Clinical phenotypes and prognosis of IgG4-RD patients with allergy history: A retrospective cohort study

There is a high prevalence of allergic conditions in patients with IgG4-related disease (IgG4-RD), paralleled by shared dysregulation in immune responses. The aim of this study was to explore the clinical features and outcomes of IgG4-RD patients with allergy history.

215 IgG4-RD patients were retrospectively enrolled and clinical features were compared between IgG4-RD patients with and without allergy history. Subsequently, 148 patients who had been followed up for ≥ 1 year were further selected to evaluate the outcomes and risk factors of relapse by conventional COX and Bayesian Cox proportional hazards models.

Among IgG4-RD patients, 81 (37.7%) had allergy history. IgG4-RD patients with allergy history were found to have lower proportion of males, younger age of disease onset, higher peripheral blood eosinophil, more proliferative subtype, less fibrotic subtype, higher incidence of lacrimal gland, parotid gland, submandibular gland involvement, sinusitis and lower incidence of retroperitoneal fibrosis, pancreas, biliary tree involvement. Allergy history was correlated with a shorter relapse-free survival time (HR 95% CI: 1.067-2.604, p = 0.02). More organs involved, higher IgG4-RD responder index (IgG4-RD RI), allergy history, lacrimal gland involvement, sinusitis, retroperitoneal fibrosis and Immunosuppressants (IMs) usage were associated with relapse.

IgG4-RD patients with allergy history have distinctive phenotypes and prognosis. Therefore, differentiated treatment strategies and novel medications are required for them. Key Points • IgG4-RD patients with allergy history exhibit high prevalence of allergic conditions. • Conventional and Bayesian Cox regression models were used to analyze the risk factors of relapse. • Allergy history are correlated with distinctive phenotypes and shorter relapse-free survival time in IgG4-RD.