Case Presentation: 72-year-old male with a history of prostate cancer status post prostatectomy, recent diagnosis of pansinusitis and multiple bilateral pulmonary lesions and a mass-like consolidation of the left upper lobe presented complaining of dyspnea. He was receiving treatment for sinusitis and pneumonia with plans for outpatient bronchoscopy to rule out malignancy. On presentation, the patient also complained of headache, hearing loss and right visual loss associated with redness and photophobia. On the physical exam, palpable purpura was noted on upper and lower extremities. Labs revealed acute kidney injury and elevated inflammatory markers. CT chest showed bilateral alveolar airspace disease with no evidence of pulmonary embolism. Steroid was initiated for concerns of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis. During the hospitalization, he developed acute hypoxic respiratory failure requiring intubation. Patient underwent bronchoscopy with evidence of diffuse alveolar hemorrhage. He was found to have a left lower extremity deep vein thrombosis for which he underwent inferior vena cava filter. Infectious work-up was negative. While he was intubated, he was started on high dose steroids, received plasma exchange as well as the first Rituximab dose. Patient required hemodialysis (HD) due to worsening kidney function. Patient improved during his hospital stay and he was able to be extubated. Rheumatological work-up was positive cytoplasmic ANCA (C-ANCA) pattern and autoantibodies to Proteinase-3 (PR3). Upon discharge, he was referred to Rheumatology, Nephrology for HD and Ophthalmology for evaluation of vision loss.
Discussion: Vasculitis is a multisystemic disease that is caused by inflammation of blood vessel walls, which affects vessels of different sizes. ANCA associated vasculitis (AAV) affects mainly small blood vessels in the body. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (1). The clinical presentation can vary considerably depending on the system involved. It may present with constitutional symptoms or with specific features of end-organ involvement (2). Multiple organs can be affected, including the skin, kidneys, respiratory tract, and nervous system. The diagnosis is built on a compatible pattern of clinical features supported by specific serological or radiological investigations and confirmatory biopsy (3). Vasculitis can progress rapidly and be fatal. Severe, untreated GPA is associated with up to 90% of patients dying within 2 years (4).
Conclusions: In this case, the patient was diagnosed with AAV, based on clinical presentation and positive C-ANCA pattern and PR3 autoantibodies. Early diagnosis of vasculitis is crucial to allow early initiation of therapy and avoid poor outcomes. In this patient, the treatment was initiated without confirmatory kidney biopsy. We recognize that tissue biopsy is vital for diagnosis, however, this should not delay treatment when presentation is suggestive of vasculitis. Reducing delays in diagnosis can reduce morbidity and mortality and improve clinical outcomes.