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,
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