ANCA-Associated Vasculitis Clinical Presentation and Clinical Predictors of Relapse in Saudi Arabia

Abstract

Background: Anti-neutrophil cytoplasm antibodies-associated vasculitis (AAV) is a rare autoimmune condition with high-relapsing rate and incidence of complications, resulting in increased morbidity and mortality. Characters of patients with anti-neutrophil cytoplasm antibodies-associated vasculitis in Saudi Arabia require further exploration. Objective: To evaluate the clinical profile, relapse rate and disease-related complications among patients with AAV at a tertiary hospital in Saudi Arabia. To estimate the role of BVAS score at the time of presentation in predicting relapse during the disease course. Design and Setting: This retrospective cohort study was performed through data collection from patients' records who had AAV, who visited the rheumatology clinic. The collected data involved the demographics of patients and their investigations, medications, and outcomes of treatment. Statistical analysis was executed through SPSS version 26. Results: Fifty-two patients were eligible for inclusion, while 48 patients were analyzed because of missing data. Females represented 60.4%. Half of the patients were more than 50 years old, and 68.8% had comorbidities. As for diagnosis, 62.5% had granulomatosis with polyangiitis, 25% had eosinophilic granulomatosis with polyangiitis, and 12.5% had microscopic polyangiitis. The rate of relapse was 31.3%, while the remission rate was 68.8%. Additionally, 66.7% had lower respiratory involvement, and 43.8% had renal involvement. More than half of the patients had BVAS score below 14.5 points. The study did not explore a positive correlation between the disease relapse and high BVAS at the first presentation. Conclusion: Early prediction of relapse and such intervention is of paramount importance in order to avoid accrual of organ damage with treatments that prevent further relapses. BVAS score was not found to be a potential predictor in our study. Future studies are highly endorsed, with prospective design and large sample size to achieve statistical significance for the incidence of relapses and complications.

Anti-neutrophil cytoplasmic antibody associated vasculitis following rituximab: Outcomes of 50 patients in a tertiary single centre

“…Other studies have reported similar results. 29,30 Pulmonary manifestations were common in our cohort of patients with GPA and MPA, similar to previous studies. 29,30 Due to the high percentage of patients with lower lung involvement (77.8%), various complications were identified, with infection (10%) being the most common.…”

“…Pulmonary manifestations were common in our cohort of patients with GPA and MPA, similar to previous studies. 29,30 Due to the high percentage of patients with lower lung involvement (77.8%), various complications were identified, with infection (10%) being the most common. While pulmonary manifestations were slightly more common in our patients with GPA, more serious respiratory conditions were present in our patients with MPA.…”

COVID-induced pulmonary renal syndrome with severe intra-alveolar hemorrhage

“…Antineutrophil cytoplasmic antibody mediated vasculitis includes several subtypes of diseases including Wegner's granulomatosis where the C-ANCA or anti-PR3 is positive in around 90% of the cases. The other one is the MPA or microscopic polyangiitis (Churg -Strauss disease) in which the myeloperoxidase (MPO) antibody is positive with a positive perinuclear antibody P-ANCA positive pattern [6].…”

Common clinical pattern of antineutrophil cytoplasmic antibody -associated vasculitis

“…3 These disorders tend to occur equally in both genders, with a peak age between 65 and 74 years at diagnosis. 4 Individuals diagnosed with AAV can present with severe clinical symptoms that may threaten their lives, for example, alveolar hemorrhage and rapidly progressive glomerulonephritis, while other patients may complain of a less severe clinical picture involving any part of the upper respiratory tract. 5 Two fluorescence patterns of ANCAs distinguished by indirect immunofluorescence (IIF) have been associated with AAV: the cytoplasmic staining pattern (C-ANCA) and perinuclear staining pattern (P-ANCA).…”

“…14 The characteristics of patients with AAV in Saudi Arabia require further exploration. 4 Numerous studies have been conducted on AAV in Saudi Arabia, many of which have explored the manifestations of these disorders. 15 Research into these disorders is not common; as such, we sought to understand the most common type and clinical manifestations of AAV and to gain additional background knowledge to publish more studies in this field.…”