Safety and effectiveness of intravenous CT-P13 in inflammatory arthritis: post-marketing surveillance study in Thailand.

Background: The infliximab biosimilar CT-P13 was approved in Thailand in 2015. Methods: This open-label, multicenter, post-marketing surveillance study evaluated the safety (events of special interest [ESIs]; primary end point) and effectiveness of 46 weeks of CT-P13 treatment according to routine practice in patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS), or psoriatic arthritis (PsA), with 1 year follow-up post-treatment. Results: 30 patients were enrolled (16 RA, 8 AS and 6 PsA). Infections were the most frequently reported study drug-related ESIs (2 RA and 2 AS). One patient with RA and one with PsA experienced infusion-related reactions. No cases of tuberculosis, malignancy (as expected, given 1 year follow-up), or drug-induced liver disease were reported. Disease activity improved across indications. Conclusion: CT-P13 was well tolerated and effective across indications.

Infliximab is one biological medicine used to treat inflammatory diseases, including rheumatoid arthritis (RA), ankylosing spondylitis (AS) and psoriatic arthritis (PsA). CT-P13 is a near-identical copy, called a biosimilar, of the original ('reference') version of infliximab. CT-P13 is the first biosimilar to receive regulatory approval for treatment of the same three diseases from the European Medicines Agency (EMA) and US Food and Drug Administration. Biosimilarity means that CT-P13 does not differ from the original version of infliximab in clinically important ways, such as how safe it is and how well it works. CT-P13 and reference infliximab provided similar symptom relief during previous clinical trials, and both drugs caused similar side effects. It is important to monitor the safety and performance of CT-P13 when given during routine clinical practice, and in different ethnic populations, such as through the study reported here. Following regulatory approval in Thailand, 30 patients prescribed CT-P13 during routine clinical practice participated in this study. The study included 16 patients with RA, eight with AS and six with PsA. The patients took CT-P13 for 46 weeks and were monitored for a further year. Side effects of CT-P13 were as expected based on previous experience and did not raise any safety concerns. Based on the known safety profile of CT-P13, the study looked at some side effects in particular: infections were the most common of these side effects, experienced by 16 patients overall (seven patients with RA, five patients with AS and four patients with PsA). CT-P13 improved symptoms for all of the diseases. The study suggests that CT-P13 can be given safely and reduces symptoms in Thai patients with AS, RA or PsA. Thai Clinical Trials Registry: TCTR20170817005 (www.thaiclinicaltrials.org/show/TCTR20170817005).

Comparison of clinical features, disease activity, treatment and outcomes between late-onset and early-onset patients with systemic lupus erythematosus. A sex- and year at diagnosis-matched controlled study

