Systemic Vasculitis Often First Diagnosed in ICU

PARIS — Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is not uncommonly first diagnosed in the intensive care unit.

Cardiac, pulmonary, and intestinal manifestations of systemic necrotizing vasculitis were the most frequent reasons for admission to the intensive care unit (ICU) in his series of patients with previously undiagnosed ANCA-associated vasculitis, Dr. Roberto Caporali reported in a presentation at the annual European Congress of Rheumatology.

“It may be important for ICU physicians to include ANCA-associated vasculitis/systemic vasculitis in the differential diagnosis for patients admitted to the ICU with unexplained severe systemic manifestations,” added Dr. Caporali of the University of Pavia (Italy).

The rheumatologist presented a retrospective study of 76 patients with ANCA-associated vasculitis—46 with Wegener's granulomatosis and 30 with Churg-Strauss syndrome—of whom 12 were admitted to the ICU. In 10 of the 12, the ICU was where the diagnosis of vasculitis was first made.

The two patients whose diagnosis was known prior to ICU admission had advanced disease, and both died in the hospital of multiorgan failure. In contrast, all 10 patients diagnosed in the ICU remained alive after a minimum follow-up of 24 months.

Five of the 10 patients diagnosed in the ICU were admitted because of cardiac involvement, 2 for intestinal manifestations of active systemic vasculitis, 2 because of alveolar hemorrhage, and 1 for laryngeal stenosis.

Patients with Wegener's granulomatosis had classic prodromal symptoms of ANCA-associated vasculitis—including asthma, sinusitis, nasal polyposis, and/or peripheral eosinophilia—for a median of 3 months prior to their ICU stay.

Patients with Churg-Strauss had prodromal symptoms longer, for more than 1 year on average, Dr. Caporali continued.

He said the rheumatic diseases most frequently encountered in the ICU are rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and vasculitis. Few studies have been published on systemic vasculitis in the setting of the ICU, and they are small in size because the diseases are so uncommon.

Dr. Caporali noted that last year intensivists at the Mayo Clinic reported on 38 consecutive patients with necrotizing small-vessel vasculitis admitted to the ICU. Nineteen of the patients had Wegener's granulomatosis, 16 had microscopic polyangiitis, 2 had CNS vasculitis, and 1 patient had Churg-Strauss. In contrast with the Italian experience, in only one-third of the Mayo Clinic cases was the diagnosis of vasculitis established during this hospitalization.

The reasons for ICU admission included diffuse pulmonary alveolar hemorrhage in 14 patients, sepsis in 5, seizures in 3, and pneumonia in 2. The median ICU length of stay was 4 days (Chest 2007;131:972–6).

The 28-day mortality rate was 11%, with a 1-year mortality of 29%. That was a markedly lower short-term mortality than predicted by Acute Physiology And Chronic Health Evaluation (APACHE III) scores.

A German audience member reported good success using plasmapheresis in patients with microscopic polyangiitis and pulmonary involvement, and she asked if Dr. Caporali has had a similarly favorable experience.

He replied that all patients in his study received the classic combination of high-dose steroids and cyclophosphamide, although plasmapheresis was added with good results in two patients in the ICU because of combined intestinal and renal involvement.

“I think plasmapheresis could also be a good option for patients with alveolar hemorrhage,” he added.