Clinical and Laboratory Parameters of Autoinflammatory Disorders in Single Tertiary Care Center Publisher Pubmed



Sherkat R¹ ; Rafiei A² ; Homayouni V¹ ; Kalantari E¹ ; Sepahvandi R³ ; Memarmontazarin S¹ ; Mosayebian A² Show Affiliations
Source: Iranian Journal of Allergy, Asthma and Immunology Published:2022

Abstract

Autoinflammatory diseases (AIDs) are disorders with an inborn error of innate immunity, characterized by recurrent episodes of fever and inflammatory attacks. The spectrum of AIDs is expanding, but there are no standardized clinical criteria for the diagnosis of the patients. This study aims at establishing the first autoinflammatory registry of an Iranian population focusing on the clinical and laboratory features that may help clinicians for a better understanding and diagnosis of these disorders. Clinical and laboratory characteristics of patients who were clinically and or genetically diagnosed with AIDs were collected for 15 years. The updated version of classification criteria from the Eurofever Registry was used for the clinical diagnosis. Twenty-eight patients (16 males and 12 females) with a mean±SD age of 28.03±14.49 years (from 2 to 68 years) entered this study. About 29% were genetically diagnosed. Familial Mediterranean fever (FMF) was the most common diagnosis of the patients. Fever duration episodes were between 1-10 days. Some of the clinical manifestations from the most to the least common were as follows: arthralgia and arthritis (80%), myalgia (76%), coughs and shortness of breath (68%), fatigue (60%), abdominal pain (56%), increased erythrocyte sedimentation rate(ESR) (48%), and splenomegaly (24%). Here, we presented the most common clinical manifestations of Iranian AIDs who have registered in our AID registry which would be a useful guide for managing the same patients and also designing the future studies. Copyright © 2022 Sherkat et al. Published by Tehran University of Medical Sciences.