Background Primary vasculitides usually pose a diagnostic challenge. They represent a wide spectrum of heterogeneous vascular disorders characterized by variable target vessel involvement, vascular abnormalities, and end organ damage . Several studies about primary vasculitides expose epidemiologic data, mainly from North America and Europe. Nevertheless, data in Latin America is scarce .
Objectives To describe the prevalence and clinical presentation of primary vasculitides.
Methods This was an observational cross-sectional study in which well-characterized patients were assessed from 2001 to 2016. Each patient was evaluated by the same rheumatologist in a single rheumatology outpatient center in Bogota, Colombia. Patients were classified either according to ACR 1990 criteria (Granulomatosis with polyangiitis, Eosinophilic granulomatosis with polyangiitis, Henoch-Schönlein purpura, Polyarteritis nodosa, Takayasu arteritis, Giant cell arteritis), EMA algorythm (Microscopic polyangiitis) or 2012 revised Chapell Hill nomenclature criteria (Variable Vessel Vasculitis, Single-Organ Vasculitis). ANCA-Associated Vasculitis (AAV) was used to define Small Vessel Vasculitis (SVV) not fulfilling either ACR 1990 criteria or EMA algorythm.
Results A total of 56 patients were included. Baseline characteristics of patients were as follows: female gender 75%, mean age 56±15.7 years, and median disease duration 3 (IQR 6) years. Most of patients (73%) were diagnosed at first year of disease onset, and fulfilled international classification criteria (63%). SVV was the most frequent phenotype (55%), and C-ANCA/anti-PR3 were the most frequently identified auto-antibodies (40%). Musculoskeletal manifestations (arthralgia, arthritis, and myalgia), mucocutaneous disorders (including vasculitic/necrotic lesions, mucosal ulcers, and purpura), neurological compromise (peripheral and central), and renal involvement (acute renal failure, glomerulonephritis, and lung-kidney syndrome) were the most frequently reported onset symptoms in 29%, 29%, 23%, and 21%, respectively. Interestingly, most of patients did not develop organic compromise other than the onset manifestation form. Thirteen percent of patients fulfilled polyautoimmunity criteria and 9% presented with multiple autoimmune syndrome. Antiphospholipid syndrome was the most common associated autoimmune disease described. Corticosteroids were the most common treatment used in 93% of patients, followed by azathioprine in 57%, cyclophosphamide, methotrexate, and rituximab in 29%, and antimalarials in 27% (Tab.1). No deaths occurred during follow-up.
Conclusions Vasculitides are conditions with several subphenotypes, being ANCA-associated the most frequently reported. Onset symptoms seem to be the main drivers of disease evolution. Appropriate and prompt diagnosis is critical to enable timely intervention, aimed to prevent end organ damage and reduce morbidity in these patients. Controlling disease activity and preventing progression is the milestone of treatment. Characterization of Latin America population is pivotal to raise awareness of health-care workers, and policy makers.
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Disclosure of Interest None declared