ANCA-Positive late-onset lupus and diffuse alveolar hemorrhage: Overlap or new clinical entity?

A case report.

Abstract

Introduction: Systemic lupus erythematosus (SLE) usually occurs in young people and manifests acutely, whereas in late cases (over 50 years of age), it is rare and insidious. The co-presence with neutrophil cytoplasmic antibodies (ANCA), characteristic of most vasculitis, is rare and leads to worse clinical outcomes.

Clinical case: A 65-year-old man diagnosed with SLE for 4 years, with mild skin and joint symptoms. In the last 3 months, he presented with asthenia, adynamia, cough with hemoptysis, edema of the lower limbs, and proteinuria in the nephrotic range. Chest CT showed alveolar occupation with a ground glass pattern; Bronchoscopy showed diffuse alveolar hemorrhage. Renal biopsy reported class IV lupus nephritis, diffuse extracapillary proliferation, and grade I interstitial fibrosis. The diagnosis of lung-renal syndrome is considered.

Conclusion: the concomitance between these autoimmune pathologies is unusual; there is a late-onset overlap syndrome between lupus nephritis and MPO-ANCA pauciimmune glomerulonephritis. In turn, as it presents more aggressive evolutions, it requires greater renal replacement therapy, plasmapheresis, and tumor necrosis factor inhibitors.