[A rare cause of recurrent monarthritis of the knee]. / Seltene Ursache für rezidivierende Monarthritis.

Isolated monarthritis caused by Tropheryma whipplei without involvement of the gastrointestinal tract is rare but is nowadays often described as an early manifestation of the disease. In a male patient with recurrent knee joint arthritis for several years, we could ultimately diagnose Whipple's disease based on PCR positive biopsies of synovial tissue and fluid. Furthermore, the patient was found to be an asymptomatic T. whipplei carrier. With adequate antibiotic therapy the patient has meanwhile fully recovered.

Factors explaining limitations in activities and restrictions in participation in rheumatoid arthritis.

AIM: The objectives of this study were to examine which factors, according to the International Classification of Functioning, Disability and Health (ICF) framework contribute to the explanation of activity limitations measured by the Health Assessment Questionnaire (HAQ - model I) and which factors contribute to the explanation of participation restrictions measured by the Social Function Scale of SF-36 (model II) in patients with rheumatoid arthritis (RA). METHODS: Cross-sectional data collection of variables concerning the health status of 239 consecutively included patients with RA at the outpatient Departments of Physical Medicine and Rehabilitation of the University Hospital of Zurich and of the University Hospital of Munich was conducted. Measures included: disease activity score (DAS-28), Rheumatoid Arthritis Disease Activity Index (RADAI), HAQ, Short-form-36 (SF-36), Sociodemo-graphy Questionnaire, Comorbidity Questionnaire (SCQ), Muscle Strength Index (MSI), range of motion (EPM-ROM), grip strength, Sequentional Occupational and Dexterity Assessment (SODA), radiologic score (Ratingen Score). Multivariate regression analyses were conducted building models of explanation. RESULTS: Model I included vitality, RADAI, DAS, SODA PAIN Score, MSI and EPM-ROM as explaining variables with a globally explained variance of 53%. Model II included vitality, mental health, the HAQ and living alone as explaining variables with a globally explained variance of 42.4%. CONCLUSION: Activity limitations in RA were mainly explained by vitality and disease activity factors. Restrictions in participation in RA were mainly explained by vitality and mental health.

[Diffuse pulmonary calcifications caused by hyperparathyroidism in a renal transplant recipient]. / Diffuse Lungenverkalkungen bei renal bedingtem, autonomem Hyperparathyreoidismus.

HISTORY AND CLINICAL FINDINGS: Two years after a first renal transplantation a 53-year-old man suffering from slowly progressing renal failure had developed progressive amyasthenia and myalgia in his legs and arms, but no dyspnea. Clinical examination, especially for neurological and respiratory abnormalities, was unremarkable. INVESTIGATIONS: Laboratory tests revealed a slightly raised calcium level and an extremely high parathormone (PTH) level. The chest x-ray revealed apical infiltrates, which were interpreted as diffuse pulmonary calcifications caused by hyperparathyroidism. TREATMENT AND COURSE: "Tertiary" hyperparathyroidism was diagnosed. After resection of five hyperplastic parathyroid glands the muscular symptoms disappeared, but the lung infiltates persisted. Four years later a second renal transplantation was necessary. Five years thereafter the patient died of congestive heart failure caused by coronary, hypertensive and valvular heart disease. CONCLUSION: In patients with chronic renal failure and pulmonary infiltrates - with or without respiratory symptoms - calcifications in the context of hyperparathyroidism have to be considered. Calcifications in the lung occur with several other diseases and have a variety of clinical presentations. Prognosis is related to the underlying disease.

Plasma levels of tamoxifen, N-desmethyl tamoxifen and anastrozole in a patient with metastatic breast cancer and chronic hemodialysis.

Endocrine therapy for hormone-sensitive breast cancer is a well-established treatment option, both in adjuvant and palliative settings. For patients undergoing chronic hemodialysis, only scant pharmacokinetic data have been published for tamoxifen, and no data have been published for anastrozole. We therefore measured plasma levels of tamoxifen, its major metabolite, N-desmethyl tamoxifen, and anastrozole in a breast cancer patient undergoing chronic hemodialysis. Clinical tolerability was good. The blood levels for tamoxifen, N-desmethyl tamoxifen and anastrozole were within the expected therapeutic ranges. From this study, we can conclude that endocrine therapy for breast cancer with tamoxifen or anastrozole seems feasible and safe for patients undergoing chronic hemodialysis.

