Chronic recurrent multifocal osteomyelitis in children: nine years' experience at a statewide tertiary paediatric rheumatology referral centre.

OBJECTIVE: To describe the clinical features, management and outcome of 34 children with chronic recurrent multifocal osteomyelitis (CRMO) diagnosed at a single centre over 9 years. METHODS: All children identified with CRMO for the period 2005-13 were identified from a prospectively collected database, with additional data from hospital records. RESULTS: Thirty-four patients, 21 female and 13 male, were identified. The average age at symptom onset was 9.8 years (range 3.8-17.9) and at diagnosis was 10.9 years (range 5.2-18.2), with an average delay in diagnosis of 12 months. Follow-up was 0.3-7.9 years (average 2.1), with 104 individual bony lesions identified, with a median of 3 (range 1-9) per patient. Six patients had unifocal disease. The sites involved included the tibia (n = 19), femur (n = 14), clavicle (n = 12), vertebrae (n = 10) and fibula (n = 8). Approximately half of patients had an inflammatory arthritis at diagnosis, and two-thirds in total eventually developed an arthritis. Pustulosis occurred in eight patients (24%), severe acne in four (12%) and psoriasis in three (9%). NSAIDs were used in 91%, CSs in 82% and MTX in 38%. Two patients were treated with anti-TNF agents. Episodic disease was most common (79%), while 21% had a monophasic pattern. Clinical remission occurred in 94% of children, with prolonged remission in 17%. Seven patients did not require medications for >12 months. CONCLUSION: CRMO is more common than previously recognized, but diagnosis may be delayed. Episodic multifocal disease was most common, but some had unifocal and/or monophasic disease. Most patients responded to NSAIDs and/or intermittent CSs, but many required DMARDs.

Joint Aspiration for Acute Hemarthrosis in Children Receiving Factor VIII Prophylaxis for Severe Hemophilia: 11-year Safety Data.

OBJECTIVE: The aims of this study were (1) to document the prevalence of acute hemarthrosis in a cohort of 46 boys with severe hemophilia A receiving full primary prophylaxis in Western Australia (WA), and (2) to investigate the safety of the WA protocol over 11 years for management of hemarthrosis. METHODS: Case review. The WA protocol involves a pediatric rheumatologist washing out all acute hemarthrosis of large joints promptly and then instilling intraarticular (IA) corticosteroids. RESULTS: This study showed that joint bleeds occurred in 22 boys of 46 (47.8%). In over 11 years, 84 washouts were performed on 32 joints in 22 boys. No adverse events occurred. Fifteen of 22 boys had normal joints with a Hemophilic Joint Health Score = 0. Fifteen boys who had had all hemarthrosis washed out had clinically normal joints (100%). Seven boys had sustained joint damage prior to full instigation of the protocol, each having had documented hemarthrosis without aspiration. Parents needed to understand that joint bleeds constituted an emergency. CONCLUSION: Of our cohort, 47.8% of patients with severe hemophilia receiving prophylaxis developed joint bleeding. The WA protocol is safe. There is evidence suggesting joint outcomes of hemophilic patients having hemarthrosis despite factor VIII prophylaxis may be much improved if there is access to a center using a procedure similar to the WA protocol.

Fevers and the rheumatologist.

Fevers in children are mainly due to infection, malignancy or inflammatory conditions. Rheumatologists have an important role in the care of inflammatory conditions, many of which are associated with fevers. Seven conditions, the hereditary recurrent fever syndromes, have been defined with the presenting symptom of recurring fever, and for which mutation of a single gene has been defined: Chronic infantile neurological articular syndrome (CINCA), Familial cold autoinflammatory syndrome (FACS), Familial Mediterranean fever (FMF), hyperimmunoglobulinemia D (HIDS), Muckle-Wells syndrome (MWS), Pyogenic sterile arthritis and Pyoderma gangrenosum (PAPA) and Tumour necrosis factor receptor-associated periodic syndrome (TRAPS) . These conditions will be discussed in detail in regard to how they fit into the wider picture of pediatric rheumatological conditions, how the diagnoses may be established and the current recommended treatments for each condition.

The place of pediatric rheumatology in India.

Rheumatology is a relatively new specialty in the field of Pediatrics in India where there are only a few centres of excellence. Awareness about these conditions in childhood is lacking and patients are often referred late resulting in poor long term outcomes. This article highlights the needs of the paediatric rheumatology patients which are complex and should encompass both acute and chronic care and also plan a smooth transition to the adult rheumatology world. In the year 2010, attaining remission is the goal for every child with a rheumatologic disorder, which is seldom achieved for the Indian pediatric rheumatologic patient. The article discusses the reasons for poor awareness, the current outcome for these patients and details the burden of disease in India. The health care delivery system and finally the way forward have been discussed.

