Factors affecting relapse in patients with Granulomatosis Polyangiitis: a single-center retrospective cohort study

Abstract

Background and aim This study aimed to determine the frequency of relapse, the risk factors for relapse, and the correlation of relapse with immunosuppressive regimens in patients with granulomatosis polyangiitis (GPA). Materials and methods The demographic characteristics, the clinical, laboratory, and radiological findings, the immunosuppressive treatment regimens, and the remission and relapse rates of 50 patients with GPA were obtained retrospectively from medical records. Results : The mean relapse-free survival rates at years 1, 3, and 5 were 82%, 60%, and 50%, respectively. Increased relapse rates were observed in patients who had cavitary lung lesions (52.2% vs. 22.2%, p = 0.04) and in those who had elevated serum creatinine levels (1.8 vs. 0.9, p = 0.00). The patients received two different types of remission induction therapies; 36% of them received the combination therapy involving cyclophosphamide (CYC) and rituximab (RTX), and 62% received CYC alone. Relapse was observed in 22.3% of the patients who received the combination remission induction therapy and in 61.3% of the patients who received CYC alone (P = 0.003). Conclusion An increased risk of relapse was observed in patients who had cavitary lung lesions and in those who had elevated serum creatinine levels. The combined use of RTX and CYC for the remission therapy in GPA reduced the relapse rates compared with the use of CYC alone

Acute Pancreatitis as a First Presentation of Granulomatosis With Polyangiitis

“…A high clinical suspicion allows for early diagnosis and timely treatment with immunosuppressive therapy which achieves remission in 85%–90% of patients and increases survival. 11 , 12 This knowledge is important for clinicians to keep a broad differential and consider vasculitides in clinical practice for prompt diagnosis and best patient outcomes.…”

Unexpected Relapse: Insights Into Granulomatosis With Polyangiitis

“…The presentation of GPA typically involves the upper respiratory tract, with 90% of patients complaining of nasal ulcerations, nasal and sinus pain, purulent discharge, and epistaxis, among other conditions [ 7 ]. Multiple case reports also mention ocular and otic involvement as the presenting symptoms of GPA [ 6 ]. Pulmonary parenchymal or airway involvement varies significantly from hoarseness to alveolar hemorrhage [ 8 ].…”

“…The incidence of GPA relapse is estimated to be roughly 13% within 36 months [3]. Many utilize the French vasculitis relapse score at the time of diagnosis to assess the relapse risk; however, the prevalence of relapse varies widely in the documented literature [1][2][3][4][5][6]. This is important as the survival rate of relapse-free patients over a nine-year span is 25%, while patients who experience relapse with severe renal involvement have a significantly elevated rate of mortality [7].…”