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ANCA-negative microscopic polyangiitis with diffuse alveolar hemorrhage masquerading as congestive heart failure

  • Mollaeian, Arash1
  • Chan, Nina1
  • Aloor, Rohit1
  • Iding, Jeffery S.2
  • Arend, Lois J.3
  • Saeidabadi, Seyed Hootan Forghani4
  • Haas, Christopher J.1
  • 1 MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA , Baltimore (United States)
  • 2 MedStar Franklin Square Hospital, Baltimore, MD, USA , Baltimore (United States)
  • 3 Johns Hopkins Hospital, Baltimore, MD, USA , Baltimore (United States)
  • 4 Nasseri Clinics of Rheumatic and Arthritic Diseases, Baltimore, MD, USA , Baltimore (United States)
Published Article
Autoimmunity Highlights
BioMed Central
Publication Date
Jan 06, 2021
DOI: 10.1186/s13317-020-00143-z
Springer Nature


BackgroundMicroscopic polyangiitis (MPA) is a subtype of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), involving small and medium sized vessels, often affecting the kidneys and lungs. Anti-neutrophil cytoplasmic antibody (ANCA) is detected in up to 90% of cases of MPA and its detection helps guide diagnosis, however cases of ANCA-negative MPA have been reported, hence definitive diagnosis relies on tissue biopsy.Case reportA 23-year-old man was evaluated for dyspnea and pleuritic chest pain, and found to have bilateral intra-alveolar opacities and hilar adenopathy. Diagnostic work up revealed positive anti-nuclear antibodies (ANA) and negative ANCA, which in the setting of a non-classical presentation, delayed diagnosis and appropriate treatment. Due to persistent symptoms and a high suspicion for autoimmune disease with pulmonary-renal syndrome, he underwent lung biopsy which revealed intra-alveolar hemorrhage and capillaritis indicative of microscopic polyangiitis (MPA). Surprisingly, kidney biopsy was not typical of classic MPA, but revealed less common features. Due to therapeutic noncompliance he was readmitted multiple times thereafter with rare complications of MPA such as acute pancreatitis and hemorrhagic pericardial effusion with tamponade.ConclusionThis case serves as an important clinical reminder to consider AAV even in those with negative ANCA serologies and a high suspicion for pulmonary-renal syndrome. It also demonstrates the high morbidity in cases of diagnostic delay and inadequate treatment.

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