Systemic Lupus Erythematosus Presenting as Acute Adrenal Insufficiency: A Rare Clinical Presentation
A 20-year-old female unmarried regularly menstruating presented to us with a history of anorexia, progressive darkening of the face and hands for the last 3 months and 4 days history of fever and vomiting. Patient also gave a history of periorbital puffiness and multiple joint pains for the last 2 months.\nOn examination, patient had pallor, puffiness of face, hyperpigmentation over face, nose and lips []. Vital examination revealed pulse rate of 90 beats/min and blood pressure of 90/60 mm Hg supine and 80/50 mm Hg standing. Rest of the general and systemic examination was unremarkable. Investigations revealed hemoglobin of 9.3 g/dl, Leukocyte count of 2200/mm3, Platelet count of 60,000/mm3. Kidney function tests, Liver function tests, serum calcium, and electrolytes were normal. Spot urine examination revealed pus cells of 2-3/high power field, no red blood cells and casts and albumin was ++. Chest X-ray showed right mid and lower zone infiltrates., 24 h urine examination showed 1.2 g of protein per day. Blood and urine cultures were sterile.\nPatient was put on intravenous fluids and antibiotics were started empirically. Bone marrow examination performed in view of pancytopenia revealed hypercellular marrow with megaloblastoid and erythoid maturation with adequate iron stores. Antinuclear antibodies and antidouble stranded deoxyribonucleic acid (DNA) antibodies were positive. Patient was labeled as a case of SLE and renal biopsy was carried out in view of proteinuria. Histopathological examination of the biopsy tissue showed features consistent with focal lupus nephritis, International Society of Nephrology/Renal Pathology Society (ISN/RPS) class III, National Institute of Health (NIH) activity score of 9/24 and chronicity 3/12 []. Immunofluorescence showed immune deposit pattern suggestive of SLE. Echocardiography was normal. Patient continued to be in hypotension even on inotropic support and her clinical condition remained same. Based on clinical parameters, adrenal insufficiency was suspected. After taking 8 am sample for cortisol, patient was started on steroids. Serum antiadrenal antibody test showed results as 3 U/ml (normal range < 1 U/ml). Patient showed dramatic improvement in clinical parameters and hemodynamics. Morning cortisol levels were 7.5 μg/dl (normal range > 15 μg/dl). A short synecthen test (SST) using 250 mcg synecthen carried out after stopping steroids showed 30 min value of 10 μg/dl and 60 min value of 12 μg/dl. Computed tomographic scan of the abdomen was normal. Thyroid function test was normal. Antithyroid peroxidase antibody levels were normal.\nPatient was managed as a case of SLE with lupus nephritis with adrenal insufficiency and was discharged on steroids and hydroxychloroquine in a satisfactory clinical condition.