Abstract Background Several studies have compared the clinical features and outcomes of late- and early-onset systemic lupus erythematosus (SLE) patients. However, these previous studies were uncontrolled. The current study aimed to compare late- and early-onset SLE patients while controlling for sex and year at diagnosis (± 1 year). Methods The medical records of SLE patients in a lupus cohort from January 1994 to June 2020 were reviewed. Late-onset patients were identified as those with an age at diagnosis ≥ 50 years. The early-onset patients (age at diagnosis < 50 years) were matched by sex and year at diagnosis with the late-onset patients at a ratio of 2:1. Clinical manifestations, disease activity (mSLEDAI-2K), organ damage scores, treatment, and mortality were compared between the two groups. Results The study comprised 62 and 124 late- and early-onset patients, respectively, with a mean follow-up duration of 5 years. At disease onset, when comparing the early-onset patients with the late-onset patients, the latter group had a higher prevalence rate of serositis (37.0% vs. 14.5%, p < 0.001) and hemolytic anemia (50.0% vs. 33.9%, p = 0.034) but lower prevalence rate of malar rash (14.5% vs. 37.1%, p = 0.001), arthritis (41.9% vs. 62.1%, p = 0.009), leukopenia (32.3% vs. 50.0%, p = 0.022) and lymphopenia (50.0% vs. 66.1%, p = 0.034). The groups had similar SLE disease activity (7.41 vs. 7.50), but the late-onset group had higher organ damage scores (0.37 vs. 0.02, p < 0.001). The rates of treatment with corticosteroids, antimalarial drugs, or immunosuppressive drugs were not different. At their last visit, the late-onset patients still had the same pattern of clinically significant differences except for arthritis; additionally, the late-onset group had a lower rate of nephritis (53.2% vs. 74.2%, p = 0.008). They also had a lower level of disease activity (0.41 vs. 0.57, p = 0.006) and received fewer antimalarials (67.7% vs. 85.5%, p = 0.023) and immunosuppressive drugs (61.3% vs. 78.2%, p = 0.044), but they had higher organ damage scores (1.37 vs. 0.47, p < 0.001) and higher mortality rates/100-person year (3.2 vs. 1.1, p = 0.015). After adjusting for disease duration and baseline clinical variables, the late-onset patients only had lower rate of nephritis (p = 0.002), but still received fewer immunosuppressive drugs (p = 0.005) and had a higher mortality rate (p = 0.037). Conclusions In this sex- and year at diagnosis-matched controlled study, after adjusting for disease duration and baseline clinical variables, the late-onset SLE patients had less renal involvement and received less aggressive treatment, but had a higher mortality rate than the early-onset patients.

Paclitaxel-induced diffuse scleroderma with possible scleroderma-renal crisis: a case report and literature review of taxanes-induced scleroderma.

INTRODUCTION/OBJECTIVES: Scleroderma is a rare complication in taxanes therapy. Although individual cases of taxanes-induced scleroderma have been reported, the clinical manifestation and treatment outcomes were reviewed and summarized rarely. This study reported a patient who developed diffuse scleroderma and possible scleroderma renal crisis after paclitaxel therapy for ureter cancer. METHOD: A PubMed literature review on published cases of taxanes-induced scleroderma up until April 2022 was included for analysis. RESULTS: The search identified 27 patients with adequate information for analysis. Of the 28 patients, including the one presented here, 22 were female. Peripheral edema was the most common symptom in all but one patient, and often accompanied by erythema in 11. Symptoms usually occurred in half of the patients within the 4th course of treatment. Skin lesions gradually progressed to skin fibrosis, and extended proximally. Internal organ involvements were uncommon. Antinuclear antibody tests were positive occasionally, but anti-Scl70 and anti-centromere usually were negative. Taxanes therapy was discontinued, continued and unavailable in 21, 3, and 4 patients, respectively. Corticosteroids for skin lesions with or without immunosuppressive drugs were given to 15 patients. Of 25 patients with available skin outcomes, 19 improved. There was no significant skin improvement between those who did or did not receive skin treatment (62.5% vs. 75.0%, p = 0.37). Skin usually improved after discontinuing taxanes. CONCLUSION: Taxanes-induced scleroderma is different from idiopathic scleroderma. Physicians should be aware of this condition in order to provide early diagnosis and apply appropriate management in order to avoid serious complications from severe skin sclerosis. Key Points • Scleroderma is a rare but unique and serious complication of taxanes therapy • Skin manifestations and distribution are similar to idiopathic scleroderma, but vascular phenomenon, internal organ involvement and scleroderma-associated auto-antibodies are presented rarely. Skin improvement usually occurs shortly after discontinuing taxanes • The role of immunosuppressive therapy in treating taxanes-induced scleroderma is not clear.

2018 update of the APLAR recommendations for treatment of rheumatoid arthritis.

