Rheumatoid Arthritis: Cabral DA

Houghton KM, Cabral DA, Petty RE, Tucker LB. · Division of Rheumatology, British Columbia's Children's Hospital and University of British Columbia, Vancouver, Canada. · J Rheumatol. · Pubmed #16078341 No free full text.

Abstract: Primary Sjögren's syndrome (pSS) is uncommonly recognized in childhood, and familial cases are rare. Pulmonary involvement in pediatric pSS is infrequently reported. In adults, asymptomatic pulmonary involvement is increasingly recognized, manifest by pulmonary function test abnormalities and changes on high resolution computerized tomographic scan. We describe a case of pSS in a 14-year-old Vietnamese-Canadian girl who presented with pulmonary symptoms, radiologic changes, and biopsy confirmation of lymphocytic interstitial pneumonia. Her dizygotic twin sister has primary SS without extraglandular manifestations. To our knowledge this is the first report of pediatric pSS with lymphocytic interstitial pneumonia and multiple pulmonary nodules on chest radiograph. We review the literature on pulmonary involvement and familial cases of pSS in childhood.

Oen K, Malleson PN, Cabral DA, Rosenberg AM, Petty RE, Nickerson P, Reed M. · Department of Paediatrics, University of Manitoba, Winnipeg, Canada. · Rheumatology (Oxford). · Pubmed #15901906 links to  free full text

Abstract: OBJECTIVES: Single nucleotide polymorphisms (SNPs) in cytokine genes have been associated with risk of a number of autoimmune diseases. Moreover, some SNPs are associated with variations in rates of in vitro gene expression, and it is therefore possible that these functional polymorphisms may differentially affect inflammatory processes and disease outcome. This project's objective was to determine whether cytokine genotypes correlate with disease outcomes in patients with juvenile rheumatoid arthritis (JRA). METHODS: Genotypes of SNPs of pro-inflammatory cytokines, tumour necrosis factor-alpha -308G -->A, interleukin-6 (IL-6) -174G -->C and interferon-gamma +874G -->A, and anti-inflammatory, immunosuppressive cytokines, interleukin-10 -1082G -->A, -819C -->T and -592A -->C and transforming growth factor-beta1 (TGF-beta1) codon 10T -->C and codon 25G -->C, were determined for patients with JRA who previously participated in a long-term outcome study. Cytokine genotypes and clinical variables showing significant correlations with clinical outcomes at the alpha = 0.100 level in univariate analyses were entered in multivariate tests. RESULTS: In multivariate tests, the IL-6 genotype -174G/G was positively correlated with pain [regression coefficient B = 0.899, 95% confidence intervals (CI) 0.185, 1.612, P = 0.014]. The homozygous TGF-beta1 codon 25G/G genotype showed a protective effect against joint space narrowing on radiographs taken within 2 yr of disease onset, but confidence intervals were wide [odds ratio (OR) 0.176, 95% CI 0.037, 0.837 P = 0.029]. CONCLUSIONS: The correlation of IL-6 genotype with pain and the possible association of the TGF-beta1 codon 25 genotype with short-term radiographic damage (G/C with greater risk and G/G with decreased risk) suggests that both these polymorphisms may be useful early prognostic indicators. Further studies of the relation between cytokine genotypes and outcomes in patients with all forms of juvenile idiopathic arthritis (JIA) are warranted.

Tucker LB, Cabral DA. · Division of Pediatric Rheumatology, Centre for Community Child Health Research, British Columbia Children's Hospital, 4880 Oak Street, Room K4-120, Vancouver, BC V6H 3V4, Canada. · Pediatr Clin North Am. · Pubmed #15820382 No free full text.

Abstract: This article discusses the definitions of transition and models for providing transition care to youth and young adults with rheumatic diseases in the context of differing cultural and health care systems. Issues and questions relating to pediatric rheumatology transition programming are outlined, and a model program of pediatric rheumatology transition is presented.

Ortiz-Alvarez O, Morishita K, Avery G, Green J, Petty RE, Tucker LB, Malleson PN, Cabral DA. · Division of Rheumatology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada. · J Rheumatol. · Pubmed #15570658 No free full text.

