Multiple nodules in juvenile chronic polyarthritis.

108

Objective. To describe mortality rates, causes of death, and potential mortality risk factors in pediatric rheumatic diseases in the US.Methods. We used the Indianapolis Pediatric Rheumatology Disease Registry, which includes 49,023 patients from 62 centers who were newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Deaths were confirmed by death certificates, referring physicians, and medical records. Causes of death were derived by chart review or from the death certificate. Standardized mortality ratios (SMRs) and 95% confidence intervals (95% CIs) were determined.Results. After excluding patients with malignancy, 110 deaths among 48,885 patients (0.23%) were confirmed. Patients had been followed up for a mean ؎ SD of 7.9 ؎ 2.7 years. The SMR of the entire cohort was significantly decreased (0.65 [95% CI 0.53-0.78]), with differences in patients followed up for >9 years. The SMR was significantly greater for systemic lupus erythematosus (3.06 [95% CI 1.78-4.90]) and dermatomyositis (2.64 [95% CI 0.86-6.17]) but not for systemic juvenile rheumatoid arthritis (1.8 [95% CI 0.66-3.92]). The SMR was significantly decreased in pain syndromes (0.41 [95% CI 0.21-0.72]). Causes of death were related to the rheumatic diagnosis (including complications) in 39 patients (35%), treatment complications in 11 (10%), non-natural causes in 25 (23%), background disease in 23 (21%), and were unknown in 12 patients (11%). Rheumatic diagnoses, age at diagnosis, sex, and early use of systemic steroids and methotrexate were significantly associated with the risk of death.Conclusion. Our findings indicate that the overall mortality rate for pediatric rheumatic diseases was not increased. Even for the diseases and conditions associated with increased mortality, mortality rates were significantly lower than those reported in previous studies.The practice of pediatric rheumatology includes more than 170 conditions, both inflammatory and noninflammatory (1). Approximately 3 in 1,000 children have a rheumatic condition (1). While we tend to study pediatric rheumatology outcomes in terms of remission versus active disease, organ and radiologic damage, function, and quality of life, there is a small but significant increase in mortality rates among these patients. An increased rate of mortality has been found in juvenile rheumatoid arthritis (JRA) (2-12), childhood systemic lupus erythematosus (SLE) (13-21), dermatomyositis (DM) (22-25), various vasculitides (26-30), and systemic sclerosis (SSc) (31-33).However, most of those studies were of relatively small cohorts, reported mortality outcomes only on specific diseases, had a followup time of Ͻ10 years, and were conducted prior to the 1990s, when new therapies were developed. Even larger studies were flawed; most were based on physician surveys and questionnaires with no strategies to verify response accuracy (4,8). The diagnoses in studies of national cohorts were usually not assigned by pediatric rheumatologists (...

Section: Discussioncontrasting

confidence: 94%

mentioning

confidence: 99%

Mortality outcomes in pediatric rheumatology in the US

Hashkes

Wright

Lauer

et al. 2010

108

“…T lymphocytes and plasma cells are the predominant cells in situ in the inflammatory synovial membrane in both RA and JRA (5,(18)(19)(20)(21)(22)(23). According to our findings, recent-onset JRA differs from chronic JRA both in the intensity of the mononuclear cell infiltration and in the composition of the infiltrates.…”

Section: Discussionsupporting

confidence: 54%

The value of biopsy in patients with monarticular juvenile rheumatoid arthritis of recent onset

Konttinen

Bergroth

Kunnamo

et al. 1986

The histopathology of arthroscopic biopsy material from the knees of 8 patients with monarticular juvenile rheumatoid arthritis (JRA) of recent onset and of 4 control patients was examined using a histochemical method for acid a-naphthyl acetate esterase and an avidin-biotin-peroxidase complex method for different cell subtype-specific surface antigens. According to results of our prospective, single-blind study, nonspecific synovitis was observed in those biopsy samples obtained early in the course of disease. The samples were also characterized by cellular changes that are quite distinct from those described in patients with chronic rheumatoid synovitis. JRA must be considered the cause of symptoms if no orthopedic or infectious disease is found at arthroscopy in children with monarticular symptoms of recent onset and if nonspecific synovitis is observed in the histopathologic specimen. This pathologic description, however, does not correspond to that of classic rheumatoid synovitis. In our studies, we found that

“…In adult RA, rheumatoid nodules are classically detected in patients with severe active disease and are associated with other extraarticular manifestations (1). In JRA, rheumatoid nodules are rarely observed and are usually restricted to patients with polyarticular-onset, RF-positive disease without other extraarticular findings (2). It is well known that SAARDs may accelerate the appearance and pro- gression of nodules in RA patients, and MTX is the SAARD most frequently associated with nodule progression (10)(11)(12)(13).…”

Section: Discussionmentioning

confidence: 99%

“…In juvenile rheumatoid arthritis (JRA), rheumatoid nodules seldom occur (5-10% of cases) and are usually confined to the subset of patients with polyarticular, rheumatoid factor (RF)-positive disease. In children, as in adults, this extraarticular manifestation is associated with a poor outcome (2).…”

Section: Methotrexate-associated Appearance and Rapid Progression Of mentioning

confidence: 99%

Methotrexate‐associated appearance and rapid progression of rheumatoid nodules in systemic‐onset juvenile rheumatoid arthritis

Falcini

Taccetti

Ermini

et al. 1997

Rheumatoid nodules are a rare extraarticular manifestation of juvenile rheumatoid arthritis (JRA), usually detected in patients with polyarticular‐onset disease and positive rheumatoid factor (RF). To date, there has not been a published report of rheumatoid nodules in systemic‐onset JRA. Low‐dose methotrexate (MTX) is generally considered to be the most useful second‐line drug in the treatment of polyarticular JRA. In adult RA, MTX has been shown to be associated with appearance and progression of rheumatoid nodules. This report describes a 3‐year‐old girl with RF‐negative, antinuclear antibody‐negative systemic JRA who developed multiple rheumatoid nodules on the scalp and trunk during MTX therapy. The first nodule developed on the scalp 6 months after MTX treatment was initiated. Previous treatment with azathioprine was not associated with nodulosis. This represents an atypical case of MTX‐associated accelerated nodulosis in systemic JRA, and raises the problem of treatment plan modification in the presence of this side effect.