AIM: To update recommendations based on current best evidence concerning the treatment of rheumatoid arthritis (RA), focusing particularly on the role of targeted therapies, to inform clinicians on new developments that will impact their current practice. MATERIALS AND METHODS: A search of relevant literature from 2014 to 2016 concerning targeted therapies in RA was conducted. The RA Update Working Group evaluated the evidence and proposed updated recommendations using the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach, to describe the quality of evidence and strength of recommendations. Recommendations were finalized through consensus using the Delphi technique. RESULTS: This update provides 16 RA treatment recommendations based on current best evidence and expert clinical opinion. Recommendations 1-3 deal with the use of conventional synthetic disease-modifying antirheumatic drugs. The next three recommendations (4-6) cover the need for screening and management of infections and comorbid conditions prior to starting targeted therapy, while the following seven recommendations focus on use of these agents. We address choice of targeted therapy, switch, tapering and discontinuation. The last three recommendations elaborate on targeted therapy for RA in special situations such as pregnancy, cancer, and major surgery. CONCLUSION: Rheumatoid arthritis remains a significant health problem in the Asia-Pacific region. Patients with RA can benefit from the availability of effective targeted therapies, and these updated recommendations provide clinicians with guidance on their use.

Effectiveness and Drug Survival of Anti-Tumor Necrosis Factor α Therapies in Patients With Spondyloarthritis: Analysis From the Thai Rheumatic Disease Prior Authorization Registry.

OBJECTIVE: This study aimed to evaluate the long-term effectiveness and safety of the first anti-tumor necrosis factor α therapy (TNFi) and to identify the associated factors of drug discontinuation in patients with spondyloarthritis. METHODS: This was a medical records review study. Patients with spondyloarthritis who were prescribed the first TNFi between December 2009 and October 2014 in the Rheumatic Disease Prior Authorization registry were enrolled. Baseline clinical data were retrieved. The Cox proportional hazards model was used to identify factors associated with discontinuation of drugs. RESULTS: Among 138 patients, 97 had ankylosing spondylitis (AS), and 41 had psoriatic arthritis (PsA). The effectiveness of TNFi in AS and PsA was 55% to 59% at 4 months and 75% to 96% at 3 years, as measured by a 50% decrease in the Bath Ankylosing Spondylitis Disease Activity Index from baseline. For PsA with peripheral arthritis, improvement of the joint count by 50% was observed in 61.8% of patients at 4 months and 100% at 3 years. Survival from TNFi was 63% for AS and 56% for PsA at 3 years. For AS, the factors associated with good response leading to discontinuation of TNFi were baseline patient global assessment 3 to 6/10 (hazard ratio [HR], 6.3) and the use of leflunomide (HR, 6.0) and infliximab (HR, 4.8). A good response (38.5%) was the most common cause of discontinuation of the first TNFi, followed by toxicity (28.2%), nonadherence (20.5%), and lack of effectiveness (12.8%). CONCLUSIONS: Ankylosing spondylitis and PsA responded well to TNFi during the 3-year follow-up. The retention rate was approximately 60% for AS and PsA. A good response to the first TNFi was the most common reason for discontinuation.

2016 updated Thai Rheumatism Association Recommendations for the use of biologic and targeted synthetic disease-modifying anti-rheumatic drugs in patients with rheumatoid arthritis.

AIM: In June 2015, the Thai Rheumatism Association (TRA) approved an update of its recommendation for the use of biologic disease-modifying anti-rheumatic drugs (bDMARDs) and targeted synthetic (tsDMARD) in the treatment of rheumatoid arthritis (RA) to cover those currently available in Thailand (etanercept, infliximab, golimumab, rituximab, tocilizumab, abatacept and tofacitinib). METHOD: A search of the literature was performed between January 2000 and June 2015. Existing RA recommendations, in relation to the use of bDMARDs and tsDMARD, were identified and evaluated by the AGREE II instrument prior to their use as a 'guide' for developing this TRA recommendation. An additional literature search was performed in order to answer specific clinical questions that could not be found in existing guidelines. RESULT: Thirteen recommendations were developed. They covered the use of RA classification criteria, the aim of RA treatment, when to initiate bDMARDs/tsDMARD or taper or switch them to other medications, as well as monitoring these drugs during their use. In addition, specific issues including their use and vaccination, malignancies, pregnancy and lactation, and perioperative period also were addressed. Public hearings were performed at the annual meeting of the TRA and of the Royal College of Physicians of Thailand. The recommendations were distributed to other professional associations related to RA management, as well as government sectors associated with the reimbursement policy, prior to development of the final version. CONCLUSION: These recommendations will help Thai rheumatologists prescribe bDMARDs and tsDMARD more appropriately when treating RA patients.

