Background Recent clinical studies indicated that there is a geographical difference in the incidence of SNV and anti-neutrophil cytoplasmic antibodies (ANCA) pattern between Asian and Caucasian (1). Therefore we cannot exclude that there are regional differences in the clinical pattern of SNV, it is important especially for PAN, which has no specific laboratory markers. The data about it is limited in Ukraine.
Objectives To identify the main early characteristics of PAN and to compare clinical and laboratory features with other SNV in the cohort of 192 patients (pts) from 4 regions of the north and central parts of Ukraine observed from 1979 till the end of 2012.
Methods All pts who fulfilled the ACR 1990 criteria or the CHCC of PAN or other SNV were included. Their clinical features, date/age at diagnosis, sex, laboratory findings including ANCA, rheumatoid factor (RF), antinuclear antibody (ANA) at diagnosis were analyzed. Clinical activities of pts were calculated according to the Birmingham Vasculitis Activity Score (BVAS).
Results 112 PAN pts were compared with 80 pts with other SNV (granulomatosis with polyangiitis – 38.7%, eosinophilic granulomatosis with polyangiitis – 42.5%, microscopic polyangiitis – 18.8%). There were no significant differences between both groups in median age of onset and clinical activity according to BVAS (p>0.05). The pts with PAN were predominantly males (65% vs 38% in other SNV, p=0.0001.) Cutaneous manifestations (44% vs 29%), abdominal pain (13% vs 2.5%), peripheral nervous system (PNS) involvement (31% vs 11%), fever (96% vs 86%), myalgia (79% vs 16%), weight loss (61% vs 45%), arthritis (48% vs 30%), diastolic arterial hypertension (DAH) (30% vs 1.3%) occurred more often in pts with PAN (p<0.05), whereas pulmonary involvement (15 % vs 49%) was more frequent in compared group (p=0.0001). Testicular pain occurs only in 2 pts of PAN’s group. Asthma, upper respiratory tract involvement, massive haemoptysis, ocular manifestations and glomerulonephritis were found exclusively in pts with other SNV. The groups also differed in the frequency of laboratory findings: leukocytosis (37% pts with PAN and 23% pts with other SNV), eosinophilia (8% vs 20%), HBs Ag (9% vs 1%), haematuria (16% vs 38%) and c-ANCA (13.6% vs 46.4%), (all p<0.05). There were no significant differences between PAN’s and SNV’s groups in other laboratory results and in the frequency of involvement of other organs and systems: CNS involvement (7.14% vs 3.75% respectively), cranial nerve dysfunction (6.25% vs 3.25%), cardiac involvement (6.25% vs 3.25%), myositis (5.4% vs 1.25%), Raynaud’s syndrome (8% vs 2.5%).
Conclusions General symptoms (fever, weight loss), myalgia, arthritis, cutaneous and PNS involvement, DAH, gastrointestinal symptoms, leukocytosis, positive HBs Ag are common for pts with PAN, compared with other SNV. Asthma, upper respiratory tract involvement, massive haemoptysis, ocular manifestations and glomerulonephritis not occur in pts with PAN from the north and central regions of Ukraine.
Fujimoto S., et al. Comparison of the epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis between Japan and the U.K. Rheumatology (Oxford).2011;50:1916-20
Disclosure of Interest None Declared