Abstract: OBJECTIVE: To assess the utility of the American College of Rheumatology guidelines for monitoring methotrexate (MTX)-related toxicity in a cohort of children with juvenile idiopathic arthritis (JIA). METHODS: Eighty-nine patients with JIA treated with MTX were monitored prospectively: aspartate aminotransferase (AST), alanine aminotransferase (ALT), complete blood count (CBC), and differential blood count were measured prior to starting MTX, and then monthly. Significantly abnormal blood tests (SABT) were prospectively defined as (1) significantly elevated liver enzymes (SELE) greater than twice the upper limit of normal; (2) granulocyte count < 1.5 109/l; (3) lymphocyte count < 0.9 109/l; or (4) hemoglobin decreased by > 2 g/l from previous level. Clinical interventions, current and cumulative MTX dose, duration of treatment, comorbidity, and concurrent medications at the time of the first SABT identification were recorded. Independent t tests and chi-squared tests were used for comparisons, and the probability of developing a SABT was calculated by Kaplan-Meier survival analysis. RESULTS: Forty percent of patients had a SABT: 26% had hematological abnormalities and 14% had SELE. Ninety-five percent of patients with SABT had symptoms consistent with a viral infection when the SABT was drawn and MTX dose was withheld until results had normalized on repeat testing. SABT persisting beyond one month occurred in only 2 patients, and their abnormalities resolved by 6 months with no specific identified cause; they resumed MTX at a later time without recurrence of SABT. There were no differences between patients with and without SABT with respect to current or cumulative MTX dose, duration of treatment, and concurrent medications at the time of the SABT. The probability of developing a SABT was estimated to be 11% at 3 months, compared to 10% probability of having an abnormal blood test by chance alone. CONCLUSION: Routine blood tests every 4 to 8 weeks in children with JIA are unnecessarily frequent.

Prendiville JS, Tucker LB, Cabral DA, Crawford RI. · Division of Pediatric Dermatology, Department of Pediatrics, British Columbia's Children's Hospital, Vancouver, British Columbia, Canada. · Pediatr Dermatol. · Pubmed #15461768 No free full text.

Abstract: The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.

Malleson PN, Oen K, Cabral DA, Petty RE, Rosenberg AM, Cheang M. · University of British Columbia, Vancouver, British Columbia, Canada. · Arthritis Rheum. · Pubmed #15077263 links to  free full text

Abstract: OBJECTIVE: To examine demographic and disease-related variables that affect pain in a large cohort of patients with juvenile rheumatoid arthritis (JRA). METHODS: Selection criteria were an onset of JRA >/=5 years prior to study and age >/=8 years at the time of the study. Pain was measured by a self-administered 10-cm visual analog scale. Possible explanatory variables studied included age at study, sex, race, onset subtype, active disease duration, active joint count, and physician's global assessment (PGA). RESULTS: In a multiple regression model, active disease duration, PGA, and age at study were independent predictors explaining 22% of the variation in pain scores. Stratified analyses showed an effect of age in the 8-15-year group, but not in older patients. CONCLUSION: Disease-related factors explain only a small proportion of the variation in pain scores. Age has an effect on pain scores only in younger patients. The role of other factors, including psychosocial factors, needs further study.

Alsufyani K, Ortiz-Alvarez O, Cabral DA, Tucker LB, Petty RE, Malleson PN. · Division of Rheumatology, Department of Pediatrics, University of British Columbia, Vancouver, BC, Canada. · J Rheumatol. · Pubmed #14705239 No free full text.

Abstract: OBJECTIVE: To describe the outcome of patients with juvenile idiopathic arthritis (JIA) treated with subcutaneous (Sc) methotrexate (MTX) after failing oral MTX (either because of inefficacy or toxicity) in a clinic population. METHODS: The study cohort was identified from our clinical database, and consisted of 61 children with JIA treated with MTX between 1988-2001. All patients fulfilled International League Against Rheumatism (ILAR) criteria for JIA and had disease duration of >/= 6 months and 3 or more active joints before institution of MTX. All patients had a core set of outcome variables assessed at baseline and at 3 months after achieving both maximum oral and SC MTX. Outcome variables included physician global assessment of disease activity, number of active joints, number of joints with limited range of motion, duration of early morning stiffness, and erythrocyte sedimentation rate (ESR). Improvement was defined as at least 30% improvement from baseline in 3 of 5 variables in the core set, with no more than one of the remaining variables worsening by more than 30%. RESULTS: A total of 61 patients, 43 females and 18 males with JIA were studied. The disease subtypes were systemic 8, polyarticular 25 (12 rheumatoid factor positive), oligoarticular 14, enthesitis related arthritis 5, and unclassified 4. Thirty-one patients were switched to SC MTX, 13 of whom had not improved, and 18 who had improved, but had nausea (11) or insufficient clinical improvement (7). After 3 months of SC MTX treatment, 76% of patients were classified as improved and 23% as not improved. Toxicity on SC MTX was less than on oral MTX. CONCLUSION: Our results suggest that for patients failing oral MTX either because of inefficacy or toxicity, the use of SC MTX has a high likelihood of success with more than 70% of patients achieving clinically significant improvement, without clinically significant toxicity.

