The important role of Internist in the recognition of ANCA-associated vasculitis in an ordinary Internal Medicine ward

ABSTRACT

Introduction: Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) are a group of systemic vasculitides that predominantly affect small vessels. Clinical phenotype is heterogeneous kidney and/or pulmonary involvement is described in up to 75-90% of cases. Among these, rapidly progressive glomerulonephritis and diffuse alveolar haemorrhage are the most serious manifestations. Description of the case A 73-year-old man presented with 15 days history of fever, productive cough and fatigue (SARS-CoV- 2 nasopharyngeal swab negative);antibiotic therapy prescribed was ineffective. Afterwards he developed necrosis of right hand distal phalanx, oedema of lower extremities, dyspnoea for mild exertion and acute renal failure, that needed dialysis. A lung HRCT showed ground glass opacities, blood tests underlined high inflammatory indices and high title PR3-ANCA positivity. Diagnosis of PR3-ANCA associated vasculitis was made and glucocorticoid therapy with methylprednisolone (1mg/Kg/day) was started. For the occurrence of emoptysis, respiratory failure and anemization, suspecting diffuse alveolar haemorrhage, methylprednisolone pulse therapy (500 mg/day for 5 days), plasmapheresis and rituximab (375 mg/m2/week for 4 infusions) were administered. There was a progressive clinical-laboratory improvement. However the patient developed intestinal perforation and died. Conclusions: AAV represent rare diseases, burdened by a poor prognosis. They need a careful internist and a multidisciplinary approach to ensure an early therapeutic intervention.