The Correlation of Muscle Biopsy Scores with the Clinical Variables in Idiopathic Inflammatory Myopathies.

OBJECTIVES: To compare the muscle pathology findings among subgroups of idiopathic inflammatory myopathies (IIM) patients, and to determine the correlations of muscle biopsy scores with muscle power and creatine kinase (CK). METHODS: The medical records of IIM patients consisting of the demographic data, clinical parameters and laboratory conducted were retrospectively reviewed. Their initial muscle biopsies were reviewed, and four domains were scored: inflammation, vascular, muscle, and connective tissue. RESULTS: Ninety-five IIM patients (28 patients with idiopathic polymyositis (PM) 9 idiopathic dermatomyositis (DM), 5 DM associated with malignancy, and 53 PM/DM associated with connective tissue disease) with median (IQR: Q1, Q3) disease duration of 1.2 (0.5, 3.1) months were included. No significant differences in initial muscle pathology findings and muscle pathology score among the subgroups were found. Muscle degeneration and endomysial fibrosis scores were negatively correlated with muscle power (r=-0.23 and-0.24, respectively, p<0.05) and positively correlated with CK (r=0.27 and 0.39, respectively, p<0.01). No significant correlation was detected either inflammation or vasculitis scores with muscle power and CK levels. CONCLUSION: In this study, muscle biopsy cannot be used to differentiate among subgroups of IIM patients. In addition, we found only modest correlation of muscle biopsy scores with muscle power and CK. Further study is necessary to confirm our findings.

Treatment considerations in patients with concomitant viral infection and autoimmune rheumatic diseases.

Widespread use of immunosuppressive drugs, both conventional disease-modifying antirheumatic drugs (cDMARDs) and biologic disease-modifying antirheumatic drugs (bDMARDs), in autoimmune rheumatic diseases (ARDs) has been found to be associated with the reactivation of underlying latent viruses. The clinical features of virus reactivation can sometimes mimic flare of the underlying ARDs. The correct diagnosis and management of such reactivation is crucial, as increasing the dose of immunosuppressive drugs to treat a presumed flare of underlying ARDs would probably be of no benefit, and it could exert a detrimental effect on the host. This review focused on the effects of immunosuppressive drugs on underlying chronic viral infections, particularly hepatitis B virus, hepatitis C virus, human immunodeficiency virus, varicella zoster virus, Epstein-Barr virus, cytomegalovirus, John Cunningham (JC) virus, Kaposi sarcoma-associated herpesvirus, and human papillomavirus in patients with ARDs. It also covered the effect of interferon-α, which is used to treat chronic hepatitis infection, and the induction of autoimmunity.

Study for Updated Gout Classification Criteria: Identification of Features to Classify Gout.

OBJECTIVE: To determine which clinical, laboratory, and imaging features most accurately distinguished gout from non-gout. METHODS: We performed a cross-sectional study of consecutive rheumatology clinic patients with ≥1 swollen joint or subcutaneous tophus. Gout was defined by synovial fluid or tophus aspirate microscopy by certified examiners in all patients. The sample was randomly divided into a model development (two-thirds) and test sample (one-third). Univariate and multivariate association between clinical features and monosodium urate-defined gout was determined using logistic regression modeling. Shrinkage of regression weights was performed to prevent overfitting of the final model. Latent class analysis was conducted to identify patterns of joint involvement. RESULTS: In total, 983 patients were included. Gout was present in 509 (52%). In the development sample (n = 653), the following features were selected for the final model: joint erythema (multivariate odds ratio [OR] 2.13), difficulty walking (multivariate OR 7.34), time to maximal pain <24 hours (multivariate OR 1.32), resolution by 2 weeks (multivariate OR 3.58), tophus (multivariate OR 7.29), first metatarsophalangeal (MTP1) joint ever involved (multivariate OR 2.30), location of currently tender joints in other foot/ankle (multivariate OR 2.28) or MTP1 joint (multivariate OR 2.82), serum urate level >6 mg/dl (0.36 mmoles/liter; multivariate OR 3.35), ultrasound double contour sign (multivariate OR 7.23), and radiograph erosion or cyst (multivariate OR 2.49). The final model performed adequately in the test set, with no evidence of misfit, high discrimination, and predictive ability. MTP1 joint involvement was the most common joint pattern (39.4%) in gout cases. CONCLUSION: Ten key discriminating features have been identified for further evaluation for new gout classification criteria. Ultrasound findings and degree of uricemia add discriminating value, and will significantly contribute to more accurate classification criteria.

