Outcome of 27 Patients with Granulomatosis with Polyangiitis (Wegener's Granulomatosis) Patients in Iran

Background: Granulomatosis with polyangiitis (GPA) which also known as Wegener's Granulomatosis is a rare disease. GPA is a heterogeneous disease that involves different organs with variable severities and a relapsing course. Objective: This study aimed to investigate the demographic data, clinical and laboratory presentations, prevalence of involvement in various organs, therapeutic regimens, response to treatment and relapse rate in Granulomatosis with polyangiitis (GPA) patients in Yazd Province, central Iran. Method: This is a retrospective descriptive study, including 27 patients with definite diagnosis of GPA from 2005 to 2014. Mean duration of follow up was 5 years. Results: Patients mean age was 35.42 years and male/female ratio 1.45. Initially involved organs were lower respiratory tract (40.7%), musculoskeletal system (33.3%) and upper respiratory tract (25.9%). In course of disease upper respiratory tract (74.7%), renal system (66.7%) and lower respiratory tract (62.9%) were the most involved organs. Cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCA) was positive in 88.8% and perinuclear anti-neutrophilic cytoplasmic antibodies (pANCA) in 18.51% of patients. Erythrocyte sedimentation rate (ESR) was high in 88.8%. Therapeutic regimens included prednisolone and cyclophosphamide were prescribed for induction in most patients (88.8%). Three patients were treated with plasmapheresis due to diffuse alveolar hemorrhage (DAH), and three with Rituximab due to refractory to conventional treatment, successfully. Complete remission was seen in 44.4% and partial remission in 33.3% patients after three months, also in 74.07% and 14.81% of cases after 12 months. Relapse occurred in 66.7% of cases. There was no significant correlation between cANCA and clinical manifestation or outcome in this study. (P value>0.05). Conclusion: In our patients, the involvement of lower respiratory tract was more than the upper part as presentation. However, during the course of disease involvement of the upper respiratory tract was observed more. Administration of rituximab was successful in patients with refractory to conventional therapy.