Periodic fever: a review on clinical, management and guideline for Iranian patients - part II

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

[Epidemiologic investigation of juvenile rheumatoid arthritis: a ten-year study]

Background: Juvenile Rheumatoid Arthritis [JRA] is the most common chronic pediat-ric rheumatologic disease. There is a wide range of variation in disease subgroup distribution and evolution according to different populations, environments and genetic predisposition. This study was designed to evaluate epidemiologic characteristics of JRA in Iran

Methods: A cross-sectional studies was performed on children suffering from JRA according to American College of Rheumatology during 10 year [2002-2011]

Patients' data was recorded in hospital questionnaires; Patients' age at the first presentation and time of diagnosis, sex, ethnicity, early clinical and constitutional presentations, joint involvement pattern and presence of associated diseases were evaluated

Results: In this study, 171 children [91 boys and 80 girls] were enrolled. The mean age of patients was 5.2 +/- 3.5 years at the time of presentation and 5.7+/-3.6 years when the diagnosis was confirmed

The most common type of disease was polyarticular arthritis [41.9%]

The prevalence of pauciarticular and systemic onset subtype were 31.6% and 19.3%, respectively

According to ethnicity, the most common incidence was seen in Pars and Turk populations respectively

The most common constitutional presentation was fever

Fever and morning stiffness was significantly higher in polyarticular patients. Extra articular manifestation was seen in 40.4% patients. Among extra-articular manifestations, skin involvement was seen more than others

There was no correlation between sex and type of chronic arthritis. Uveitis was detected in 5.8% of our cases

The mean age was significantly higher in polyarticular group [6.04 year] in comparison to pauciarticular and systemic group [4.4 year in both groups] [P= 0.005]

There was no correlation between sex and type of chronic arthritis

Conclusion: The prevalence of JRA subgroups was different in Iran comparing with western countries and polyarticular disease was the most common subgroup in this study. Uveitis was less common in this study in comparison to studies in other countries. There is no sex predilection in each subgroup of JR

Periodic fever: a review on clinical, management and guideline for Iranian patients - part I

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

Relapsing polychondritis: a case report

Relapsing Polychondritis [RP] is an uncommon inflammatory disorder of unknown cause characterized by an episodic and progressive course affecting predominantly the cartilage of the ears, nose and laryngotracheobronchial tree. It has seldom been described in children. We report on a 2-year old girl with a preliminary diagnosis of Juvenile Chronic Arthritis [JCA], when she was 1 year old. She was treated with Non Steroidal Anti-inflammatory Drugs [NSAIDs] and oral steroids. Six months later her polyarthritis relapsed and she had additional bilateral auricular chondritis; at this time RP was diagnosed due to the appearance of criteria of McAdam-Michet et al. RP should be considered in differential diagnosis of JCA. Steroids and systemic immunosuppressants can be used to treat the RP

Evaluation of laboratory findings in children with wilson's disease in Emam Khomeini hospital during 1994-2003

Wilson's disease is a rare but treatable condition with variable clinical presentations. Its diagnosis depends on a combination of clinical and laboratory findings. We evaluated the clinical and laboratory findings in children with Wilson's disease [WD]. Twenty-seven children [4-14 years, 59.2% male, 40.7% female] with confirmed WD were evaluated between 1994 and 2003 at Imam Khomeini Hospital. Seventeen patients [64%] presented with liver abnormalities, 3[11%] with neurological features, 3[11%] with fulminant hepatic failure and 4[14%] were asymptomatic siblings of patients with WD. The presence of Kayser Fleischer rings, high urine copper excretion, low ceruloplasmin level, and elevated liver copper concentration were detected in 51.8%, 77.7%, 92.5% and 72.7% of patients respectively. We emphasize on clinical findings together with one or more laboratory findings as a diagnostic guide in WD and also recommend evaluation of serum ceruloplasmin level and 24 hour urine copper excretion particularly when liver biopsy may not be obtained