PMID- 33407881
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20210112
IS  - 2038-0305 (Print)
IS  - 2038-3274 (Electronic)
IS  - 2038-0305 (Linking)
VI  - 12
IP  - 1
DP  - 2021 Jan 6
TI  - ANCA-negative microscopic polyangiitis with diffuse alveolar hemorrhage 
      masquerading as congestive heart failure.
PG  - 1
LID - 10.1186/s13317-020-00143-z [doi]
LID - 1
AB  - BACKGROUND: Microscopic 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 REPORT: A 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. CONCLUSION: This 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.
FAU - Mollaeian, Arash
AU  - Mollaeian A
AUID- ORCID: 0000-0002-1483-2241
AD  - MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA. 
      arash.mollaeian@medstar.net.
FAU - Chan, Nina
AU  - Chan N
AD  - MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA.
FAU - Aloor, Rohit
AU  - Aloor R
AD  - MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA.
FAU - Iding, Jeffery S
AU  - Iding JS
AD  - Department of Pathology, MedStar Franklin Square Hospital, Baltimore, MD, USA.
FAU - Arend, Lois J
AU  - Arend LJ
AD  - Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA.
FAU - Saeidabadi, Seyed Hootan Forghani
AU  - Saeidabadi SHF
AD  - Nasseri Clinics of Rheumatic and Arthritic Diseases, Baltimore, MD, USA.
FAU - Haas, Christopher J
AU  - Haas CJ
AD  - MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA.
LA  - eng
PT  - Journal Article
DEP - 20210106
PL  - England
TA  - Auto Immun Highlights
JT  - Auto- immunity highlights
JID - 101532391
PMC - PMC7788985
OTO - NOTNLM
OT  - ANCA
OT  - Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis
OT  - Microscopic Polyangiitis
OT  - Vasculitis
COIS- Authors hereby declare no personal or financial conflict of interest.
EDAT- 2021/01/08 06:00
MHDA- 2021/01/08 06:01
PMCR- 2021/01/06
CRDT- 2021/01/07 05:51
PHST- 2020/05/19 00:00 [received]
PHST- 2020/10/07 00:00 [accepted]
PHST- 2021/01/07 05:51 [entrez]
PHST- 2021/01/08 06:00 [pubmed]
PHST- 2021/01/08 06:01 [medline]
PHST- 2021/01/06 00:00 [pmc-release]
AID - 10.1186/s13317-020-00143-z [pii]
AID - 143 [pii]
AID - 10.1186/s13317-020-00143-z [doi]
PST - epublish
SO  - Auto Immun Highlights. 2021 Jan 6;12(1):1. doi: 10.1186/s13317-020-00143-z.