Impact of renal involvement on survival in ANCA-associated vasculitis

Rathi, Manish; Pinto, Benzeeta; Dhooria, Aadhaar; Sagar, Vinay; Mittal, Tarun; Rajan, Roopa; Dhir, Varun; Kumar, Susheel; Sharma, Kusum; Nada, Ritambhra; Singh, Surjit; Minz, Ranjana Walker; Sharma, Aman

doi:10.1007/s11255-016-1330-z

Cited by 9 publications

(6 citation statements)

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“…Thus, it was not an unduly delayed diagnosis but rather a truly more severe disease course from the beginning which characterizes men with GPA. Indeed, there are some reports suggesting that, at least in terms of kidney involvement, this might be the case [34][35][36]. These results also raise the question about the most appropriate treatment regimen and monitoring in men with GPA.…”

Section: Discussionmentioning

confidence: 92%

Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry

et al. 2019

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Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence Electronic supplementary material The online version of this article (

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Section: Discussionmentioning

confidence: 92%

Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry

et al. 2019

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“…As was shown previously by our group, renal outcomes were good in Indian patients with GPA with 42.9% of patients having normalization of renal functions, 28.6% having persistent renal insufficiency and only 2.9% patients developing end-stage renal disease. 16 Whether any genetic factors are contributing to this difference needs to be studied in detail. Indian patients seem to have a slightly lower frequency of upper airway involvement, as only 79% of patients in our study and 80% of patients in the study by Kumar et al had ear, nose and throat manifestations compared to 90-98% among European and American patients.…”

Section: Discussionmentioning

confidence: 99%

Clinical features and long‐term outcomes of 105 granulomatosis with polyangiitis patients: A single center experience from north India

et al. 2017

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Aim: To describe the clinical features, treatment and long-term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA).Methods: Clinical details, Birmingham Vasculitis Activity Score (BVAS-v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories. The findings were compared with large single center cohorts from USA, Germany and France.Results: One hundred and five patients (median age 40 years; 60 females and 45 males) were diagnosed as having GPA. Median duration of follow up was 28 months. Upper respiratory tract, renal and lung involvement were seen in 76.19%, 51.43% and 67.62% of patients, respectively. Ocular, ear, central nervous system, peripheral nervous system, heart, gastrointestinal and skin involvement were seen in 40.95%, 18.1%, 26.67%, 11.43%, 5.71%, 12.38% and 29.52%, respectively. The median BVAS v3 score was 17. Localized, early systemic, generalized, severe and refractory disease were seen in three, 44, 38, 17 and one patient, respectively. Eighteen patients expired and the cause of death was disease activity in 11, disease activity plus sepsis in four and sepsis alone in three patients. Twenty-six patients experienced relapses. No hemorrhagic cystitis or bladder cancers were observed; however, one patient developed myelodysplastic syndrome during follow up.Conclusion: Indian patients are younger and have less arthritis, renal and peripheral nerve involvement as compared to the patients in Western cohorts.

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“…Renal involvement has not been shown to affect the overall survival among patients with AAV . Out of the 35 GPA patients, 15 (42.9%) patients had normalization of renal parameters, 9 (28.6%) had persistent renal dysfunction, 1 developed end‐stage renal failure and 8 (22.9%) patients died.…”

Section: Response and Relapsesmentioning

confidence: 98%

“…Rituximab has been shown to be effective in treatment‐resistant pachymeningitis and pyoderma gangrenosum in GPA . Other modes of therapy used in the treatment of GPA were plasma exchange, for severe renal dysfunction and diffuse alveolar hemorrhage, intravenous immunoglobulin, methotrexate and mycophenolate mofetil . After therapy with glucocorticoids and immunosuppressants, remission was achieved in 53%‐64% of patients .…”

Section: Response and Relapsesmentioning

confidence: 99%

Is granulomatosis with polyangiitis in Asia different from the West?

et al. 2018

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The incidence and clinical features of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) have been shown to vary according to geographical areas, with granulomatosis with polyangiitis (GPA) being more common in northern Europe and microscopic polyangiitis (MPA) being more common in Asian countries.The annual incidence of GPA among Asians varies between 0.37/million to 2.1/million population. The prevalence of GPA has been estimated to be 1.94/100 000 in a Chinese population. Polymorphisms in class II major histocompatibility genes and ETS1 proto-oncogene has been shown in Asian patients with GPA. There is a difference in mean age at onset and proteinase 3 (PR3) or myeloperoxidase (MPO) positivity in GPA patients from different Asian countries. Those from India had mean age of 40 years and those from Japan had mean age of 65 years. Sixty percent of GPA patients from China and Japan were MPO ANCA positive while the majority of patients from India and Korea were PR3 positive. Geographical variation with lower frequency of renal involvement in Indian studies and higher frequency in Chinese patients has also been noted. Treatment outcomes have been similar to those reported from other parts of the world. Remission was achieved in about two-thirds of patients while relapses were noted in one-third to half of the patients. Apart from minor differences in the organ systems involved, MPO-ANCA GPA and PR3-ANCA GPA had similar rates of remission and relapses. K E Y W O R D S Asia, granulomatosis with polyangiitis, vasculitisWe searched PubMed using the search terms "Granulomatosis with polyangiitis", "GPA" and "Wegener's granulomatosis". We screened for articles published from Asian countries and describing the epidemiology, pathogenesis, clinical features and outcomes of GPA patients. Case reports and small case series of less than 5 patients were not included. The basis of diagnosis of GPA in different studies was either histopathology suggestive of GPA or fulfilling the American College of Rheumatology (ACR) criteria for GPA or classified according to European Medicines Evaluation Agency classification. We also included large case series on GPA from Europe and USA to compare them with Asian studies. | EPID EMIOLOGYThe incidence and prevalence of GPA shows a regional and ethnical difference with higher incidence of GPA in northern Europe and lower incidence in Asian countries. 1 The precise estimates of incidence and prevalence of AAV and GPA are not available from Asian countries. In the epidemiological study by Fujimoto et al, 1 the annual incidence of GPA in Japan was calculated to be 2.1/million population, which was much lower than that noted in the UK (annual incidence of 14.3/million). The annual incidence of MPA was higher in Japan than the UK and the overall incidence of all AAV was similar between Japan and the UK. Liu et al 7 used DIS-MOD II software to estimate prevalence based on the inpatient data of GPA patients in 100 hospitals across China. They estimated the prevalence of GPA to be 1...

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Impact of renal involvement on survival in ANCA-associated vasculitis

Abstract: Renal involvement was more common in males. BVAS was higher in patients with renal involvement. The mean survival and mortality were similar in patients with or without renal involvement.

Cited by 9 publications

References 23 publications

Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry

Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry

Clinical features and long‐term outcomes of 105 granulomatosis with polyangiitis patients: A single center experience from north India

Is granulomatosis with polyangiitis in Asia different from the West?

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