Epidemiology of the rheumatic diseases of childhood.

Epidemiologic studies of pediatric rheumatologic disease are becoming increasingly focused on subfields of epidemiology. Genetic epidemiology is assuming an ever-greater role in the understanding of disease risks and pathogenesis. Such studies involve major histocompatibility complex, cytokine gene polymorphisms, T cell studies, and microarray-based expression technology. Clinical epidemiology, the study of occurrence and outcomes, increasingly relies on multicenter studies with data improving as study parameters become more standardized. With multicenter studies, there is an increasing trend to study ethnic differences in rheumatologic diseases of children.

Classification of juvenile idiopathic arthritis: should family history be included in the criteria?

OBJECTIVE: (i) To determine the efficacy of the Durban classification for children with juvenile idiopathic arthritis (JIA) where < 5 joints were involved at onset (with systemic arthritis excluded) by determining the proportion of the cohort that proved to be "unclassifiable"; (ii) to define reasons for cases being "unclassifiable," particularly regarding family history; and (iii) to compare the efficacy of a proposed hierarchical system (an unofficial modification of the Durban classification) with the Durban classification, where family history details are included as descriptors, rather than as classification criteria. METHODS: Charts were reviewed of 50 children with fewer than 5 joints involved at presentation for JIA, followed for at least 12 months, with systemic arthritis excluded. Cases were classified according to the EULAR criteria, the Durban criteria, and by a proposed "modified Durban" classification subject to hierarchy, with exclusions in the following order: systemic arthritis, rheumatoid factor (RF) positive arthritis, psoriasis or a combination of dactylitis and psoriatic nail changes (psoriatic arthritis), and HLA-B27 positive arthritis (enthesitis related arthritis), with the remainder of children being classified as having either RF negative polyarthritis or RF negative oligoarthritis, depending on number of joints involved, with additional information noted as descriptors. The "modified Durban" classification was proposed only to stimulate discussion among clinicians. RESULTS: Of 50 children, 56% were "unclassifiable" by the Durban classification, mainly because of inadequate family history despite appropriate questioning. Using the proposed "modified Durban" classification, 2% were "unclassifiable." Family history was classified as inadequate for the following reasons: The parents did not know family history; the child or parent was adopted; the father was unknown or parent died early; parents never attended; extended family had lost communication with parents; or a relative was considered to have psoriasis, but not confirmed by dermatologists. Other reasons for "unclassifiable" included: dermatologists unable to confirm psoriasis; family history of inflammatory bowel disease and sacroiliitis but B27 status unknown; proband B27 negative but family history of B27-related disease; family history of psoriasis, but patient had insufficient criteria for psoriatic arthritis and therefore excluded from oligoarthritis, psoriatic arthritis and other groups. CONCLUSION: (i) The Durban classification showed poor efficacy for JIA where < 5 joints were involved at onset, with more than half the cases being "unclassifiable". (ii) The most common reason was that appropriate family history was not available despite being sought by the clinician. (iii) A proposed hierarchical system, an unofficial modification of the Durban classification, showed good efficacy, with only one of 50 cases being "unclassifiable."

Worldwide prevalence of juvenile arthritis why does it vary so much?

OBJECTIVE: To review epidemiological studies of childhood arthritis from 1966, and to identify possible reasons for the wide-ranging results for both prevalence and incidence of juvenile arthritis (JA). JA is the term used here collectively for juvenile rheumatoid arthritis, juvenile chronic arthritis, or juvenile idiopathic arthritis as defined in the respective published studies. METHODS: A review of 34 epidemiological studies of JA since 1966 was undertaken. RESULTS: Prevalence of JA is reported as 0.07 to 4.01 per 1000 children. Annual incidence is reported as 0.008 to 0.226 per 1000 children. The major factors contributing to differences in estimates include (1) factors due to diagnostic difficulties, to the development of new diagnostic criteria, and to the differing definitions of clinical cases; (2) differences in case ascertainment (community based versus clinical case studies, qualification and experience of study clinicians, definition of study population); (3) factors occurring with the passage of time, i.e., standard of living, health care resources, and increasing knowledge; and (4) small studies and hence more chance fluctuation. The major variation in reported prevalence was due to the difference between true community based studies involving children from within classrooms or homes (and not necessarily previously diagnosed with JA) compared with clinical case studies of children who (by definition) had been previously diagnosed. The highest prevalence was reported for true community based studies. CONCLUSION: Many factors contribute to the discrepancies between reported prevalence and incidence for JA. Studies based truly in the community reported the highest prevalence, as previously undiagnosed cases were included. Future studies involving standardized criteria and standardized case ascertainment done by fully trained clinicians should show greater consistency of results.