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

Roya Sherkat; Alireza Rafiei; Vida Homayouni; Elmira Kalantari; Roya Sepahvandi; Sahar Memarmontazarin; Ali Mosayebian

doi:10.18502/ijaai.v21i2.9227

Roya Sherkat Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
Alireza Rafiei Department of Immunology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran
Vida Homayouni Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
Elmira Kalantari Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
Roya Sepahvandi Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
Sahar Memarmontazarin Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
Ali Mosayebian Department of Immunology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran

DOI: https://doi.org/10.18502/ijaai.v21i2.9227

Keywords: Cryopyrin-associated periodic syndromes; Familial Mediterranean fever; Hereditary autoinflammatory diseases

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.