hepatosplenomegaly. Blood, urine, and sputum cultures were negative. She was started on intravenous co-amoxiclav and clarithromycin and two units of leukodepleted blood were transfused slowly.
The initial ANA titer was negative. Conversely, the repeated test done 3 weeks later was found to be positive (titer:1:160), whereas simultaneous C3 (20 mg/dL) and C4 (10 mg/dL) complement levels were low (reference ranges: 50-120, 20-50 mg/dL, respectively).
Anti-double-stranded DNA, virological studies for SARS-CoV-2, human immunodeficiency virus, Epstein-Barr virus (EBV), cytomegalovirus, hepatitis B and C were all found to be negative. Sputum, Mantoux, and polymerase chain reaction testing for tuberculosis yielded negative results. There was no echocardiographic evidence of infective endocarditis.
Lymph node biopsy histology revealed reactive secondary follicles with hyalinization which were suggestive of CD. Bone marrow biopsy was negative for hematological malignancies or plasma cell disorders. Serum protein electrophoresis was negative for monoclonal gammopathy. The patient was referred to the consultant rheumatologist and was started on intravenous methylprednisolone pulse therapy (1 g per day for 3 days), oral steroid 45 mg per day, and hydroxychloroquine. She was taken over by the nephrology team for further management of renal complications of SLE. A dramatic improvement was noted clinically and biochemically with the normalization of inflammatory markers and platelet counts with the gradual but steady decline of proteinuria which has been normalized in 1-month review by the medical team (Graph 1(a) and (b)). Due to a satisfactory response to steroid therapy, renal biopsy was not opted for by the nephrology team at this point. Two months following her discharge, she was readmitted with pleurisy with pleural effusion suggestive of serositis. She was commenced on cyclophosphamide therapy as an adjunct by the pulmonologist.