Primary adrenal insufficiency in case of antiphospholipid syndrome
A 44-year-old lady presented with hemoptysis, cough and shortness of breath. She had a 3-month history of progressive darkening of skin along with weakness, anorexia, nausea and weight loss of 3 kg over 1 month. She had a past history of a first trimester abortion. History of deep vein thrombosis, pulmonary embolism, connective tissue disorder was ruled out. Her body mass index was 18, goiter was absent, she had tachycardia (pulse rate 110/min) and blood pressure was 108/76 mm Hg with postural drop of 26. Pallor and skin and mucosal pigmentation were present.\nOn investigations: Hb 11.6 g%, total cholesterol 10,100 (N 72%, L 23%, E 5%), erythrocyte sedimentation rate:62 (1st h), platelets 86,000, urine R/E normal, Cr 1.1 mg/dl, liver function tests normal, echo normal.\nChest X-ray showed right middle zone opacity. Computed tomography (CT) scan of lungs revealed subpleural consolidation in mid and lower zones of the right lung with ground glass opacity and areas of hemorrhage and filling defect in right descending pulmonary artery. CT pulmonary angiography confirmed right descending pulmonary artery embolism. Duplex scan revealed right superficial femoral vein thrombosis. Renal and adrenal veins were normal in Duplex scan and magnetic resonance angiography (MRA).\nProthrombin time was 13.8 (normal [N] 11.2-14.2), activated partial thromboplastin time 142 s (N 31-42), fibrin/fibrinogen degradation products was raised, lupus anticoagulant and anticardiolipin antibody were markedly positive. Anti-nuclear antibodies, double-stranded deoxyribonucleic acid, anti-neutrophil cytoplasmic antibody and rheumatoid factor were negative. Human immunodeficiency virus and hepatitis serology was negative. Sputum and blood for Bactec culture for acid-fast bacteria and fungus was negative.\nBaseline 8 A.M. cortisol was 2.3 mcg/dL with an adrenocorticotropic hormone (ACTH) of 306 pg/ml. 250 mcg ACTH stimulation test showed a 1-h cortisol level of 2.5 mcg/dL. Thyroid function test was normal, but thyroid peroxidase antibody was 590 mU/ml (normal <35). Follicle-stimulating hormone was 7 mIU/ml CT of adrenals was normal.\nBased on clinical features and investigations our diagnosis was PAI probably of autoimmune nature in a case of pulmonary thromboembolism due to APS along with autoimmune thyroid disease. Our limitations: Antiadrenal antibody not estimated.\nShe was started on hydrocortisone 100 mg three times daily and heparin. She was subsequently discharged on replacement dose of hydrocortisone and warfarin. After 6 months follow-up, the patient did not have further thrombotic episodes and requires replacement hydrocortisone.