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.

The term PERIODIC fever and diseases classified under PERIODIC fever have particular situation in pediatrics. PERIODICity and fever are the hallmark of these SYNDROMES, though there could be other symptoms and signs in each syndrome. On the other hand, most of these SYNDROMES have familial pattern. Since some of these SYNDROMES are common in north-west Iran, the understanding and recognition of these diseases seems to be necessary. Nowadays at least seven PERIODIC fever SYNDROMES have been explained in pediatrics: The first one is FMF and the last and new one is PFAPA. The rest of them are: Hyper IgD, FCAS, Muckel- Wells, CINCA, and TRAPS.

PERIODIC fever SYNDROMES are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. In the first part of this paper, we presented a guideline for approaching patients with PERIODIC fever and reviewed two common disorders with PERIODIC fever in Iranian patients including familial Mediterranean fever (FMF) and PERIODIC fever SYNDROMES except for PERIODIC fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA). In this part, we review other autoinflammatory disorders including hyper IgD, tumor necrosis factor receptor–ASSOCIATED PERIODIC syndrome (TRAPS), CRYOPYRIN ASSOCIATED PERIODIC SYNDROMES, autoinflammatory bone disorders and some other rare autoinflammatory disorders such as Sweet’s and Blau SYNDROMES. In CRYOPYRIN ASSOCIATED PERIODIC SYNDROMES group, we discussed chronic infantile neurologic cutaneous and articular (CINCA) syndrome, Muckle-Wells syndrome and familial cold autoinflammatory syndrome. Autoinflammatory bone disorders are categorized to monogenic disorders such as pyogenic arthritis, pyoderma ;gangraenosum and acne (PAPA) syndrome, the deficiency of interleukine-1 receptor antagonist (DIRA) and Majeed syndrome and polygenic background or sporadic group such as chronic recurrent multifocal osteomyelitis (CRMO) or synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome are classified in sporadic group. Other autoinflammatory SYNDROMES are rare causes of PERIODIC fever in Iranian system registry.

PERIODIC fever SYNDROMES are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. The first manifestation of these disorders are present in childhood and adolescence, but infrequently it may be presented in young and middle ages. Genetic base has been known for all types of PERIODIC fever SYNDROMES except PERIODIC fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA). Common PERIODIC fever disorders are Familial Mediterranean fever (FMF) and PFAPA. In each patient with PERIODIC fever, acquired infection with chronic and PERIODIC nature should be ruled out. It depends on epidemiology of infectious diseases. Some of them such as Familial Mediterranean fever and PFAPA are common in Iran. In Iran and other Middle East countries, brucellosis, malaria and infectious mononucleosis should be considered in differential diagnosis of PERIODIC fever disorders especially with fever and arthritis manifestation. In children, urinary tract infection may be presented as PERIODIC disorder, urine analysis and culture is necessary in each child with PERIODIC symptoms. Some malignancies such as leukemia and tumoral lesions should be excluded in patients with PERIODIC syndrome and weight loss in any age. After excluding infection, malignancy and cyclic neutropenia, FMF and PFAPA are the most common PERIODIC fever disorders. Similar to other countries, Hyper IgD, Chronic Infantile Neurologic Cutaneous and Articular, TRAPS and other auto-inflammatory SYNDROMES are rare causes of PERIODIC fever in Iranian system registry. In part 1 of this paper we reviewed the prevalence of FMF and PFAPA in Iran. In part 2, some uncommon auto-inflammatory disorders such as TRAPS, Hyper IgD sydrome and CRYOPYRIN ASSOCIATED PERIODIC SYNDROMES will be reviewed.