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FRI0278 Life-Threatening Onset of Systemic Vasculitis Requiring Intensive Care Unit Admission: A Case Series
  1. S. Monti1,
  2. L. Cavagna1,
  3. S. Pieropan2,
  4. C. Montecucco1,
  5. R. Caporali1
  1. 1Rheumatology, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia
  2. 2Rheumatology, Azienda ospedaliera universitaria integrata, Verona, Italy


Background The 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 and treatment is essential to improve organ survival and reduce mortality (1).

Objectives To identify predictive signs or symptoms and past medical history warnings that can be useful to suggest a diagnosis of AAV in patients admitted to the ICU.

Methods Medical records of all patients followed at our Department with a diagnosis of AAV were analyzed, selecting those with an ICU onset.

Results A total of 88 patients with AVV were followed at our Department. We selected 10 (11.36%) patients whose disease onset was characterized by life-threatening manifestations leading to ICU admission. Mean age 41±13.55, M/F: 4/6. The type and frequency of different disease manifestations are presented in table 1. The most frequent AAV diagnosed in the ICU in our case series was eosinophilic granulomatosis with polyangiitis (EGPA) (60%), followed by granulomatosis with polyangiitis (GPA) (20%) and microscopic polyangiitis (MPA). Out of 3 patients presenting with alveolar hemorrhage, 2 (20%) had a pulmonary-renal syndrome with acute kidney injury (AKI), nephrotic-range proteinuria and microhematuria and both were diagnosed MPA. The most frequent anamnestic warning was history of asthma, presented by 40% of patients and lasting up to 30 years. 30% of patients reported recurrent nasal polyps, often treated with frequent surgical procedures without a conclusive diagnosis. Mild eosinophilia, characteristically increasing at the time of disease flare, was detectable in 30% of cases years before the diagnosis. The most frequent symptoms shortly preceding ICU admission were arthralgia and fever (30% of cases). Purpuric lesions were observed in 10% of patients. ANCA were positive in 50% of our cases: 30% c-ANCA (PR3), 20% p-ANCA (MPO). Severe anemization treated with multiple blood transfusion was present in all patients presenting with respiratory failure due to alveolar hemorrhage. Mean Birmingham Vasculitis Activity Score (BVAS) at diagnosis was 16±8.43 and 0.88±1.45 at latest follow up visit. All patients survived with a 10% rate of chronic kidney disease (CKD) 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 of disease 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.


  1. Holguin F, Ramadan B, Gal AA, Roman J. Prognostic factors for hospital mortality and ICU admission in patients with ANCA-related pulmonary vasculitis. Am J Med Sci 2008;336(4):321-6.

Disclosure of Interest None declared

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