Oen K, Reed M, Malleson PN, Cabral DA, Petty RE, Rosenberg AM, Cheang M. · Department of Pediatrics, University of Manitoba, Winnipeg, Manitoba, Canada. · J Rheumatol. · Pubmed #12672208 No free full text.

Abstract: OBJECTIVE: To determine the radiologic outcome in juvenile rheumatoid arthritis (JRA) and the relationship of radiologically detected joint damage to functional disability using multivariate analyses. METHODS: Selection criteria included a diagnosis of JRA made by 1977 American College of Rheumatology criteria, onset of arthritis > or = 5 years prior to study, current age > or = 8 years, a minimum grade 3 reading ability, and the availability of radiographs. Disability was measured by the Childhood Health Assessment Questionnaire (CHAQ) and Steinbrocker classifications. Radiographs taken within 2 years after onset (early) and the most recent radiographs (late) were examined by a single pediatric radiologist blinded to patients' identities, diagnoses, and outcomes. Multiple regression analyses were performed. RESULTS: On late radiographs the frequencies of joint space narrowing were 38, 14, 43, and 79%, respectively, among patients with systemic, pauciarticular, rheumatoid factor (RF) negative polyarticular, and RF positive polyarticular onset; erosions occurred in 63, 25, 39, and 75%, respectively. Early erosions were most frequent in patients with RF+ polyarticular onset, while both joint space narrowing and erosions occurred early in systemic onset. Radiologic signs of joint damage were most frequent at hips and wrists, while knees and ankles were relatively spared. Based on patients who had radiographs performed within one year of clinical study, 17.7% of the variation in CHAQ score was explained by joint space narrowing, 32.4% by pain, and 5% by a severe rating on physician's global estimate of disease activity. The odds of a Steinbrocker class > I were increased by joint space narrowing, pain, systemic onset, and active joint count. CONCLUSION: Differences in the frequencies and patterns of joint damage occur both among JRA onset subtypes and among individual joints. Radiographic damage, especially joint space narrowing, correlates with functional disability. However, pain is the major contributor to variation in CHAQ scores.

Oen K, Malleson PN, Cabral DA, Rosenberg AM, Petty RE, Reed M, Schroeder ML, Cheang M. · Departments of Paediatrics, Radiology, and Community Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada. · J Rheumatol. · Pubmed #12610821 No free full text.

Abstract: OBJECTIVE: To determine early predictors of longterm outcome in juvenile rheumatoid arthritis (JRA) in a multicenter cohort. METHODS: Patients were selected if they were > or = 8 years of age; the onset of arthritis occurred > or = 5 years before study; and a diagnosis of JRA was made at a participating center. Outcome variables were scores on self-administered Childhood Health Assessment Questionnaires (CHAQ) and active disease duration. Possible explanatory variables assessed included characteristics present at onset, HLA alleles, in particular the rheumatoid arthritis associated shared epitope (RASE), and radiographic indicators of joint damage within 2 years of onset. Data for 393 patients were available. Multivariate analyses were performed for the total group and for each onset subtype. RESULTS: Male sex correlated with worse disability in systemic onset JRA but less disability in RF negative, and a shorter active disease duration in RF positive polyarticular onset JRA. Positive antinuclear antibody correlated with a longer active disease duration in patients with pauciarticular onset JRA. Younger age at onset predicted longer active disease duration in pauciarticular and RF negative polyarticular, and a shorter active disease duration in systemic onset JRA. Residence on a reserve, rather than native North American race, correlated with worse disability. The RASE correlated with less disability in systemic JRA; but no correlation with outcome was evident for patients with rheumatoid factor positive polyarticular JRA. CONCLUSION: Variables predictive of longterm outcome in JRA are specific for each onset subtype. The most important early predictors were age at onset and sex of the patient. Place of residence may have a greater effect on disability than race. RASE may associate with a more favorable outcome in systemic onset disease.

