Calcinosis universalis and breast cancer: A distinctive association.

Calcinosis universalis (CU) is characterised by diffuse deposition of insoluble calcium salt in the skin, subcutaneous tissue or organs. Calcium deposits in the breast may be associated with an increased risk for developing breast cancer. We present a case of a 65-year-old woman diagnosed with CU secondary to undifferentiated connective tissue disease. She developed progressive calcification of her skin, which did not improve with oral medications aimed at reducing the calcification. Investigations to look for possible causes of calcification were all unremarkable. During follow-up, calcification was also found in both her breasts. Initial mammography was reported as fibroadenoma. However, 3 years later, she returned with metastatic breast cancer which presented with a massive pleural effusion of the right lung. Calcinosis universalis should now be considered as a risk factor for breast cancer.

Performance of the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus in a multiethnic Malaysian cohort.

AIM: To evaluate the performance of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) in a multi-ethnic Malaysian cohort and to compare it against the Systemic Lupus International Collaborating Clinics (SLICC) 2012 and ACR 1997 criteria. METHOD: We conducted a retrospective observational study of 205 patients with a diagnosis of SLE and 100 controls who formed the validation cohort. The sensitivity and specificity of the three classification criteria were evaluated and a further sub-analysis was performed in patients with early disease and among the various ethnicities. RESULTS: The sensitivities and specificities of the three classification criteria are as follows: EULAR/ACR (90.8%; 94%), SLICC 2012 (96.1%; 94%), and ACR 1997 (82%; 96%). Among patients with early disease, the sensitivity of the SLICC 2012 was higher than that of EULAR/ACR and ACR 1997 (98% vs 94% and 86%); however, the specificity of EULAR/ACR and ACR 1997 were similar (95.2%) and higher than the SLICC 2012 (93.5%). The SLICC 2012 had higher sensitivity than that of the EULAR/ACR among the Malays (94% vs 90%), Chinese (98% vs 90%), and Indians (100% vs 95%). The specificity of the EULAR/ACR and SLICC 2012 were similar in the Malay and Chinese (93.3% each, and 92% vs 94.6%). CONCLUSION: The EULAR/ACR performed well in our cohort. The EULAR/ACR and SLICC 2012 showed higher sensitivity than the ACR 1997, and the EULAR/ACR showed similar specificity to the ACR 1997 and SLICC 2012 overall, in early disease, and across the different ethnicities.

Pulmonary manifestation as initial presentation for systemic lupus erythematosus.

We report a 29-year-old Malay man who had pulmonary manifestations as an initial presentation for systemic lupus erythematosus. He had prolonged hospitalization and was treated with intensive care therapy with immunosuppressants.

Real-world clinical experience of biological disease modifying anti-rheumatic drugs in Malaysia rheumatoid arthritis patients.

The effect of biologic disease modifying anti-rheumatic drugs (bDMARDs) in treating rheumatoid arthritis (RA) in real-world clinical practice remains unknown in Southeast Asia. We aimed to assess the efficacy and safety of bDMARDs among Malaysian RA patients treated in routine clinical practice. A retrospective medical chart review of RA patients from 11 government hospitals were conducted from January 2003 to January 2014. A standardized questionnaire was used to abstract patient's demographic, clinical and treatment data. Level of disease activity was measured by DAS28 collected at baseline, 3, 6 and 12 months. Three hundred and one patients were available for analysis, mean age 41 (SD, 10.8) years, mean RA duration 12.3 (SD, 6.9) years and 98% had history of two or more conventional-synthetic DMARDs. There were 467 bDMARD courses prescribed with mean bDMARDs duration use of 12.9 months (SD 14.7). Tumour necrosis factor alpha inhibitors were the most common prescribed bDMARDs (77.1%), followed by Tocilizumab (14.6%) and Rituximab (8.4%). We observed significant improvement in mean DAS28 values from baseline to 3, 6 and 12 months (p < 0.001). Overall, 16.9% achieved DAS28 remission at 6 months. A third (35.6%) of patients reported adverse events, three commonest being infections (46.5%), allergy (22.9%) and laboratory abnormalities (12.9%). 3.7% of our patients had tuberculosis. Biologic DMARDs were effective in treating RA in real-world practice in Malaysia, despite a lower remission rate compared to developed countries. Except for higher rates of tuberculosis, the AEs were similar to the published reports.

