A Diagnostic Difficulty on ANA Negative/ Seronegative Systemic Lupus Erythematosus with Diffuse Alveolar Haemorrhage

Abstract

A 45-year-old hypertensive woman had a dry cough, dyspnea, and a crusted nonhealing ulcer on her right ankle for one month. She was suffering from worsening anaemia, proteinuria, and thrombocytopenia. A CT pulmonary angiogram revealed pulmonary hypertension, a thrombus in the left internal jugular vein, and bilateral multilobar opacities. A Doppler USG revealed thrombus in the right popliteal and left jugular veins. Serology revealed a low C3, C4, negative ANA with antiphospholipid antibodies that were twice positive. Diffuse alveolar bleeding was confirmed by bronchoalveolar lavage (DAH). An ulcer biopsy revealed lymphocytic vasculitis, which is a sign of immune complex deposition disorder.ANA negative SLE with DAH was identified after meeting the clinical and immunological criteria of the Systemic Lupus International Collaborating Clinics (SLICC).