Al-Matar MJ, Petty RE, Tucker LB, Malleson PN, Schroeder ML, Cabral DA. · University of British Columbia, Vancouver, British Columbia, Canada. · Arthritis Rheum. · Pubmed #12384930 links to  free full text

Abstract: OBJECTIVE: To evaluate features during the first 6 months of disease that may be associated with a poor outcome as measured principally by extension to a polyarticular disease course in patients with oligoarticular-onset juvenile rheumatoid arthritis (oligo-JRA). METHODS: This study was a retrospective review of patients who fulfilled the American College of Rheumatology criteria for oligo-JRA, were followed up for at least 5 years, and did not have juvenile psoriatic arthritis, spondylarthropathy-like disease, or rheumatoid factor positivity. Data from the first 6 months of disease were collected. Continuous variables were dichotomized and then screened by univariate analysis for association with poor outcome at the last followup visit, as measured by extension of involvement (>4 accumulated involved joints) and by "clinically meaningful" extension (> or =10 accumulated joints). Variables significantly associated with this latter outcome, with the addition of disease duration as a confounding independent variable, were included in a multiple logistic regression analysis. The same variables were then examined in separate multiple logistic regression models to look at other measures of outcome, including use of disease-modifying antirheumatic drugs (DMARDs) at any time, erosive disease on radiographs, any remission of disease ever occurring, physician's global assessment of disease activity at the last visit, and disability as measured by the Childhood Health Assessment Questionnaire (C-HAQ)/HAQ. RESULTS: Of the 205 patients (160 of whom were female) studied for a median of 10.8 years (range 5-26.6 years), 39.5% developed extension to >4 joints and 17.6% developed arthritis in > or =10 joints. Using the logistic regression model, symmetric disease was predictive of all measures of poor outcome: extension to > or =10 joints (odds ratio [OR] 19.2), the need to use DMARDs (OR 11.5), radiographic demonstration of erosive disease (OR 4.73), inflammatory activity at last followup visit (OR 3.23), no remission of disease (OR 4.73), and disability as measured by a C-HAQ score >0.12 (OR 2.95). Ankle and/or wrist disease was predictive of extension (OR 6.61) and erosions (OR 3.59). Wrist disease alone was predictive of the need to use DMARDs (OR 5.87) and of inflammatory disease activity at the last followup visit (OR 4.01). An elevated erythrocyte sedimentation rate (ESR) was predictive of extension (OR 3.76), the need to use DMARDs (OR 6.47), and no remission of disease (OR 2.30). Disease duration was a confounding variable for extension (OR 1.18) and erosive disease (OR 1.19). CONCLUSION: The early presence of ankle and/or wrist disease, symmetric joint involvement, and an elevated ESR in a child with oligo-JRA indicates the likelihood of disease progression.

Oen K, Malleson PN, Cabral DA, Rosenberg AM, Petty RE, Cheang M. · Department of Paediatrics, University of Manitoba, Winnipeg, Canada. · J Rheumatol. · Pubmed #12233897 No free full text.

Abstract: OBJECTIVE: To determine the disease course and outcome in a multicenter cohort of patients with juvenile rheumatoid arthritis (JRA). METHODS: All patients with JRA seen at 3 pediatric rheumatology centers were identified from databases and/or clinic records. Inclusion criteria were a diagnosis of JRA (1977 American College of Rheumatology criteria), a followup period of at least 5 years since onset, and a minimum age of 8 years. Patients were examined and completed a Childhood Health Assessment Questionnaire (CHAQ). Kaplan-Meier curves were constructed to estimate rates of remission, relapse, and arthroplasty. Remission was defined as absence of active arthritis while off treatment for at least 2 years. Outcome measures were active disease duration, CHAQ scores, pain determined by visual analog scales, physician's global assessments, and Steinbrocker functional classifications. Years of education and employment status were ascertained. RESULTS: We studied 392 patients of 652 (60%) who met the selection criteria. The probabilities of remission at 10 years after onset were 37, 47, 23, and 6% for patients with systemic, pauciarticular, RF- polyarticular, and RF+ polyarticular JRA, respectively. The probability of relapse varied from 30 to 100% at 15 years. The probability of arthroplasty varied from 13 to 57% after 15 years of active disease. We found 2.5% of patients assessed were in Steinbrocker Classes III or IV and 6% were in the highest CHAQ score (> 1.5) group. Compared with national statistics, fewer female patients received post-secondary education and unemployment rates for patients 20 to 24 years of age were higher. CONCLUSION: Our results indicate that JRA is a disease that often extends into adulthood. Compared to previous decades, functional outcome has improved; however, the estimated rate of arthroplasty remains very high. Patients with JRA may have difficulty entering the workforce.