Periodontal disease in Thai patients with rheumatoid arthritis.

AIM: To evaluate the prevalence and severity of periodontal disease in patients with rheumatoid arthritis (RA) who attended a rheumatology clinic in a university hospital. METHODS: All consecutive patients with RA who attended the rheumatology clinic between June 2009 and January 2010 were asked to enroll in this study. All participants answered questionnaires, which included demographic data, medical history, medications used and smoking habits. A full mouth periodontal examination, including gingival index, plaque index, probing pocket depth and clinical attachment level was performed. Only cases that had at least 20 teeth were included in this study. Rheumatoid arthritis parameters, including number of tender and swollen joints, erythrocyte sedimentation rate, the presence of rheumatoid factor (RF), hand radiographs, Disease Activity Index (DAS) and health status using the Thai Health Assessment Questionnaire (HAQ), were determined. The association between RA parameters and periodontal condition was examined. RESULTS: There were 196 participants (87.2% female) with a mean age of 51.7 ± 9.70 years, mean disease duration of 9.62 ± 7.0 years and mean DAS score of 4.64 ± 1.25. Eighty-two per cent were RF-positive. Moderate and severe periodontitis were found in 42% and 57%, respectively. Higher age, male gender, previous or current smoking and high level of plaque score were associated with severe periodontal disease. No differences in RA parameters were found between groups of patients who had moderate and severe periodontitis. CONCLUSIONS: We found a high prevalence of periodontitis in Thai patients with RA. However, there was no association between RA parameters and periodontal conditions.

Comparison of proteinuria determination by urine dipstick, spot urine protein creatinine index, and urine protein 24 hours in lupus patients.

BACKGROUND: A simple, convenient, and accurate method for detecting urine protein excretion in lupus nephritis is crucial. OBJECTIVES: The objectives of the study were to determine the sensitivity and the specificity of the qualitative urine dipstick test value to detect 0.50 g or greater of the quantitative 24-hour urine protein (24-hUP) in lupus patients, to evaluate an overall agreement of the dipstick test results and the magnitude of 24-hUP, and to examine the correlation between the spot urine protein creatinine index (S-UPCI) and the 24-hour UPCI with that of the 24-hUP. METHODS: A prospective study was conducted in 92 patients with lupus. All dipstick test values from 5 dipstick assays (Bayer, Roche, Meditest USA, Standard Diagnostics, and Arkray) and the S-UPCI were obtained within 6 hours of the 24-hUP collection. Of 149 urine samples, only 39% were collected properly and were used for analysis. RESULTS: The sensitivity and specificity of a ≥ 2+ dipstick test result to detect 0.50 g or greater 24-hUP were 56-% to 80% and 67% to 92%, respectively. The agreement of the urine dipstick test values and the magnitude of 24-hUP was fair (κ = 0.23-0.32). The correlation between the S-UPCI and the 24-hUP was 0.83 (P < 0.0001), and that of the 24-hour UPCI and the 24-hUP was 1 (P < 0.0001). Using 24-hUP 2 g/d or less, the bias ± 1.96 SD of the difference of S-UPCI and 24-hUP was 0.23 (SD, 0.96) g. CONCLUSIONS: A ≥ 2+ dipstick test is relatively sensitive to detect significant proteinuria, but it is poorly correlated with quantitative 24-hUP. The S-UPCI and the 24-hUP can be used interchangeably for follow-up in lupus nephritis patients with proteinuria of less than 2 g/d.

