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Granulomatosis with polyangiitis: clinical course and outcome of 60 patients from a single center in South India.
- Source :
-
Clinical and experimental medicine [Clin Exp Med] 2018 Aug; Vol. 18 (3), pp. 347-353. Date of Electronic Publication: 2018 Feb 28. - Publication Year :
- 2018
-
Abstract
- Granulomatosis with polyangiitis (GPA) previously known as Wegener's granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study. Disease activity and damage were assessed by Birmingham Vasculitis Activity Score v. 3 (BVAS v. 3) and Vasculitis Damage Index (VDI), respectively. Relapses were defined as recurrence of GPA of sufficient severity to require treatment or increase in the dose of treatment on a patient who was previously stable. Out of 60 patients, initial BVAS evaluation showed that 57 (95%) patients had severe disease and 3 (5%) patients had limited disease where median BVAS was 21.5 (range 17-44). Follow-up BVAS evaluation for severe disease showed that 13 (22.8%) patients continued with severe disease of which 9 patients did not survive, 24 (42.3%) had remission, 11 (19.2%) had persistent disease, and 9 (15.7%) were lost to follow-up. The mean VDI score was 2.5 ± 2. Renal involvement was established in 42 (70%) patients. Upper and lower respiratory involvement was seen in 38 (63%) patients. Nervous system involvement was noted in the 15 (25%) patients. Articular manifestations were seen in 16 (27%) patients. Diverse clinical manifestation delay early diagnosis and treatment of this potentially treatable vasculitis. Focused approach could expedite early diagnosis and can reduce the mortality.
- Subjects :
- Adolescent
Adult
Aged
Aged, 80 and over
Blood Vessels drug effects
Blood Vessels immunology
Cyclophosphamide therapeutic use
Delayed Diagnosis
Female
Granulomatosis with Polyangiitis diagnosis
Granulomatosis with Polyangiitis drug therapy
Granulomatosis with Polyangiitis immunology
Humans
Hypertension diagnosis
Hypertension drug therapy
Hypertension immunology
Immunoglobulins, Intravenous therapeutic use
India
Kidney drug effects
Kidney immunology
Lung drug effects
Lung immunology
Male
Middle Aged
Nervous System drug effects
Nervous System immunology
Prednisolone therapeutic use
Recurrence
Retrospective Studies
Severity of Illness Index
Survival Analysis
Treatment Outcome
Blood Vessels pathology
Granulomatosis with Polyangiitis pathology
Hypertension pathology
Kidney pathology
Lung pathology
Nervous System pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1591-9528
- Volume :
- 18
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Clinical and experimental medicine
- Publication Type :
- Academic Journal
- Accession number :
- 29492716
- Full Text :
- https://doi.org/10.1007/s10238-018-0492-7