Huemer C, Malleson PN, Cabral DA, Huemer M, Falger J, Zidek T, Petty RE. · Department of Pediatrics, University of Vienna, Austria. · J Rheumatol. · Pubmed #12136915 No free full text.

Abstract: OBJECTIVE: To compare the patterns of joint involvement of patients with oligoarticular onset juvenile psoriatic arthritis (Oligo-JPsA) and pauciarticular onset juvenile rheumatoid arthritis (Pauci-JRA) in order to estimate the predictive performance of specific patterns for the diagnosis of Oligo-JPsA. METHODS: Twenty-three children who fulfilled the diagnostic criteria for JPsA (Vancouver criteria) and who had fewer than 5 joints involved in the first 6 months of disease (Oligo-JPsA), and 64 children with Pauci-JRA (ACR criteria) were enrolled. Patients were also classified with respect to the ILAR criteria for juvenile idiopathic arthritis (JIA). Patient characteristics and clinical features at onset and during followup were determined. Patterns of joint involvement at onset of disease and their ability to differentiate between Oligo-JPsA and Pauci-JRA/Oligo-JIA were evaluated. RESULTS: Small joint disease (defined as involvement of any of the metatarsophalangeal or proximal or distal interphalangeal joints of the foot, or metacarpophalangeal or proximal or distal interphalangeal joints of the hand) was significantly more frequent in Oligo-JPsA than in Pauci-JRA at disease onset. The odds of patients with Oligo-JPsA having small joint disease or wrist disease within 6 months of disease onset were much higher than those with Pauci-JRA or Oligo-JIA (p < 0.05 or 0.001). CONCLUSION: Small joint disease and wrist disease are suggestive of Oligo-JPsA. The use of a criterion consisting of small joint disease and/or wrist disease and/or dactylitis instead of dactylitis alone may increase the ability to differentiate Oligo-JPsA from Pauci-JRA or Oligo-JIA.

Al-Matar MJ, Petty RE, Cabral DA, Tucker LB, Peyvandi B, Prendiville J, Forbes J, Cairns R, Rothstein R. · Department of Pediatrics and Radiology, University of British Columbia at British Columbia's Children's Hospital, Vancouver, Canada. · J Rheumatol. · Pubmed #11824958 No free full text.

Abstract: We describe 2 patients with very unusual rheumatological presentations presumably caused by Bartonella infection: one had myositis of proximal thigh muscles bilaterally, and the other had arthritis and skin nodules. Both patients had very high levels of antibody to Bartonella that decreased in association with clinical improvement. Bartonella infection should be considered in the differential diagnosis of unusual myositis or arthritis in children.

Al-Matar MJ, Cabral DA, Petty RE. · Department of Pediatrics, University of British Columbia, Vancouver, Canada. · J Rheumatol. · Pubmed #11196526 No free full text.

Abstract: Isolated monoarthritis caused by Mycobacterium tuberculosis in the absence of clinical pulmonary disease is extremely rare in North America. After decades of consistent declines in incidence, a remarkable resurgence of tuberculosis (TB) is occurring in North America. It must always be considered in the differential diagnosis of chronic monoarthritis if devastating sequelae are to be avoided. We describe 2 cases of tuberculous arthritis in young children presenting with monoarthritis of the knee. The presumptive diagnosis in each case was oligoarticular onset juvenile rheumatoid arthritis (JRA). Each had an atypical course for JRA, with lack of response to intraarticular corticosteroid. The diagnosis of TB arthritis was made only with synovial biopsy.

Ramsey SE, Bolaria RK, Cabral DA, Malleson PN, Petty RE. · Division of Pediatric Rheumatology, University of British Columbia, Vancouver, Canada. · J Rheumatol. · Pubmed #10813302 No free full text.

