PMID- 36874702
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230307
IS  - 2168-8184 (Print)
IS  - 2168-8184 (Electronic)
IS  - 2168-8184 (Linking)
VI  - 15
IP  - 1
DP  - 2023 Jan
TI  - A Diagnostic Dilemma of a Case of Granulomatosis With Polyangiitis (GPA) 
      Presenting With Thrombotic Vasculopathy.
PG  - e34479
LID - 10.7759/cureus.34479 [doi]
LID - e34479
AB  - Granulomatosis with polyangiitis (GPA) is a rare disease with a prevalence of 
      about three in 100,000 persons in the United States. GPA is an antineutrophil 
      cytoplasmic antibody (ANCA)-associated vasculitis affecting predominantly 
      small-sized vessels. It can present with localized or systemic symptoms with 
      multiple organ involvement, thus making diagnosis challenging. Common skin 
      lesions in GPA are palpable purpura, petechiae, ulcers, and livedo reticularis. 
      These lesions usually have underlying vasculitis with or without granuloma on 
      histology findings. To date, there have been no previous reports about thrombotic 
      vasculopathy in GPA before. We present a case of a 25-year-old female who 
      presented with intermittent joint pain for weeks, purpuric rash, and mild 
      hemoptysis for a few days. A review of systems was notable for a 15-pound weight 
      loss in one year. Physical examination was significant for a purpuric rash on the 
      left elbow and toe, and left knee swelling and erythema. Presenting laboratory 
      results were notable for anemia, indirect hyperbilirubinemia, mildly elevated 
      D-dimers, and microscopic hematuria. Chest radiograph revealed confluent airspace 
      disease. Extensive infectious workup was negative. A skin biopsy of her left toe 
      revealed dermal intravascular thrombi without evidence of vasculitis. The 
      thrombotic vasculopathy did not favor vasculitis but raised concern for a 
      hypercoagulable state. However, extensive hematologic workup was negative. 
      Bronchoscopy findings were consistent with diffuse alveolar hemorrhage. Later, 
      cytoplasmic ANCA (c-ANCA) and anti-proteinase 3 (PR3) antibody titers 
      were positive. Her diagnosis was unclear since both skin biopsy and bronchoscopy 
      were nonspecific and inconsistent with her positive antibody results. The patient 
      eventually underwent a kidney biopsy, which showed pauci-immune necrotizing and 
      crescentic glomerulonephritis. Finally, a diagnosis of granulomatosis with 
      polyangiitis was made based on the kidney biopsy and positive c-ANCA. The patient 
      was treated with steroids and IV rituximab and discharged home with outpatient 
      rheumatology follow-up. Due to multiple signs and symptoms including thrombotic 
      vasculopathy, there was a diagnostic dilemma requiring a multidisciplinary 
      approach. This case highlights the importance of pattern recognition for the 
      diagnostic framework of rare disease entities and the multidisciplinary 
      collaborative efforts required to reach the final diagnosis.
CI  - Copyright (c) 2023, Jaroenlapnopparat et al.
FAU - Jaroenlapnopparat, Aunchalee
AU  - Jaroenlapnopparat A
AD  - Internal Medicine, Mount Auburn Hospital, Harvard Medical School, Cambridge, USA.
FAU - Banankhah, Peymaan
AU  - Banankhah P
AD  - Internal Medicine, Mount Auburn Hospital, Harvard Medical School, Cambridge, USA.
FAU - Khoory, Joseph
AU  - Khoory J
AD  - Internal Medicine, Mount Auburn Hospital, Harvard Medical School, Cambridge, USA.
FAU - Jani, Chinmay
AU  - Jani C
AD  - Internal Medicine, Mount Auburn Hospital, Harvard Medical School, Cambridge, USA.
FAU - Sehra, Shiv
AU  - Sehra S
AD  - Rheumatology, Mount Auburn Hospital, Harvard Medical School, Cambridge, USA.
LA  - eng
PT  - Case Reports
DEP - 20230131
PL  - United States
TA  - Cureus
JT  - Cureus
JID - 101596737
PMC - PMC9981548
OTO - NOTNLM
OT  - anca vasculitis
OT  - gpa
OT  - granulomatosis with polyangiitis
OT  - hemoptysis
OT  - thrombotic vasculopathy
OT  - wegener's granulomatosis
COIS- The authors have declared that no competing interests exist.
EDAT- 2023/03/07 06:00
MHDA- 2023/03/07 06:01
PMCR- 2023/01/31
CRDT- 2023/03/06 03:59
PHST- 2023/01/31 00:00 [accepted]
PHST- 2023/03/06 03:59 [entrez]
PHST- 2023/03/07 06:00 [pubmed]
PHST- 2023/03/07 06:01 [medline]
PHST- 2023/01/31 00:00 [pmc-release]
AID - 10.7759/cureus.34479 [doi]
PST - epublish
SO  - Cureus. 2023 Jan 31;15(1):e34479. doi: 10.7759/cureus.34479. eCollection 2023 
      Jan.