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Objective:To analyze the clinical characteristics of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods:A retrospective study was conducted on AAV patients with long-term follow-up in the rheumatology outpatient clinic of Peking Union Medical College Hospital between February 2015 and February 2022. The demographic characteristics, clinical manifestations, concurrent events, treatment, and prognosis of the three clinical subtypes of AAV were collected and analyzed.Results:There were 71, 45, and 31 cases of granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatous polyangiitis (EGPA), respectively, among 147 patients. The ANCA positivity rates in the three groups were 91.5%, 95.6%, and 19.4% ( χ2=76.68, P<0.01), respectively. The upper respiratory tract and lungs were the most frequently affected organs in GPA and EGPA, and the kidneys and lower respiratory tract were the main organs involved in MPA. In addition, cardiac and neurological involvement and thrombosis rates were significantly higher in EGPA patients than in GPA and MPA (12.9%, 9.7%, 41.9% and 19.4%, respectively; χ2=8.51、7.13、7.54、0.02, P<0.05) .The median follow-up time for the three groups of patients was 43, 28, and 46 months respectively.Relapse was more common in patients with GPA and EGPA (up to 59.2% and 64.5%; χ2=11.26, P=0.004), with the lungs and ENT being the most common relapse organs (GPA of 61.9% and 40.5%, EGPA of 55.0% and 50.0%), the lungs and kidneys were the most common manifestations in MPA relapse (64.3% and 60.0%, respectively). The main therapeutic agents were glucocortoid (95.9%), cyclophosphamide (71.4%), methotrexate (54.4%), tripterygium wilfordiz (34.0%),mycophenolate mofetil (31.3%), azathioprine (29.3%), leflunomide (19.0%), rituximab (19.0%), and tacrolimus/cyclosporine (8.8%). There were 6 deaths (4.1%) occurred during the follow-up period of this study. Conclusion:The clinical features of AAV are similar to those reported in the literature and relapses are common>he vast majority of patients need to be treated with glucocorticoid combined with immunosuppressive agents.
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Objective: To analyze the clinical characteristics and prognosis of patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV). Methods: Clinical data (general information, clinical manifestation, and laboratory indexes) of 168 patients newly diagnosed as AAV between Jul. 2008 and Dec. 2016 were retrospectively analyzed. Patients were followed up for a median period of 12 months (range, 1-96 months). End-point event was death. Results: There were 152 cases of microscopic polyangiitis (MPA), 14 cases of granulomatous polyangiitis (GPA) and 2 cases of eosinophilic granulomatosis with polyangiitis (EGPA). MPO-ANCA was positive in 145 (86.3%) patients and PR3-ANCA was positive in 20 (11.9%) patients. Forty-nine patients died, 91 patients survived, and 28 patients lost their visits. Interstitial lung disease and renal involvement were more common in MPA patients as compared with GPA patients (50.7% [77/152] vs 14.3% [2/14], 78.9% [120/152] vs 50.0% [7/14], all P<0.05). Estimated glomerular filtration rate (eGFR) in MPA patients was significantly lower than that in GPA patients (14.23 [7.27, 71.49] mL/[min · 1.73 m2] vs 104.08 [16.61, 135.72] mL/[min · 1.73 m2], P<0.05). Compared with PR3-ANCA positive patients, MPO-ANCA positive patients was more elder ([64.01 ± 10.62] years vs [50.50±16.88] years), had more renal involvement (77.9% [113/145] vs 50.0% [10/20]) and lower eGFR (19.00 [9.40, 42.85] mL/[min · 1.73 m2] vs 149.40 [86.75, 249.45] mL/[min · 1.73 m2], all P<0.05). Compared with the survivals, the dead patients were older ([67.45±10.61] years vs [61.98 ± 12.52] years), had higher incidence of interstitial lung disease (59.2% [29/49] vs 41.8% [38/91]), higher Birmingham vasculitis activity score (BVAS) (18.53 ± 8.02 vs 13.68 ± 5.98), lower eGFR (8.58 [5.73, 22.07] mL/[min · 1.73 m2] vs 45.15 [11.54, 120.79] mL/[min · 1.73 m2], lower blood sodium concentration (137.00 [134.00, 140.00] mmol/L vs 139.00 [136.00, 141.00] mmol/L), lower albumin level ([28.41 ± 5.24] g/L vs [31.92 ± 5.91] g/L), and higher serum D-dimer level (2.84 [1.20, 6.28] mg/L vs 2.24 [0.80, 3.69] mg/L) (all P<0.05). Multivariate Cox proportional regression analysis showed that age, eGFR, serum albumin level and BVAS were independent influence factors of death (hazard ratio =1.058, 0.987, 0.932, and 1.086, all P<0.05). Conclusion: The clinical manifestations of AAV are mostly pulmonary and renal involvement. Age, eGFR, serum albumin level and BVAS are independent risk factors of death in AAV patients. Interstitial lung disease, high level of D-dimer and hyponatremia may be associated with prognosis of AAV.
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Granulomatous polyangiitis is a systemic disease that may affect any organ, with a predilection for the upper respiratory tract, lungs and kidneys. This article aims to report a case of a patient with atypical nodular scleritis as the initial manifestation of granulomatous polyangiitis (Wegener), mimicking a case of tuberculosis. The patient presented ocular hyperemia and lower progressive visual acuity for 1.5 years, followed by eye pain for two months. The patient had subpleural nodules with soft tissue density, increased pulmonary lymph nodes and discrete bilateral pleural thickening, with negative alcohol-resistant acid bacilli (BAAR). The histological diagnosis revealed a granulomatous vasculitis suggestive of non-infectious vasculitis (granulomatous polyangiitis). Cyclophosphamide pulse therapy was initiated.
Poliangiite granulomatosa é uma doença sistêmica que afeta qualquer órgão, com predileção pelo trato respiratório superior, pulmões e rins. Este artigo tem como objetivo relatar um caso atípico de uma paciente com esclerite nodular como manifestação inicial da poliangiite granulomatosa (Wegener), mimetizando um quadro de tuberculose. A paciente apresentou hiperemia ocular e baixa acuidade visual progressiva por 1,5 anos, seguido por dor ocular por dois meses. A paciente possuía nódulos subpleurais com densidade de partes moles, linfonodomegalia em janela aorto-pulmonar e espessamento pleural bilateral discreto, negativo para bacilos álcool-ácido resistentes (BAAR). O diagnóstico histológico revelou uma vasculite granulomatosa sugestiva de vasculite não infecciosa (poliangiite granulomatosa). Foi iniciada pulsoterapia com ciclofosfamida.