Inappropriate prescription of allopurinol in a teaching hospital.

BACKGROUND: Allopurinol is a drug that is widely used to treat hyperuricemia, but it is often prescribed inappropriately. OBJECTIVE: The authors conducted a study to look for the appropriate allopurinol prescription and diagnosis of gout in the out-patient clinics at a university hospital. MATERIAL AND METHOD: One hundred and forty-five patients who were newly prescribed allopurinol (128 males and 17 females, mean +/- SD age of 58.5 +/-14.1 years) were enrolled in this study. RESULT: Only 77 (53.1%) received allopurinol with appropriate indications. Thirty-eight patients (26.2%) did not have allopurinol dose adjustment according to the patients' creatinine clearance. Among 131 patients, prescribed allopurinol for the diagnosis of gout, only 55 (42.0%) were diagnosed in accordance with the American Rheumatism Association criteria. CONCLUSION: Inappropriate use of allopurinol (both the indication and prescribed dosage) and inappropriate diagnosis of gout are major problems even in a large teaching hospital. An educational campaign program is warranted for achieving appropriate diagnosis of gout, and eliminating the inappropriate use of allopurinol.

Validation of the Thai version of a screening questionnaire for detection of systemic lupus erythematosus.

OBJECTIVE: The present study was performed to assess the sensitivity and specificity of the translation questionnaire for Systemic Lupus Erythematosus (SLE) in Thai speaking populations. MATERIAL AND METHOD: The 10- item questionnaire was applied to out-patients at the rheumatology clinic of the Chiang Mai University Hospital. One hundred and thirty-nine SLE, 109 Rheumatoid Arthritis (RA), and 35 Scleroderma (Scl) patients, as well as 88 Healthy Controls (HC) were enrolled into the present study. RESULTS: All subjects completed the questionnaire within 2 minutes. A positive response to three or more questions of the questionnaire gave a sensitivity and specificity of 92.81% and 76. 39%, respectively, and was comparable to the original version. CONCLUSION: This Thai-version of the screening questionnaire should be applied in the general population to determine the prevalence of SLE.

Identification of emerging human-pathogenic Pythium insidiosum by serological and molecular assay-based methods.

Pythium insidiosum is a pathogen that causes disease in both animals and humans. Human infection is rare; however, when it does occur, most patients, especially those having underlying hemoglobinopathy syndromes, such as thalassemia, exhibit a severe form. We identified four isolates of P. insidiosum. Two were recovered from tissue biopsy specimens from thalassemic and leukemic patients, one was derived from brain tissue from a thalassemic patient, and another was isolated from a corneal ulcer from a fourth patient. Western blotting and an enzyme-linked immunosorbent assay (ELISA) were performed with a serum sample derived from one thalassemic patient. The methods used to identify the P. insidiosum isolates were based on morphology, nucleic acid sequencing, and a PCR assay. To confirm the identification, portions of the 18S rRNA genes of these four isolates were sequenced. The sequences were shown to be homologous to previously described P. insidiosum DNA sequences. In addition, PCR amplification of the internal transcribed spacer region specific for P. insidiosum was positive for all four isolates. The ELISA with the serum sample from the thalassemic patient gave a positive result from a serum dilution of 1:800. Finally, Western immunoblotting with this serum sample showed positive immunoglobulin G recognition for proteins of 110, 73, 56, 42 to 35, 30 to 28, 26, and 23 kDa. The results of this study show that both molecularly based diagnostic and serodiagnostic techniques are useful for the rapid identification of human pythiosis. The predominant antigens recognized by Western blotting may be useful in the development of a more sensitive and specific diagnostic tool for this disease.

Human pythiosis, a rare cause of arteritis: case report and literature review.

