Postpartum Pulmonary-Renal Syndrome with Thrombotic Microangiopathy in Systemic Lupus Erythematosus

Postpartum pulmonary syndrome as lupus flares in inactive or mild lupus is uncommon. The diagnosis and management of postpartum lupus flare in second pregnancy presenting with crescentic lupus nephritis (LN), secondary thrombotic microangiopathy (TMA), and severe lupus vasculitis in an undiagnosed systemic lupus erythematosus is extremely challenging. Here, in this case report, we present a young lady who presented with postpartum acute kidney injury (AKI) with systemic complaints about 4 weeks post-term uneventful delivery. Renal biopsy was suggestive of crescentic LN with severe lupus vasculitis. The stormy course was further complicated with diffuse alveolar hemorrhage, portal venous thrombosis, TMA, and anuric AKI requiring renal replacement therapy. She received multiple sessions of plasmapheresis, steroid, intravenous immunoglobulin, inj. cyclophosphamide, and started showing improvement after about 6 weeks of presentation.