Effectiveness of a measurement feedback system on outcome in rheumatoid arthritis: a controlled clinical trial.

BACKGROUND: With the help of a measurement feedback system, the treatment strategy for individual patients with rheumatoid arthritis (RA) can be adjusted to achieve optimal control of disease activity. OBJECTIVE: To study whether a measurement feedback system is effective in reducing disease activity in patients with RA. METHODS: Forty eight rheumatologists and 264 patients participated in a controlled clinical trial. A three month control period was followed by a 12 month period, where feedback on disease activity, disability, and damage was provided to the rheumatologist. The primary outcome measure was the rheumatoid arthritis disease activity index (RADAI). RESULTS: The feedback system was used for 142/228 (62%) patients. Disease modifying antirheumatic drug changes occurred in 69/169 (41%) patients. In patients with high disease activity and feedback use (n=70), the RADAI decreased in the feedback period by -0.27 points per 30 days (p<0.05), as compared with the control period. Patients for whom the feedback system was used had a better outcome than non-users. CONCLUSION: Much more training on the use of a feedback system and outcome measures, as well as the inclusion of explicit treatment guidelines will be necessary to increase the clinical use of measurement feedback and, possibly, to reduce disease activity for a larger number of patients with RA.

Rheumatologists' opinions on the feasibility of a measurement feedback system in rheumatoid arthritis and the influence of motivation.

OBJECTIVE: To assess rheumatologists' opinions about the feasibility of a measurement feedback system in rheumatoid arthritis (RA) and to analyse if motivational aspects play a role in assessing the value of the system and in determining the extent to which it is used. METHODS: A survey sample (n=105) was randomly selected from participants of a measurement feedback system. A survey questionnaire assessed opinions on system outcome, structures and processes, motivation and overall satisfaction. Survey results are given descriptively and groups differing in motivation are compared. RESULTS: The overall response rate was 62%. The system was generally perceived to fulfil its aims, but the effort required to use the system was rated less positive. Rheumatologists had as their motivation either 'science/obligation' or 'individual patient evaluation'. Rheumatologists with the latter motivation were more satisfied with the measurement feedback system, perceived its feasibility as better, and made more use of it. CONCLUSION: Motivation for participating in a measurement feedback system has a significant impact on overall satisfaction with the system and the use of the system. Influencing motivation and reduction of the amount of effort required to use the system might increase overall acceptance.

The ICIDH-2 as a framework for the assessment of functioning and disability in rheumatoid arthritis.

OBJECTIVE: To investigate by a cross sectional study in patients with rheumatoid arthritis (RA) the relationship between measures of impairment, activity limitation, and participation of the model of functioning and disability (ICIDH-2). METHODS: Inclusion data of patients with RA (n=803) from the Swiss Clinical Quality Management Group were used. Impairments were measured by the Short Form-36 (SF-36) bodily pain scale, rheumatoid arthritis disease activity index (RADAI), disease activity score (DAS28), and radiographic scoring (x ray). Activity limitation was measured with the Health Assessment Questionnaire (HAQ) and the SF-36 physical functioning scale. Participation was measured with the SF-36 role and social functioning scales. Spearman (partial) correlations were used for analysis. RESULTS: Impairment and activity limitation dimensions of the ICIDH-2 model are related; correlations with the HAQ were: SF-36 bodily pain (r(s)=-0.61), RADAI (r(s)=0.58), DAS28 (r(s)=0.49), and x ray (r(s)=0.35). Similar correlations were found for SF-36 physical functioning. Activity limitation and participation restriction dimensions are also related: the HAQ correlates well with SF-36 role-physical (r(s)=-0.53) and SF-36 social functioning (r(s)=-0.43); SF-36 physical functioning correlates similarly. For impairment and participation restriction dimensions only SF-36 bodily pain is substantially correlated (r(s)=0.47 and 0.48) with SF-36 role-physical, after correcting for the influence of the activity limitation dimension (HAQ and SF-36 physical functioning). CONCLUSIONS: In this cross sectional study of patients with RA, impairments are associated with activity limitations, and activity limitations are associated with participation restrictions. Pain is the only impairment directly associated with participation restrictions. Based on the results of this study, it is strongly recommended that the ICIDH-2 framework is used in clinical trials and observational studies including the assessment of disease consequences in RA.

[Clinical quality management in rheumatoid arthritis]. / Klinisches Qualitätsmanagement bei der Rheumatoiden Arthritis.