OBJECTIVE: To present a case of Pythium insidiosum arteritis and review reported cases of human pythiosis. METHODS: Search of MEDLINE (PubMed) and the Thai Index Medicus was performed using the words "pythiosis" and "pythium." Search of abstracts from a scientific meeting also was performed. Only reported cases in the English or Thai language, with enough clinical information for analysis, were included. RESULTS: There were a total of 32 cases of human pythiosis, with 31 identified in the literature. Twenty-five cases were Thai. Twenty of 21 patients with known occupations were farmers. There was ocular infection in 9 cases, subcutaneous infection in 5 cases, arterial infection in 17 cases, and cardiopulmonary infection in 1 case. Thalassemia was a common associated finding. In addition to systemic antifungal therapy, surgery was required in most cases. The highest mortality rate was seen in the arteritic form, particularly in patients with arterial lesions proximal to the superficial femoral artery. CONCLUSIONS: Human pythiosis is rare. Physicians should be suspicious of pythiosis in individuals, particularly farmers in Thailand, who develop nonhealing cutaneous ulcers or keratitis, which do not respond to conventional treatment. P insidiosum infections should be listed as a rare cause of infectious arteritis.

Kaposi's sarcoma in rheumatic diseases.

OBJECTIVE: To review the clinical features and outcome of all reported cases of Kaposi's sarcoma in patients with rheumatic diseases. METHODS: In addition to our patient, we identified cases from a Medline search between the years 1966 and 2002. Cases associated with human immunodeficiency virus infection were excluded. RESULTS: Including our patient, there were a total of 25 cases reported (11 men and 14 women). Rheumatoid arthritis was present in 8 cases, polymyositis/dermatomyositis in 5, vasculitis syndromes in 5, systemic lupus erythematosus in 3, polymyalgia rheumatica in 2, and 1 each of undifferentiated connective tissue disease and Behcet disease. All but 1 patient had been given systemic corticosteroids for a duration that ranged from 6 weeks to 22 years, and immunosuppressive drugs from 25 days to 3.5 years. The Kaposi's lesions usually involved the skin on the extremities; internal organ involvement occurred in 7 cases. Most lesions responded to a decreasing dosage of corticosteroids and immunosuppressive drugs, or to the administration of radiation or cytotoxic therapy. Six patients died, 4 of which were related to the progression of Kaposi's sarcoma. CONCLUSION: Kaposi's sarcoma in patients with rheumatologic conditions is rare. The clinical features are similar to those with classical Kaposi's sarcoma. Tumor regression usually occurs with decreasing corticosteroids and/or immunosuppressive drugs, local irradiation, or cytotoxic therapy.

Serum muscle enzymes, muscle pathology and clinical muscle weakness: correlation in Thai patients with polymyositis/dermatomyositis.

The clinical correlation between serum muscle enzymes, muscle pathology and muscle weakness was studied in 100 Thai patients (22 males and 78 females) with polymyositis (PM) and dermatomyositis (DM). Their mean +/- SD age and duration of disease were 45.0 +/- 13.9 years and 6.3 + 13.4 months, respectively. There was idiopathic PM in 37 cases, idiopathic DM in 13, PM/DM associated with malignancy in 5 and PM associated with connective tissue disease in 45. Serum muscle enzymes including creatine phosphokinase, lactate dehydrogenase and aspartate aminotransferase were elevated in 87 per cent, 92 per cent, and 82 per cent of cases, respectively. Abnormal electromyographic findings that were compatible with inflammatory myopathy were found in 76 per cent of cases. Seventy-seven per cent had an abnormal muscle biopsy that was consistent with polymyositis. There was a significant correlation between serum muscle enzymes and muscle pathology (p < 0.01). The degree of muscle weakness correlated better with the degree of muscle destruction (p = 0.01) than the degree of muscle inflammation (p = 0.03). The erythrocyte sedimentation rate showed no correlation with serum muscle enzymes, muscle pathology or muscle weakness.