Pulmonary Capillaritis in Systemic Lupus Erythematosus

There is an increasing trend of biologics usage in treating rheumatology disorders with rituximab (RTX) being recommended in organ-threatening, refractory systemic lupus erythematosus (SLE). Diffuse alveolar hemorrhage (DAH) caused by capillaritis, described first by Sir William Osler in 1895, is associated with a high mortality in SLE. A retrospective analysis of DAH was performed in hospitalized adult lupus patients from January 2006 to June 2019, focusing on the therapeutic modality. Twenty patients (1.8% incidence), 18 females (90%) ages 19 to 67 years (38.3 + 15.0), had 27 DAH episodes with SLEDAI-2K 12 to 33 (20.3 + 6.0) and involved organ numbers 2 to 7 (4.5 + 1.3) at the onset. High-dose corticosteroids, mechanic ventilator, pulse methylprednisolone, plasma exchange, cyclophosphamide and extracorporeal membrane oxygenation were prescribed in all, 15 (75%), 13 (65%), 7 (35%), 6 (30%) and 3 (15%), respectively. In particular, 5 (25%) received RTX 375 mg/m² weekly × 4 or fortnightly × 2 with a depletion of circulating B cells, and all survived without relapse, with 3 carrying a recurrent history. Seven (35%) succumbed to acute respiratory failure. There was a higher DAH incidence (3.7%) and mortality rate (73%) in admitted adult lupus patients before 1998. Despite a better critical care quality in respiratory dysfunction, this study observes a benefit of the RTX therapy in improving survival and preventing recurrence in SLE-associated DAH manifestation.