Clinical analysis of patients with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis

Abstract

ABSTRACT. This prospective study analyzed the clinical characteristics of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis and explored the relationship between MPO-ANCA and clinical manifestations of the associated vasculitis in 132 p-ANCA and MPO-ANCA-positive patients (average age, 62.3 ± 14.8 years) who were initially diagnosed with ANCA-associated vasculitis. The p-ANCA and MPO-ANCA levels in peripheral blood were detected in all patients. Among these, 128 (97%) had microscopic polyangiitis (MPA), 3 (2.3%) had granulomatous polyangiitis, and 1 (0.7%) had eosinophilic granulomatous vasculitis. The average time of diagnosis was 10.2 ± 18 months; only 14 (10.6%) patients were diagnosed within 1 month. The main organs involved and the corresponding number of patients were: renal, 95 (72%); lung, 89 (67.4%); joints, 35 (26.5%); heart, 26 (19.7%); peripheral nerve, 23 (17.4%); skin rash, 14 (10.6%); and CNS, 13 (9.8%). Older patients were more likely to show lung involvement in the early disease stage, whereas the joints were involved mostly in the younger patients. The p-ANCA levels (mean titers, 1:60) were not correlated with disease activity and extent of organ (2015) involvement, and the MPO-ANCA levels were positively correlated with disease activity, but had no correlation with the extent of organ involvement. MPO-ANCA vasculitis is a common occurrence in China; it mainly involves the elderly and presents as clinical manifestations of MPA. However, the multiple organ damage is not specific leading to delay in diagnosis. MPO-ANCA may play a pathogenic role in the associated vasculitis, and the diverse clinical manifestations might be related with the different characteristics of MPO-ANCA.

Interstitial Pneumonia in a 94-Year-Old Woman with MPO-ANCA Positive Vasculitis

“…Older patients were more likely to show lung involvement in the early disease stage in Japan [6]. This tendency is compatible with European patients [2].…”

“…According to the guideline for IP [5], basically all IP cases are recommended to examine serum ANA, MPO-ANCA, PR3-ANCA and other autoimmune antibody as a routine screening test to exclude the collagen vascular disease from IIPs. In the elderly, other symptoms such as hearing loss, recognition impairment, and cerebral infarction often disturb the definite diagnosis which will be taken several months [2]. Therefore, improving awareness and vigilance regarding this disease is important for early diagnosis and treatment.…”

The Prevalence and Management of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in China

“…It was reported that in consistency with studies from Western countries, AAV could affect people of all ages, with elderly people being more susceptible, and the kidneys and lungs were the most vulnerable organs [8,9]. In our referral diagnostic center in Peking University First Hospital, over 200 new patients with AAV are diagnosed annually; this indicates that AAV is not rare in Chinese [10].…”

“…Compared with younger patients, older patients were more likely to suffer from MPA than GPA [16]. It was found that pulmonary involvement was more prevalent and more severe in older patients than in younger ones [9,16]. Those findings indicated that patients with different ages might have different susceptibility to variant disease entities and organ involvement of AAV.…”

Cardiac manifestations in antineutrophil cytoplasmic autoantibody (ANCA) — associated vasculitides

“…In a series of 85 MPA patients only 10% had pericarditis, cardiac failure occurred in 18%, and MI in 2% [48]. In another Chinese cohort cardiac involvement was present in one-fifth of the patients, with pericardial involvement (pericarditis and pericardial effusion), cardiomyopathy, aortic incompetence, and rhythm disturbances being described [49].…”