Abstract: OBJECTIVE: To evaluate the applicability of the ILAR criteria for classification of childhood arthritis in an outpatient pediatric rheumatology clinic population, and to determine the proportion of children who met standard classification criteria, but failed to meet ILAR criteria for specific arthritides, and therefore became unclassifiable. METHODS: We reviewed the charts of 70 consecutive patients who had arthritis for at least 6 months, and attended the clinic between September and November 1997. Sixty-nine patients were categorized according to one of the traditional classifications [ACR for juvenile rheumatoid arthritis (JRA), European Spondylarthropathy Study Group (ESSG) for spondyloarthropathy, Vancouver Criteria for juvenile psoriatic arthritis (JPsA)], and the ILAR classification system. RESULTS: Sixty-one patients (88.4%) were classifiable by the ILAR system; 8 others failed to fulfill ILAR criteria for any specific category, and were assigned to the "other arthritis" category. Of the 29 patients with oligoarticular onset JRA, 6 were unclassified, 5 because of exclusions, and one because he fulfilled criteria for 2 categories. Presence of a family history of psoriasis accounted for most of the exclusions in the oligoarthritis and enthesitis related arthritis categories. All patients with polyarticular onset or systemic onset JRA were classified in the corresponding category in the ILAR system. One 9-year-old patient with spondyloarthropathy was reclassified as "other arthritis" because of exclusions. All 6 children with definite JPsA met ILAR criteria for PsA. Of 4 patients with probable JPsA, only 2 met ILAR criteria for PsA, a third was classified as rheumatoid factor negative polyarthritis, and the fourth was classified as "other arthritis" because of exclusions. CONCLUSION: The ILAR classification criteria applied to a group of children with chronic arthritis classified by traditional criteria results in reassignment of 11.6% of the patients, predominantly in the oligoarticular group. It will be important to determine the role of the presence of a family history of psoriasis in classifying these patients.

Cabral DA, Tucker LB. · Division of Pediatric Rheumatology, Department of Pediatrics, BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada. · J Pediatr. · Pubmed #9880449 No free full text.

Abstract: OBJECTIVE: Children ultimately diagnosed with malignancy are referred to pediatric rheumatology clinics with provisional rheumatic diagnoses. We aimed to distinguish the features in these patients that lead to the correct diagnosis of malignancy. STUDY DESIGN: A retrospective review of the case records of 29 children (19 boys and 10 girls, aged 1 to 15.5 years) with malignancy who were referred to 2 pediatric rheumatology centers between 1983 and 1997. RESULTS: The suspected diagnoses on referral were: juvenile rheumatoid arthritis (12), nonspecific connective tissue disease (4), discitis (3), spondyloarthropathy (3), systemic lupus erythematosus (2), Kawasaki disease (2), Lyme disease (1), mixed connective tissue disease (1), and dermatomyositis (1). The final diagnoses were leukemia (13), neuroblastoma (6), lymphoma (3), Ewing's sarcoma (3), ependymoma (1), thalamic glioma (1), epithelioma (1), and sarcoma (1). Patients had features typical of many rheumatic disorders including musculoskeletal pains (82%), fever (54%), fatigue (50%), weight loss (42%), hepatomegaly (29%), and arthritis (25%). Features that were suggestive of malignancy included nonarticular "bone" pain (68%), back pain as a major presenting feature (32%), bone tenderness (29%), severe constitutional symptoms (32%), clinical features "atypical" of most rheumatic disease (48%), and abnormal initial investigations (68%). The atypical features included night sweats (14%), ecchymoses and bruising (14%), abnormal neurologic signs (10%), abnormal masses (7%), and ptosis (3%). Initial investigations with abnormal findings included complete blood count/smear (31%), discordant erythrocyte sedimentation rate and platelet count (28%), elevated lactate dehydrognease level (24%), plain skeletal x-ray films (28%), bone scan (21%), and abdominal ultrasonography (17%). Findings of investigations done before referral to the rheumatology clinic were not recognized as abnormal in 11 (40%) patients. CONCLUSIONS: Patients with a diverse group of malignancies, other than leukemia, may present to the pediatric rheumatologist. Pediatric care providers should be familiar with typical features of childhood rheumatic disorders, and rheumatic diagnoses should be reevaluated in the presence of any atypical or discordant clinical features.

Alsufyani K, Ortiz-Alvarez O, Cabral DA, Tucker LB, Petty RE, Malleson PN. · No affiliation provided · Arthritis Rheum. · Pubmed #15529350 links to  free full text

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