Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: a cluster analysis

Mahr, Alfred; Katsahian, Sandrine; Varet, H.; Guillevin, Loı̈c; Hagen, E. Christiaan; Höglund, Peter; Merkel, Peter A.; Pagnoux, Christian; Rasmussen, Niels; Westman, Kerstin; Jayne, David

doi:10.1136/annrheumdis-2012-201750

Cited by 202 publications

(178 citation statements)

References 33 publications

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“…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. [4][5][6][7][8][9] Lung involvement was seen in 68% of our patients which was similar to that noted in European populations. 4,7,8 Americans had a slightly higher lung involvement (75-85%) than both Indians and the Europeans.…”

Section: Discussionsupporting

confidence: 57%

“…[4][5][6][7][8][9] Lung involvement was seen in 68% of our patients which was similar to that noted in European populations. 4,7,8 Americans had a slightly higher lung involvement (75-85%) than both Indians and the Europeans. 5,6 Ocular manifestations were noted in 41% of patients in our study which was similar to that noted in previous studies.…”

Section: Discussionsupporting

confidence: 57%

“…[11][12][13]15 ANCA was negative in 10% of patients in our study, which is similar to that reported from other studies, where ANCA negativity ranged from 10 to 13%. [4][5][6][7][8] Renal involvement was seen in fewer patients in our study population compared to studies from Europe, America and also older studies from India. The more recent Indian study by Kumar et al has also shown that renal involvement is seen in 33% of patients in their cohort.…”

Section: Discussioncontrasting

confidence: 38%

“…GPA seems to affect a relatively younger population in India compared to European and American populations, as the mean age of presentation in our subjects is 40 years which is about a decade earlier than in European and American populations. [4][5][6][7][8] Older Indian studies have noted the mean age of presentation to be between 32 to 42 years, but a recent study documented a mean age of 46 years, which is comparable to that of Western studies. [11][12][13]15 ANCA was negative in 10% of patients in our study, which is similar to that reported from other studies, where ANCA negativity ranged from 10 to 13%.…”

Section: Discussionmentioning

confidence: 66%

“…2,3 Several large case series regarding GPA patients were published from various countries, with the largest series describing 445 GPA patients from Germany. [4][5][6][7][8] The first report of GPA from India was published in 1985 by Kumar et al followed by descriptions of 11 patients by Bambery et al in 1987. 9,10 Since then various case series have been published from India with the largest series of 45 patients being described by Kumar et al [11][12][13] In the present study, we describe the clinical features, organ involvement, histopathology findings, treatment and outcomes of 105 GPA patients treated at our center, a tertiary care hospital in India.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionsupporting

confidence: 57%

Section: Discussionsupporting

confidence: 57%

Section: Discussioncontrasting

confidence: 38%

Section: Discussionmentioning

confidence: 66%

Section: Introductionmentioning

confidence: 99%

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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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Long‐Term Followup of a Multicenter Cohort of 101 Patients With Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss)

Durel

Berthiller

Caboni³

et al. 2016

Arthritis Care & Research

100

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Objective. To assess the long-term outcome in eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). Methods. A total of 101 patients fulfilling the American College of Rheumatology criteria for EGPA were included between 1990 and 2011. Clinical features, antineutrophil cytoplasm autoantibodies (ANCAs), and Five-Factors Score (FFS) were assessed at diagnosis. Overall and cumulative survival rates, relapse-free survival, and sequelae were studied based on ANCA status and FFS.Results. The rate of cardiomyopathy did not differ according to ANCA status. A total of 79.6% of patients achieved first remission, but 81.1% relapsed. ANCA-positive patients did not relapse more frequently but exhibited more severe disease with mononeuritis (P 5 0.0004) and renal involvement (P 5 0.02). Being Italian was the only prognostic factor associated with a higher relapse-free survival (P 5 0.01), thanks to a longer maintenance of immunosuppressive drugs, suggesting the need for prolonged low-dose corticosteroids. Overall, survival reached 93.1% after a median followup of 6 years. No factor was associated with mortality, but patients over age 65 years with cardiomyopathy or ANCA positivity had more serious outcomes. Sequelae affected 83.2% of patients. Ear, nose, and throat (ENT) involvement was a protective factor for renal (P 5 0.04) and cardiac (P 5 0.03) morbidity. ANCA positivity was correlated with chronic kidney disease (P 5 0.03) and chronic neurologic disability (P 5 0.02). Conclusion. The actual challenges of EGPA management concern morbidity prevention and quality of life improvement. Long-term corticosteroid treatment appears to reduce relapse risk. ENT involvement is associated with less renal and cardiac morbidity. ANCA positivity predicts renal and neurologic damage.

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Validation of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis as the Cause of End‐Stage Renal Disease in the US Renal Data System

Cook

Zhang

et al. 2021

ACR Open Rheumatology

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Objective. The objective of this study was to validate the diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) as the primary cause of end-stage renal disease (ESRD) in the US Renal Data System (USRDS).Methods. We identified patients with ESRD in the Mass General Brigham (MGB) health care system who were enrolled in the USRDS. The health records of those with AAV listed as the primary cause of ESRD in the USRDS were reviewed to confirm the diagnosis and estimate positive predictive value (PPV). Sensitivity was estimated by evaluating the primary cause of ESRD listed in the USRDS for patients with ESRD due to AAV in the MGB AAV cohort.Results. We identified 89 MGB patients with ESRD due to AAV in the USRDS. Of these, 85 cases were confirmed to be true cases of AAV (PPV = 94%). Among the patients classified as having AAV, 84 (99%) had an ANCA test, which was predominantly myeloperoxidase/P-ANCA (47 [55%]); 36 (42%) had a renal biopsy, and all biopsies were supportive of the diagnosis. The majority (81 [90%]) was identified as AAV by International Classification of Diseases Ninth Revision or International Classification of Diseases 10th Revision codes for granulomatosis with polyangiitis (446.4 or M313.1). Of the 77 MGB AAV cohort patients with ESRD who were linked to the USRDS, 41 (53%) had AAV listed as the cause of ESRD; in the remainder, ESRD was attributed to nonspecific nephritis.Conclusion. The diagnosis of AAV as the cause of ESRD in the USRDS has a high PPV; sensitivity was moderate. These findings support the continued use of the USRDS to study ESRD due to AAV.

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Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: a cluster analysis

Abstract: This analysis suggests that AAV encompasses five classes associated with different outcomes. As compared with the traditional GPA-MPA separation, this classification system may better reflect the phenotypic spectrum of AAV.

Cited by 202 publications

References 33 publications

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

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

Long‐Term Followup of a Multicenter Cohort of 101 Patients With Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss)

Validation of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis as the Cause of End‐Stage Renal Disease in the US Renal Data System

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