The French version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report the results of the cross-cultural adaptation and validation into the French language of two health status instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health related quality of life instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. Five hundred children were enrolled including 306 patients with JIA classified into systemic (23%), polyarticular (22%), extended oligoarticular (25%), and persistent oligoarticular (30%) subtypes, and 194 healthy children. Both instruments were reliable with intra-class correlation (ICC) coefficients for the test-retest procedure of 0.91 for the CHAQ, and 0.87 and 0.89 for the physical and psychosocial summary scores of CHQ, respectively. Agreement between parents and children evaluated for the CHAQ was high with an ICC of 0.89 for the disability index; weighted kappa coefficients for the 8 domains ranged from 0.61 to 0.72. Convergent validity was demonstrated by significant correlations with the JIA core set of variables (physician and parent global assessment, scores for active joints and joints with limited range of motion, erythrocyte sedimentation rate) for both instruments. Both CHAQ and CHQ discriminated between healthy and JIA children, but only the disease specific CHAQ questionnaire discriminated clearly between the 4 JIA subtypes. In conclusion, the French versions of the CHAQ and the CHQ are reliable, and valid health assessment questionnaires to be used in children suffering from JIA.

[Forms of polyarthritis in idiopathic juvenile arthritis]. / Formes polyarticulaires des arthrites juvéniles idiopathiques.

DEFINITION: Idiopathic juvenile polyarthritis includes a group of inflammatory diseases that affect at least five joints, either from onset or within the first six months of the disease course in children under 16 years of age. Diagnosis is arrived at by elimination. Besides malignant disease, always to be considered as a differential diagnosis, idiopathic juvenile polyarthritis can be divided into rheumatoid factor (RF)-positive and RF-negative polyarthritis and extended forms of oligoarthritis. RF-NEGATIVE POLYARTHRITIS: Antinuclear antibody (ANA)-positive polyarthritis must be distinguished from extended forms of oligoarthritis which are also ANA-positive. ANA-positive polyarthritis generally begins early, at the age of 2 or 3 years, predominantly in girls. Characteristic torpid uveitis is frequent, requiring regular systematic screening. Joints are minimally painful with symmetrical involvement, usually of the knees and wrists. Progressive joint destruction and/or growth disorders are common. The disease progresses by acute episodes. Nonsteroidal antiinflammatory drugs are used, associated with a specific treatment and local care as needed. General corticosteroids may be required in certain cases but should be avoided if possible. SERONEGATIVE FORMS: Certain patients have no detectable antibodies. These patients generally have fewer ocular problems and less severe joint disease. The treatment is the same as in ANA-positive forms. RF-positive polyarthritis: RF-positive polyarthritis is exceptional and occurs early in young girls. SPECIALIZED CARE: Irrespective of the type of disease, all children with idiopathic juvenile polyarthritis require multidisciplinary specialized care for their chronic and severe, potentially invalidating disease.

[Systemic scleroderma and sarcoidosis: 3 new cases]. / Sclérodermie systémique et sarcoïdose: trois nouveaux cas.

We observed 3 patients with successive scleroderma (SS) and (what is considered to be) sarcoidosis (SA). The diagnosis SS included in the 3 patients: Raynaud's syndrome with pulpal necrosis and capillaritis, sclerodactylia and acro-osteolysis, multiple joint pain and FAN+. Also observed were: esophagus involvement (n = 3), pulmonary artery hypertension (n = 1), telangiectasia (n = 2) and anti-Scl 70 (n = 2). Initially, all patients had restrictive pulmonary disease. SS was diagnosed 5 to 9 years prior to SA in 2 patients. Diagnosis of SA was based on the following arguments: Loëfgren's syndrome with erythema nodosa (n = 1), parotiditis (n = 2), sicca syndrome (n = 2), myalgia (n = 2), joint involvement (n = 2), non-infectious pluropericarditis (n = 2), epitheloid and giant cell granulomas without caseous necrosis (lung = 3, liver = 1, lymph nodes = 1, salivary glands = 1, synovia = 1), negative search for bacilli, elevated conversion enzyme (n = 1) and, in each case, by the lack of any other cause. One patient died from lung cancer and another from respiratory failure. Nome of the patients had primary biliary cirhosis. This rare association between SS and SA demonstrates the confluent limits of certain systemic diseases and raises a difficult problem to differentiate pulmonary involvement in these diseases. The gravity of this localization and the poor sensitivy to corticosteroids.

Comparative study of 6 types of criteria in adult Still's disease.

OBJECTIVE: To compare the sensitivity of 6 sets of criteria proposed for adult Still's disease in published papers in a well defined patient population. METHODS: For inclusion we required a diagnosis of adult Still's disease established by a senior internist or senior rheumatologist. Sensitivity was evaluated in 65 new cases of adult Still's disease from a multicenter study based on signs and symptoms (1) in the first 6 months of illness, (2) in the same period in patients followed more than one year (52 patients), and (3) at any time in patients followed more than one year. RESULTS: The Yamaguchi criteria are most sensitive (93.5%), followed by those of Calabro and Londino. Medsger et al (80.6%), Kahn (69.3%), Reginato (55.2%), and Goldman (43.7%). Sensitivity was high even from the first 6 months, except for the 2 less sensitive criteria. CONCLUSION: Awareness of these discrepancies in adult Still's disease criteria are crucial to ensure comparability of different series of this condition.