Inflammatory arthritis in children with osteochondrodysplasias.

Osteochondrodysplasias are a heterogeneous group of genetic skeletal dysplasias. Patients with these diseases commonly develop an early degenerative arthritis or osteoarthritis. Occasional observations of inflammatory arthritis have been made in this population but such observations are based on clinical grounds alone without confirmatory imaging studies. Four patients followed up in a paediatric rheumatology clinic with three different skeletal dysplasias, who had both clinical and radiological evidence of an inflammatory arthritis and coexistent degenerative arthritis, are described.

Health assessment in the rheumatic diseases of childhood.

Assessment of children and adults with rheumatic diseases, both in clinical practice and controlled clinical trials in rheumatology, has traditionally focused on the measurement of disease activity. More recently emphasis has been placed on the need to incorporate estimates of physical, social, and mental functioning into health assessment. Thus there has been a tremendous growth in the development of measurement instruments that evaluate health status, functional status, disability, and quality of life. This type of measurement has become essential, particularly for clinical trials in adults with rheumatic diseases, for which the AIMS (Arthritis Impact Measurement Scales), the HAQ (Health Assessment Questionnaire), and the MACTAR (McMaster-Toronto arthritis) patient preference questionnaire have been the most widely used. In the past few years, similar measures have been developed for application in children with rheumatic diseases. These include the CHAIMS (Childhood Arthritis Impact Measurement Scales), the CHAQ (Childhood Health Assessment Questionnaire), the JAFAR (Juvenile Arthritis Functional Assessment Report), the JASI (Juvenile Arthritis Self-report Index), the JAQQ (Juvenile Arthritis Quality of Life Questionnaire), and the CAHP (Childhood Arthritis Health Profile). In this review, the development and measurement properties of these childhood instruments are discussed, with particular emphasis on their potential roles as supported by recent literature.

The Juvenile Arthritis Quality of Life Questionnaire--development of a new responsive index for juvenile rheumatoid arthritis and juvenile spondyloarthritides.

OBJECTIVE: To develop a disease specific measure of quality of life for application in children with juvenile rheumatoid arthritis and juvenile spondyloarthritides-the Juvenile Arthritis Quality of Life Questionnaire (JAQQ). METHODS: Patients and their parents were interviewed by a trained interviewer using a questionnaire focusing on physical function, psychosocial function, and general symptoms to determine the most appropriate items to include in the JAQQ. Respondents volunteered items and scored them for frequency of occurrence and importance. Items so generated were scored by a panel of experts for potential responsiveness and categorized into dimensions. Item number was reduced using this scoring system. The product was then pretested to confirm its construct validity and responsiveness. Thereafter, it was distributed to clinical experts to establish face and content validity. RESULTS: 91 patients, mean age 10.35 years (range 1.25-18.0), mean disease duration 3.99 years, and their parents were included in the interview process. 220 items generated were ultimately reduced to 85. Pretesting this version of the instrument in a further 30 patients showed it to have construct validity and responsiveness and led to a further reduction in items to 74, distributed in 4 dimensions: gross motor function (17 items), fine motor function (16 items), psychosocial function (22 items), and general symptoms (19 items). Face and content validity were established in 20 clinicians. Scaling was by 7 point Likert scale to enhance responsiveness. English and French versions were developed. CONCLUSION: The JAQQ measures physical and psychosocial function and an array of general symptoms. Preliminary data suggest it is valid and responsive and thus might have potential in clinical trials.

Herpes viruses in multicase families with rheumatoid arthritis.

OBJECTIVE: To investigate the rate and extent of infection by Epstein-Barr virus (EBV), cytomegalovirus, and herpesvirus 6 in families (affected and nonaffected members) with multiple cases of rheumatoid arthritis (RA). METHODS: Viral DNA was detected by polymerase chain amplification in cells from saliva and peripheral blood. Human leukocyte antigen pedigrees were characterized. RESULTS: Viral DNA, particularly EBV, was detected in increased frequency (p = 0.029) in the patients with RA compare to their nonaffected relatives. CONCLUSION: We suggest that in RA multicase families, increased frequency of viral infection is likely a consequence of the disease state and/or due to gene(s) as yet unidentified.

Detection of cytomegalovirus, Epstein-Barr virus and herpes virus-6 in patients with rheumatoid arthritis with or without Sjögren's syndrome.

