Scleritis in granulomatosis with polyangiitis: clinical study and follow-up of 43 cases / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical characteristics of granulomatosis with polyangiitis (GPA) patients with scleritis.Methods The completed clinical data of 102 GPA patients from March 2012 to July 2016 in Capital Medical University, Tong Ren Hospital were collected. Altogether 43 GPA patients with scleritis were enrolled, and the clinical data were analyzed respectively. The t test, Mann-Whitney U test and x2testwere used to analyze the data. Results The most common ocular manifestation in GPA was scleritis. The incidence of renal damage(37.2% vs 11.9%,x2=9.148,P=0.002),disease activity(BVAS/GPA score 8.9 vs 5.1,Z=7.847, P=0.000),DEI score (7 vs 4,Z=5.919, P=0.000),inflammation index(56 mm/1 h vs 36 mm/1 h, Z=2.456, P=0.016; CRP 51 mg/L vs 8 mg/L, Z=3.748, P=0.000), neutrophil percent (70% vs 61%, t=3.993, P=0.000),complement 3(1.2 g/L vs 1.0 g/L, t=2.652, P=0.009),plasma fibrinogen(3.5 g/L vs 3.3 g/L, t=3.947, P=0.000), D-dimmer (0.5 mg/L FEU vs 0.3 mg/L FEU, Z=2.371, P=0.018) and ANCA positive rate (90.7% vs 57.6%,x2=13.369,P=0.000)were higher in patients with scleritis,as well as a poor prognosis(2009-FFS 0.4 vs 0.1, Z=2.600, P=0.009). However, a lower rate of subglottic involvement was detected in scleritis group (4.7% vs 20.3%, x2=5.169, P=0.023). There were no significant differences between GPA patients with scleritis and episcleritis in clinical feature, disease activity, inflammatory index and prognosis. Aggressive immunosuppres-sive treatment was appropriate for GPA with scleritis. Tumor necrosis factor (TNF)-аinhibitor was an effective treatment. Conclusion GPA with scleritis usually suffered from higher disease activity index,broader DEI,as well as poorer prognosis. Aggressive treatment should be initiated in order to achieve disease remission. Subglottic involvement is less common in patients with scleritis. The phenotype of scleritis is not associated with disease severity.