Background/Purpose: ANCA-associated vasculitis (AAV) has a high rate of complications, both from disease itself and treatments. Hospital mortality rates for AAV range between 10-20%. There is a lack of information regarding reasons for hospitalization and outcomes of these hospitalizations.  It is important to characterize the current state of treatment and outcomes in AAV in order to improve patient care.

Methods: A retrospective chart review was performed at a large academic medical center in which all hospitalizations were reviewed in adults with a diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) from 10/1/2015 – 12/31/2018. The electronic medical record (EMR) was queried for in-patient visits with an ICD-10 code of M30.1, M31.30, M31.31, and M31.7. The diagnosis of vasculitis was made by the treating rheumatologist or nephrologist.  Patients with eosinophilic granulomatosis with polyangiitis (EGPA), without AAV or an unclear diagnosis, and absent ANCA titers were excluded. Differences in outcomes between GPA and MPA patients were measured by Fisher’s exact test and t-tests.

Results: There were 127 total hospitalizations amongst 54 patients (33 GPA, 21 MPA). Forty-one hospitalizations (35 patients) were for active disease: 25 had a new vasculitis diagnosis and 16 for recurrence or flare. Patients with new disease were most commonly admitted with simultaneous lung (n=15) and renal involvement (n=16); 8 of these patients had diffuse alveolar hemorrhage (DAH). Patients with recurrent disease were most commonly admitted for respiratory disease, 6 with lower respiratory and 5 with upper airway disease (1 required emergent tracheotomy).

Of the 86 hospitalizations in patients with inactive disease (n=31 patients), infection caused almost half of admissions (41%, n=36) with pneumonia and skin/soft tissue infections being most common. There were 24 admissions for cardiovascular related causes; 9 for blood loss (most commonly GI bleed (n=6)); 4 for a deep vein thrombosis; 4 for osteoporotic fractures, and only 1 patient had a new diagnosis of cancer (urothelial carcinoma with remote history of prior Cytoxan).

There were 27 ICU admissions among 21 patients (39% of all patients) attributed to respiratory failure in 56%, renal failure in 33%, and infection in 26% of cases. More than half (56%) were for active disease and 10 of these had new disease.  Mechanical ventilation and vasopressors were required in nearly half of ICU admissions.

Overall mortality was 7% for hospitalized patients and 19% for those admitted to the ICU (3 GPA, 1 MPA). All 4 hospital deaths occurred in the setting of infection.  An additional 5 patients died within 28 days of discharge, for a total mortality rate of 17% (n=9).

Conclusion: Patients with GPA and MPA can have frequent hospitalizations, many that are not related to active disease. Those hospitalized with new disease were more likely to have both pulmonary and renal involvement.  Although outcomes have improved considerably, mortality rates for hospitalized patients with disease remain high.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/hospital-admissions-and-mortality-in-patients-with-anca-associated-vasculitis/