title: Life-threatening onset of systemic vasculitis requiring intensive care unit admission: a case series.
abstract: OBJECTIVE
  Onset of ANCA-associated vasculitis (AAV) can be abrupt with life-threatening manifestations requiring Intensive Care Unit (ICU) admission. A high level of suspicion leading to prompt diagnosis is essential. Our objective was to investigate the epidemiologic characteristics and the type of life-threatening manifestations.
  
  
  METHODS
  Medical records of AAV patients were analysed, selecting those with an ICU onset to identify predictive signs or symptoms and past medical history warnings useful for diagnosis.
  
  
  RESULTS
  Out of 90 patients with AAV, 10 (11.1%) showed an ICU onset. The most frequent AAV diagnosed in the ICU was eosinophilic granulomatosis with polyangiitis (EGPA) (60%), followed by granulomatosis with polyangiitis (GPA) (20%) and microscopic polyangiitis (MPA) (20%). Cardio-pulmonary involvement was the main cause for ICU admission (70%) and significantly distinguished the ICU onset group from other AAV. The most frequent anamnestic warnings were history of asthma (50%), nasal polyps (30%), eosinophilia (30%). Symptoms shortly preceding ICU admission were arthralgia, fever (30%) and purpuric lesions (20%). ANCA were positive in 60% of patients. Mean Birmingham Vasculitis Activity Score (BVAS) at diagnosis was 16±8.43 and 0.88±1.45 at the end of follow up. All patients survived with a 10% rate of chronic kidney disease and a mean Vasculitis Damage Index (VDI) of 2±1.15.
  
  
  CONCLUSIONS
  Keeping a high level of suspicion for AAV is mandatory, particularly when treating life-threatening onset manifestations in the ICU. A history of asthma, nasal polyps, eosinophilia and arthralgia should always be investigated. ANCA are negative in about half of cases, therefore clinical expertise and strict collaboration with the rheumatologist are still pivotal.
authors.affiliation: Division of Rheumatolgy, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Monti
authors.affiliation: Division of Rheumatolgy, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Montecucco
authors.affiliation: Division of Rheumatology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Pieropan
authors.affiliation: Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, Section of Surgery and Anesthesiology, Unit of Anesthesia, Intensive Care and Pain Therapy, University of Pavia, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Mojoli
authors.affiliation: Anesthesia and Intensive Care Division I, Emergency Department- IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Braschi
authors.affiliation: Division of Rheumatolgy, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Caporali
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000453:epidemiology; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000453:epidemiology*; D015331:Cohort Studies; D016638:Critical Illness; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000453:epidemiology*; D006333:Heart Failure / Q000209:etiology; D006470:Hemorrhage / Q000209:etiology; D006760:Hospitalization; D006801:Humans; D007362:Intensive Care Units; D007829:Laryngostenosis / Q000209:etiology; D008171:Lung Diseases / Q000209:etiology; D008297:Male; D065666:Mesenteric Ischemia / Q000209:etiology; D055953:Microscopic Polyangiitis / Q000150:complications / Q000453:epidemiology*; D008875:Middle Aged; D012131:Respiratory Insufficiency / Q000209:etiology; D012189:Retrospective Studies