A 27-year-old woman reported she had been feeling sick for the past 3 weeks. She complained of loss of appetite, loss of weight, and extreme lethargy. There was no fever, no altered bowel habits, and no night sweats. There was minimal cough, with no sputum. She had a strong family history of connective tissue disease; her aunts had SLE and rheumatoid arthritis. She denied taking any medications or supplements.
Physical examination revealed generalized lymphadenopathy, hepatosplenomegaly, corneal calcium deposits, and bilateral pleural effusion. Her initial white blood cell count was 12.4x109/L, hemoglobin was 10.8 g/dl, and erythrocyte sedimentation rate (ESR) was 111 mm/h. She had severe hypercalcemia (corrected serum calcium 4.31 umol/L, serum phosphate 1.42 umol/L) with acute kidney injury (AKI) (serum creatinine 367 umol/L). Her chest radiograph showed bilateral pleural effusion, but results of investigations for infection and tuberculosis were negative. Initially, she was investigated for hyperparathyroidism, but intact parathyroid hormone level was low. Her serum calcium responded to intravenous pamidronate 60 mg, and the AKI resolved with hydration. With clinical features of generalized lymphadenopathy and hypercalcemia, a working diagnoses of lymphoma and tuberculosis were considered.
Her pleural fluid cytology was negative for malignant cells. CT scan thorax and abdomen revealed multiple enlarged mediastinal, axillary, paraaortic, iliac, and inguinal lymph nodes. Axillary lymph node biopsy was performed and reported as reactive lymphadenitis with no evidence of tuberculosis.
Because the patient had a strong family history of connective tissue disease and negative investigations for tuberculosis and lymphoma, she was also screened for connective tissue disease. She had profound hypocomplementemia, with C3 of 0.89 mg/dl and C4 of 0.09 mg/dl, with positive antinuclear antibodies (1: 640 homogenous) and anti-double stranded DNA. Her Coombs' test result was positive, suggesting autoimmune hemolytic anemia secondary to SLE. With these findings, the patient was treated for SLE with intravenous steroid. She responded very well, with improvement of her symptoms, normalization of her serum calcium, and resolution of her bilateral pleural effusion and generalized lymphadenopathy.