Management of rheumatoid arthritis in clinical practice using treat-to-target strategy: Where do we stand in the multi-ethnic Malaysia population?

To evaluate the achievement of treat-to-target (T2T) strategy in rheumatoid arthritis (RA) and identify factors associated with failed treatment target in a public rheumatology center. A cross-sectional study was conducted from June 2015 to February 2016. RA patients with disease duration greater than 2 years and under T2T for over a year were invited to the study. Demographic, clinical data, disease activity score of 28 joints (DAS28), and clinical disease activity index (CDAI) were collected in a single routine clinic visit. Treatment target was defined as DAS28 <3.2 or CDAI ≤10. Retrospective chart review was performed to determine reasons of failed treatment target. A total of 371 patients were recruited and 87.1% were female. Mean age and duration of RA were 53.5 years (SD 10.3) and 9.1 years (SD 6.6), respectively. Ethnic distribution was 49% Chinese, 27% Malay, and 24% Indian. T2T was achieved in 81.7% of the cohort. Non-Chinese ethnicity, positive rheumatoid factor, and treatment with three disease modifying anti-rheumatic drugs (DMARDs) were associated with failed treatment target. After controlling for covariates, Malay ethnicity (OR 2.96; 95% CI 1.47-5.96) and treatment with three DMARDs (OR 2.14; 95% CI 1.06-4.35) were associated with failed treatment target. There was no association between age, gender, duration of RA, BMI, smoking status, anti-citrulinated cyclic peptide, and achievement of T2T. The most common reasons of failed treatment target were inability to escalate DMARDs due to side effects (18.8%), lack of biologics fund (15.6%), and persistent disease despite optimum treatment (14.1%). T2T was successfully implemented. Malay patients need aggressive treatment adaptation to achieve optimal outcome.

Takayasu Arteritis in Major Rheumatology Centers in Malaysia.

OBJECTIVE: There is paucity of data for Takayasu arteritis (TAK) among South Asians. We aimed to evaluate the clinical features, angiographic findings, as well as treatment and outcome of TAK among Malaysian multiethnic groups. METHODS: This is a retrospective review of 40 patients with TAK seen in major rheumatology centres in Malaysia between April 2006 and September 2013. RESULTS: Majority were female patients (92.5%), with a female-to-male ratio of 12:1. Median duration of disease from diagnosis was 66 months (interquartile range, 33-177 months). Fifteen (37.5%) were Malays, 9 (22.5%) each were Indians and indigenous from East Malaysia and 7 (17.5%) were Chinese. Indian and indigenous from East Malaysia were overrepresented in this disease. The mean (SD) age of symptom onset and diagnosis were 25.5 (8.1) and 27.4 (8.4), respectively. The 3 most common clinical presentations at diagnosis were diminished or absent pulse, which occurred in 80% of the patients, followed by blood pressure discrepancy (60%) and arterial bruit (52.5%). There was no difference in clinical presentation among ethnic groups. The subclavian artery was the commonest vessel involved (72.5%), followed by the carotid artery (65%) and renal artery (47.5%). Eight patients had coronary artery involvement, and 2 patients had pulmonary artery involvement. Type I arterial involvement was the commonest (80.0%), followed by type IV (35%), present in isolation or mixed type. Glucocorticoid was the main medical treatment (90.0%). Nineteen patients (47.5%) underwent revascularization procedures. Five patients died during the follow-up period. CONCLUSIONS: The Malaysian TAK cohort had similarities with and differences from other published TAK cohort. A nationwide TAK registry is needed to determine the prevalence of the disease among different ethnic groups.