Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
ANCA associated vasculitis (AAV) is known as a systemic vasculitis with unknown etiology. Recently, relationship between AAV and complement have been shown and complement have an important role in the pathogenesis of AAV (1). Regarding clinical feature of AAV, hypocomplementemia is reported to be associated with a poor prognosis in a study with a small number of patients (2). However, the clinical characteristics of AAV with hypocomplementemia still remain unclear. In this study, we tried to show the clinical characteristics of AAV with hypocomplementemia.
We retrospectively analyzed patients in Nagasaki University Hospital who were newly diagnosed with AAV from April 2008 to June 2015. We analyzed the baseline variables, laboratory data, clinical symptoms and therapeutic outcomes after treatments including episodes of relapses, initiation of dialysis and death using medical records. All patients were classified as AAV based on the Chapel Hill Consensus Conference (CHCC) criteria and the European Medicines Agency (EMA) algorithm. The patients consist of all types of AAV including eosinophilic granulomatous polyangiitis (EGPA), granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), and renal limited vasculitis. We defined hypocomplementemia as the state which at least one of each complement 3 (C3), complement 4 (C4), or total complement activity (CH50) was lower than lower limits of normal range of each complements.
We included 82 patients with AAV (11 EGPA, 14 GPA, 54 MPA and 3 renal limited vasculitis) who were newly diagnosed. The median onset age was 71. Forty eight patients (59%) were female. Median follow up duration was 40 months. Seventeen patients (21%) had hypocomplementemia at diagnosis of AAV. Compared to AAV without hypocomplementemia, AAV with hypocomplementemia had significantly higher rates of occurrences of diffuse alveolar hemorrhage (DAH) (7 (41%) vs 5 (8％), p<0.01), thrombotic microangiopathy (TMA) (3 (18%) vs 0 (0％), p<0.01) and skin lesions (9 (53%) vs 8 (12％), p<0.01). Of 17 patients who had hypocomplementemia, only one patient fulfilled American college of rheumatology revised criteria for classification of systemic lupus erythematosus (SLE) concomitantly. Assessed by life-table analysis using the Kaplan-Meier method, hypocomplementemia at disease onset was associated with a poor prognosis (p=0.012).
Our result suggested that hypocomplementemia in AAV might become risk factor for DAH and TMA. Moreover hypocomplementemia was a poor prognostic factor in AAV in this study. Therefore it is significantly important to pay attention to the levels of complement at the diagnosis of AAV.
1. Nat Rev Rheumatol. 2014;10(8):463-73.
2. Nephron Clin Pract. 2014;126(1):67-74.
To cite this abstract in AMA style:Fukui S, Iwamoto N, Umeda M, Nishino A, Nakashima Y, Koga T, Kawashiri SY, Ichinose K, Hirai Y, Tamai M, Nakamura H, Origuchi T, Kawakami A. ANCA Associated Vasculitis with Hypocomplementemia Has More Diffuse Alveolar Hemorrhage and a Poor Prognosis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/anca-associated-vasculitis-with-hypocomplementemia-has-more-diffuse-alveolar-hemorrhage-and-a-poor-prognosis/. Accessed August 14, 2020.
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