Clinical quality management in rheumatoid arthritis (RA) aims at reducing inflammatory activity and pain in the short term and damage and disability in the long term. In the "Swiss Clinical Quality Management in rheumatoid arthritis" (SCQM) project, which started in 1997, a measurement-improvement system with feedback reports allows the rheumatologists to follow their RA patients with the aim of improving the quality of outcome. Inflammatory activity is measured with the Disease Activity Score (DAS28) and the Rheumatoid Arthritis Disease Activity Index questionnaire (RADAI), damage with an X-ray score and disability with the Stanford Health Assessment Questionnaire (HAQ). The feedback is used by the individual rheumatologist to optimize the therapy of his/her RA patients. Beside the aim of improving the quality of treatment, the SCQM projects wants to build a Swiss cohort of RA patients, to improve the collaboration of rheumatologists in the clinic and in practice and to establish standards of treatment in RA. In this paper we describe the SCQM project in detail, we show two cases illustrating the usefulness of the SCQM in the management of individual RA patients, and we present the cross sectional analysis of the first 1223 RA patients included in the project.

Clinical quality management in rheumatoid arthritis: putting theory into practice. Swiss Clinical Quality Management in Rheumatoid Arthritis.

Clinical quality management (CQM) in rheumatoid arthritis (RA) aims to reduce inflammatory activity and pain in the short term, and damage, and consequently disability, in the long term. Within CQM as used in Switzerland rheumatologists are provided with a measurement feedback system with which they can regularly follow their patients. Inflammatory activity is measured with the Disease Activity Score (DAS28) and the Rheumatoid Arthritis Disease Activity Index questionnaire (RADAI), damage with an X-ray score and disability with the Stanford Health Assessment Questionnaire (HAQ). Feedback is used to optimize therapy, which in the short term allows the activity of the inflammatory process to be adjusted or 'titrated'. In the long term, the therapy result for the individual patient is monitored by the course of disability and damage. In this paper we present a series of cases to illustrate the usefulness of the CQM system in the management of individual RA patients. CQM in RA may be helpful when making decisions about adjustment of treatment, and to document and communicate these decisions based on quantitative data.

Feasibility and validity of the RADAI, a self-administered rheumatoid arthritis disease activity index.

OBJECTIVE: The goal of the Rheumatoid Arthritis Disease Activity Index (RADAI) is to provide an easy to use assessment of disease activity. It is a self-administered questionnaire that combines five items into a single index: current and past global disease activity, pain, morning stiffness and a joint count. METHODS: A sample of 484 rheumatoid arthritis (RA) patients was used to assess the internal consistency and the convergent validity of the RADAI. This was achieved by calculating Cronbach's alpha and RADAI item and total score correlations with core set measures and DAS28. RESULTS: Cronbach's alpha was 0.87, supporting the summation of the items into a single index. The index correlated best with physicians' global assessment (r = 0.59; P < 0.0001), the Health Assessment Questionnaire (r = 0.55; P < 0.0001) and the number of tender joints (r = 0.55; P < 0.0001). Correlation with the erythrocyte sedimentation rate was low (r = 0.27; P < 0.0001). The RADAI and the DAS28 were correlated (r = 0.53; P < 0.0001), but there was low agreement. CONCLUSIONS: The RADAI is valid to assess disease activity in RA patients. However, the RADAI may not automatically replace other measures of disease activity, such as the DAS28.

Diffuse chronic sclerosing osteomyelitis and the synovitis, acne, pustolosis, hyperostosis, osteitis (SAPHO) syndrome in two sisters.

Two sisters with diffuse chronic sclerosing osteomyelitis of the mandible and the humerus and the synovitis, acne, pustolosis, hyperostosis and osteitis syndrome (SAPHO syndrome) are presented. The diagnoses of diffuse chronic sclerosing osteomyelitis at the age of 12 years and 27 years, respectively, were based on typical medical history, clinical symptoms and radiographic, histologic and scintigraphic findings. Because skin lesions and scintigraphic enhancement of the sternoclavicular joints with hyperostosis were present, a SAPHO syndrome was diagnosed in both sisters. Microbiological cultures of biopsy specimens revealed coagulase-negative Staphylococcus aureus at the humerus and Haemophilus parainfluenzae, Streptococcus, Actinomyces and Veilonella species at the mandible. Repeated operative procedures, including decortications, resection and reconstruction, and multiple histologic and microbiologic studies were performed over a period of up to 20 years. Since HLA typing yielded identical gene loci, we suggest that hereditary and autoimmune factors may play a role in the pathogenesis of these cases.

