Clinical Featuresof IgG4-related Disease

Abstract

BackgroundIgG4-related disease (IgG4-RD) is a rare fibro-inflammatory disease, which is prone to missed diagnosis or misdiagnosis due to nonspecific clinical manifestations.ObjectiveTo analyze the clinical characteristics of IgG4-related disease (IgG4-RD) .MethodsA retrospective design was adopted. Participants were 106 IgG4-RD patients recruited from the First Affiliated Hospital of Zhengzhou University from January 2015 to September 2019. Their clinical data were collected, including demographics, results of serological examinations, imaging examinations, histopathological examinations and treatment status. Data of follow-up conducted by telephone or checking medical history data as of January 1, 2020 were also collected.ResultsOf the 106 cases, 69 were male and 37 were female, with a mean age of onset of (54.6±13.1) years. The most common organs involved were lymph nodes (67.0%) , salivary glands (37.7%) , and pancreas (35.8%) . Single organ involvementoccurred in 17 patients (16.0%) . The median number of involved organs was 3 (2, 4) . Women had higher incidence of salivary gland involvement, lacrimal gland involvement as well as lower incidence of pancreatic involvement than men did (P<0.05) . Serum IgG4≥1.35 g/L was found in 96.1% (99/103) of the patients, and IgG4≥2.7 g/L was found in 79.6% (82/103) of the patients. Compared with those with IgG4<2.7 g/L, patients with serum IgG4≥2.7 g/L had higher RI value, serum eosinophil count, IgG concentration, and IgG2 concentration, and were more prone to hypocomplementemia as well as multiple organ involvement (P<0.05) . Patients with pancreas and kidney involvement were more likely to develop hypocomplementemia, whereas eosinophilia and multiple organ involvement were more common in patients with salivary/lacrimal gland involvement (P<0.05) . The clinical remission rate was 97.2% in 88 patients treated with glucocorticoidalone or glucocorticoidcombined with immunosuppressive agents. Eighty-six cases were followed up, and 32.6% of them (28/86) experienced recurrence.ConclusionThe clinical characteristics of IgG4-RD may be differed by sex and organsinvolved. Elevated serum IgG4 may represent higher disease activity. Timely use of glucocorticoid with immunosuppressive therapy is effective.

Keywords

• |igg4-related disease|clinical features|diagnosis|hormone replacement therapy