The frequency of latent viral infection by cytomegalovirus (CMV), Epstein-Barr virus (EBV) and herpes virus-6 (HHV-6) was investigated in patients with RA with or without Sjögren's syndrome (SS) and in normal controls. Virus presence was determined by polymerase chain amplification of DNA isolated from peripheral blood mononuclear or polymorphonuclear cells and/or saliva-derived mononuclear/epithelial cells. Anti-viral antibodies and autoantibodies were also assayed. Patients with RA both with and without SS were found to have a significantly increased frequency of latent viral infection (two-fold higher, P = 0.035 for EBV and seven-fold higher, P = 0.018 for HHV-6) compared to normal controls but only in cells isolated from saliva. The increased frequency of virally infected cells from the saliva of patients with RA, regardless of the SS status, when compared to normal controls may reflect the ongoing inflammatory process, the impact of therapy and/or a less effective local immune responsiveness.

Level of agreement between parents and children in rating dysfunction in juvenile rheumatoid arthritis and juvenile spondyloarthritides.

OBJECTIVE: To determine the level of agreement between parents and children in rating dysfunction in juvenile rheumatoid arthritis (JRA) and juvenile spondyloarthritides. METHODS: We conducted separate interviews (parents and patients) of children > or = 9 years of age with JRA and spondyloarthritis attending our clinic. A questionnaire consisting of 100 probes (57 physical activities, 26 psychosocial issues and 17 general symptoms) was used. Probes were scored by respondents on a 6-point scale for their frequency of occurrence (all probes) and importance to the patient (physical activities, only), giving a total of 157 scores. Forty patients were included. Level of agreement between patients and parents was determined by weighted kappa (kappa). RESULTS: Mean kappa for all scores was 0.60; gross motor 0.51, fine motor 0.64, psychosocial 0.56 and general symptoms 0.64. One hundred and forty scores (89%) showed at least moderate agreement. CONCLUSIONS: Our data confirm that there is good parent-child agreement on the level of dysfunction of children > or = 9 years of age with JRA and spondyloarthritis. This level of agreement extends over a wide range of general symptoms and includes physical and psychosocial function. Thus both the parent and the child are reliable informants on the impact of chronic arthritis on the quality of life of affected children and either can be used for this type of assessment.

The utility of the arthritis impact measurement scales for patients with psoriatic arthritis.

The Arthritis Impact Measurement Scales (AIMS) consists of 9 scales that measure physical function, pain and psychosocial function. It has been validated for use in various forms of arthritis, but not in psoriatic arthritis (PsA). The AIMS was administered to 145 patients attending our PsA clinic. We carried out simultaneous assessment of clinical measures of function, measures of disease activity, and measures of disease severity. Most scales of physical function were moderately to highly correlated with clinical measures of function (r = 0.33-0.57; p = 0.0001), measures of disease activity (r = 0.24-0.53, p = 0.003-0.0001), and measures of disease severity (r = 0.23-0.6; p = 0.02-0.0001). The pain scale was highly correlated with clinical measures of function and measures of disease activity (r = 0.38-0.58; p = 0.0001) but not with measures of disease severity. Of the psychosocial scales, the depression scale was moderately correlated with clinical measures of function (r = 0.27-0.3; p = 0.001-0.0001). Our data suggest that the physical function and pain scales are good indicators of overall function and disease activity and are valid for use in PsA.

Infection and disease activity in systemic lupus erythematosus: a review of hospitalized patients.

Infection is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). A review of all SLE admissions to our hospital during a 5-year period was conducted to determine the rate and nature of infection, and its association with overall disease activity, measured by the SLE Disease Activity Index (SLEDAI). Eighty-one patients (79 women, 2 men) were admitted for a total of 2,738 days (176 admissions). There were 53 proven infections, giving an infection rate of 1.94/100 hospital days. Twenty-three (43.4%) of these were major infections (requiring IV antibiotics). Two of 3 deaths were due to septicemia. By logistic regression analysis, infection was significantly associated with disease activity (p = 0.005), but not with disease duration or prednisone dosage. Our data confirm that infection is common in hospitalized patients with SLE, is associated with overall disease activity independent of prednisone dose, and causes significant mortality. These facts should be borne in mind when hospitalization is considered for patients with SLE.