Primary chronic osteomyelitis associated with synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO syndrome).

Diffuse sclerosing osteomyelitis may indicate the mandibular localisation of the SAPHO syndrome. Twelve patients with diffuse sclerosis of the mandible were examined for symptoms of the SAPHO syndrome. Nine patients were found to have primary chronic osteomyelitis and eight of these represented a SAPHO syndrome. Results in this series support the hypothesis of an association between primary chronic osteomyelitis and the SAPHO syndrome.

Drug treatment for rheumatoid arthritis.

The general goals of drug treatment for patients with rheumatoid arthritis are to reduce morbidity and mortality. Because rheumatoid arthritis is a potentially devastating disease, a more aggressive treatment approach has emerged in the last decade. The modern treatment pyramid consists of nonsteroidal antiinflammatory drugs and glucocorticoids for symptomatic relief, and disease modifying antirheumatic drugs for reducing disease activity in the short term and joint damage in the long term. There is increasing evidence that a reduction of disease activity by disease modifying antirheumatic drugs alters the course of rheumatoid arthritis and that patients benefit from early installation of these compounds. The major problem with disease modifying antirheumatic drugs is their low efficacy to toxicity ratio, leading to marked reduction of the length of time a patient is taking a given drug. The new treatment strategies, including combination regimens and new drugs that are being investigated, promise better efficacy and tolerance in the near future. A step in this direction is the development of biologic agents targeting specific mechanisms in the immune response. Early results in clinical trials with antitumor necrosis factor-alpha monoclonal antibodies are encouraging.

Management of rheumatoid arthritis.

There is increasing evidence that reduction of disease activity by disease-modifying drugs alters the disease course of rheumatoid arthritis and that patients benefit from early introduction of disease-modifying antirheumatic drugs. To ensure accurate diagnosis and timely referral, training of medical students and residents in rheumatology and access to a physician experienced in the management of rheumatic diseases is important. Because spontaneous remission is rare, the risk of overtreatment of a few patients is outweighed by the benefits of early treatment for the majority of patients. Also, side effects may be reduced with optimation of titration of disease activity based on standardized, reliable, valid, sensitive, and easily interpretable measures with clear-cut decision rules. A potential solution to the practical problems involving the scoring of patient questionnaires such as the Health Assessment Questionnaire and the Rheumatoid Arthritis Disease Activity index and clinical algorithms such as the Disease Activity Score is the provision of a feedback system similar to a laboratory. To feed back the results of titration of disease activity with the goal of permanently improving health outcomes in an ongoing learning process may be called clinical quality management. From a health care system or political perspective, clinical quality management that demonstrates the commitment of rheumatology toward continuous improvement of rheumatoid arthritis care may become an important activity of rheumatology societies in countries where specialty care is under scrutiny and where specialists are increasingly required to show the benefits of their care.

[Pericarditis in chronic inflammatory bowel disease: underlying disease or side effects of therapy? Clinical problem solving]. / Perikarditis bei chronisch entzündlicher Darmerkrankung: Grundkrankheit oder Nebenwirkung der Therapie? Ein "clinical problem solving".

Pericarditis and myocarditis are rare extraintestinal manifestations of chronic inflammatory bowel disease (ulcerative colitis and Crohn's disease). Pericarditis as a side effect induced by sulfasalazine or 5-aminosalicylic acid, drugs used in the therapy of these diseases, was first described only 7 years ago. In older case reports the relationship between the use of these drugs and pericarditis is unclear. We analyze the reported cases of 68 patients (38 men, 24 women) with ulcerative colitis (n = 45) or Crohn's disease (n = 15) who had one or more episodes of pericarditis or myopericarditis. Pericarditis was not associated with high activity of bowel disease in all cases. In most cases therapy with corticosteroids led to uneventful recovery. In drug induced pericarditis omission of the 5-ASA therapy was sufficient in a few cases. There was one fatal case (with myocarditis). The decision whether pericarditis is a symptom of the underlying disease or a side effect of the drug used for the treatment of the disease is not always easy. We present an analysis (clinical problem solving) of a pertinent observation in a patient with Crohn's disease and pericarditis, showing the dilemma of pericarditis in chronic inflammatory bowel disease and its therapy.