PMID- 31890388 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 11 IP - 11 DP - 2019 Nov 18 TI - A Case of Granulomatosis with Polyangiitis: Consequences of Delayed Diagnosis in a Life-threatening Malady. PG - e6182 LID - 10.7759/cureus.6182 [doi] LID - e6182 AB - Granulomatosis with polyangiitis (GPA) is one of three described anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Early diagnosis and treatment of GPA is paramount, as it may help prevent irreversible end-organ damage, especially renal and pulmonary failure. A 72-year-old male with a past medical history of lung adenocarcinoma in remission, chronic sinusitis status-post multiple sinus surgeries, and coronary artery disease presented with shortness of breath, dark urine, and asymmetric polyarthralgias. He had an acute kidney injury, leukocytosis, with urinalysis demonstrating pyuria and hematuria, without casts. Chest imaging showed cavitary nodular opacities in addition to interval increase of existing nodules compared to the most recent scan one month prior. His acute kidney injury progressed to renal failure requiring hemodialysis, and he developed an inflammatory polyarthritis. GPA was suspected clinically so he was started on high-dose intravenous corticosteroids, and subsequently plasmapheresis and rituximab. Serology returned with highly positive proteinase-3 antibodies, and cytoplasmic ANCA positivity on immunofluorescence. Renal biopsy demonstrated severely active pauci-immune glomerulonephritis. Several months after discharge, the patient passed away from gram positive bacteremia. This patient's recurrent sinusitis, pulmonary nodules, and subsequent renal failure were highly suggestive of GPA. A biopsy is recommended to confirm the diagnosis of GPA, but treatment should not be delayed if there is a high index of suspicion for the disease. Induction therapy with corticosteroids combined with rituximab or cyclophosphamide has significantly decreased the mortality of patients with GPA. Patients with GPA often have preceding history of nasopharyngeal and upper airway disease, and can present with fluctuating pulmonary infiltrates. Early recognition and treatment of patients with GPA can prevent life-threatening complications and reduce mortality. CI - Copyright (c) 2019, Razmjou et al. FAU - Razmjou, Amir A AU - Razmjou AA AD - Internal Medicine, University of California Los Angeles, Los Angeles, USA. FAU - Seo, Young-Ji AU - Seo YJ AD - Internal Medicine, University of California Los Angeles, Los Angeles, USA. FAU - Ayoub, Michael F AU - Ayoub MF AD - Internal Medicine, University of California Los Angeles, Los Angeles, USA. FAU - Zuckerman, Jonathan AU - Zuckerman J AD - Pathology and Laboratory Medicine, University of California Los Angeles, Los Angeles, USA. FAU - Patel, Satya AU - Patel S AD - Internal Medicine, University of California Los Angeles, Los Angeles, USA. LA - eng PT - Case Reports DEP - 20191118 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC6919960 OTO - NOTNLM OT - anca vasculitis OT - granulomatosis with polyangiitis OT - wegener granulomatosis COIS- The authors have declared that no competing interests exist. EDAT- 2020/01/01 06:00 MHDA- 2020/01/01 06:01 PMCR- 2019/11/18 CRDT- 2020/01/01 06:00 PHST- 2020/01/01 06:00 [entrez] PHST- 2020/01/01 06:00 [pubmed] PHST- 2020/01/01 06:01 [medline] PHST- 2019/11/18 00:00 [pmc-release] AID - 10.7759/cureus.6182 [doi] PST - epublish SO - Cureus. 2019 Nov 18;11(11):e6182. doi: 10.7759/cureus.6182.