Ratanavadee Nanagara

Right ventricular systolic pressure assessed by echocardiography: a predictive factor of mortality in patients with scleroderma.

Pulmonary arterial hypertension (PAH) is a well‐known complication of systemic sclerosis (SSc). Doppler echocardiographic screening for the detection of PAH (by measuring right ventricular systolic pressure [RVSP]) is therefore recommended for all patients with SSc. However, the validity of RVSP as a predictor of mortality in patients with SSc is not well established.

Pericardial fluid profiles of pericardial effusion in systemic sclerosis patients.

BACKGROUND Cardiac involvement is one of clinical presentations in systemic sclerosis (SSc). The pericardial fluid profile and the causes of pericardial effusion were our focus. OBJECTIVES To demonstrate the characteristics and causes of pericardial effusion in SSc. METHODS A descriptive retrospective study was performed on SSc patients with symptomatic pericardial effusion at Srinagarind Hospital, Khon Kaen University, Thailand, between January 1, 2000 and December 31, 2010. We excluded pericardial fluid detected by screening echocardiography. RESULTS Thirty medical records of SSc patients with pericardial effusion were reviewed. The respective mean age and median duration of disease at the time of effusion detection was 52.2±10.8 years and 11.4 months (IQR 0.03-59.1). The female to male ratio was 3.3:1. The most common signs and symptoms included tachycardia (65%) and right-sided heart failure (30%). One patient was diagnosed with cardiac tamponade due to tuberculous pericarditis. Pericardiocentesis and fluid analysis were performed in 9 patients, all of whom had pericardial fluid lactate dehydrogenase (LDH) <200 U/L and a fluid-serum LDH ratio <0.6. Most of the cases (87.5%) had a fluid-serum total protein ratio <0.5. The median white blood cell (WBC) count was 10 cell/mm3 (IQR 6-830), one-third of which was predominated by mononuclear cells. The pericardial biopsy in 8 patients revealed 4 pericardial fibrosis, 2 non-specific inflammation and 2 granuloma. CONCLUSIONS The pericardial fluid profile for SSc was similar to the exudative profile of pleural fluid. Cardiac tamponade was rare. Most pathological findings of pericardial tissue were associated with fibrosis related to the disease itself.

Asymptomatic cardiac involvement in Thai systemic sclerosis: prevalence and clinical correlations with non-cardiac manifestations (preliminary report)

OBJECTIVE To determine the prevalence of asymptomatic cardiac involvement and its correlation with non-cardiac manifestation in Thai SSc patients. METHODS A cross-sectional study was carried out between January 2012 and June 2013 at Srinagarind Hospital, Khon Kaen University, Thailand, on adult SSc patients without signs or symptoms suggestive of cardiac involvement. We excluded those with overlap syndrome, having serum creatinine >123.8 µmol/l, history of cardiac diseases, any atherosclerosis risk factors and receiving angiotensin-converting enzyme inhibitors. Non-invasive tests related to cardiac involvement were performed, including: echocardiography, ECG, chest X-ray, inflammatory biomarkers, cardiac enzymes and N-terminal prohormone of brain natriuretic peptide. RESULTS A total of 103 SSc patients were enrolled, 61.2% of whom were in the subset. Of these, 63 patients had at least one test abnormality (prevalence 61.2%; 95% CI 51.6, 70.7). The two leading cardiac abnormalities were diastolic dysfunction (44.7%) and elevation of cardiac enzymes (36.9%). The only predictor for cardiac involvement per multivariate analysis was the dcSSc subtype with a higher modified Rodnan skin score, and shorter disease duration (odds ratio = 3.37; 95% CI 1.07, 10.65). Compared with the limited subtype, dcSSc was also significantly associated with elevated cardiac enzyme and prolonged distance between a Q wave and T wave in an ECG (QT interval). CONCLUSION Asymptomatic cardiac involvement in Thai SSc was not uncommon, and the most common finding was diastolic dysfunction. Elevated cardiac enzymes were found in one-third of the patients, which correlated with the dcSSc subtype with a higher modified Rodnan skin score and shorter disease duration, suggestive of early myocardial microcirculation disruption. Long-term follow-up was performed to elucidate the clinical implication of these abnormalities.

Prognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in Thailand.

Aims:  Pulmonary arterial hypertension (PAH) is a major complication and cause of death in systemic sclerosis (SSc). Our objective was to identify the predictive factors of mortality and the 2‐year survival rate among Thai sufferers of PAH‐SSc.

Clinical differences between Thai systemic sclerosis patients with positive versus negative anti-topoisomerase I.

Anti‐topoisomerase I antibody (ATA) carries an increased risk of systemic sclerosis (SSc) internal organ involvement. There have been no published comparisons of the clinical characteristics of patients positive and negative for ATA in Thailand, where the positive rate for ATA is higher than among Caucasians.

Incidence rate and causes of infection in Thai systemic sclerosis patients.

Infection is a common cause of death in systemic sclerosis (SSc) but despite immunosuppressant therapy, there are few reports of opportunistic infection.

Salmonella group D septic arthritis and necrotizing fasciitis in a patient with rheumatoid arthritis and diabetes mellitus.

Necrotizing fasciitis is an uncommon manifestation of Salmonella infection. We report a case of Salmonella group D septic arthritis complicated with necrotizing fasciitis in a 51-year-old man who had noninsulin dependent diabetes mellitus and rheumatoid arthritis. He presented with fever and severe right hip pain complicated with septic shock and disseminated intravascular coagulation. Crepitation was noticed upon physical examination, and plain films showed numerous air bubbles in the soft tissue around the hip joint. Prompt antibiotic therapy and surgical management were performed with a successful response. The causative organism was Salmonella group D. Antibiotic was given in the total course of 3 months, and there was no relapse of salmonellosis after 2 years follow up. The differential diagnosis of causes of non-clostridial crepitant soft tissue and muscle infections must include Salmonella, especially in patients who have underlying diseases or are taking immunosuppressive drugs. Prompt management is needed to reduce mortality and morbidity. Long-term suppressive therapy may be needed to prevent relapse.

Effectiveness of add-on therapy with domperidone vs alginic acid in proton pump inhibitor partial response gastro-oesophageal reflux disease in systemic sclerosis: randomized placebo-controlled trial

Objectives. Twice-daily dosing of proton pump inhibitor (PPI), the standard therapy for gastro-oesophageal reflux disease (GERD), is an effective therapy for GERD in SSc. The aim of this study was to compare the efficacy of omeprazole in combination with domperidone vs in combination with algycon in reducing the severity and frequency of reflux symptoms of PPI partial response (PPI-PR) GERD in SSc. Methods. Adult SSc patients having PPI-PR GERD were randomly assigned to receive domperidone plus algycon placebo or algycon plus domperidone placebo in a 1:1 ratio plus omeprazole for 4 weeks. The assessment included severity of symptom grading by visual analogue scale, frequency of symptoms by frequency scale for symptoms of GERD and quality of life (QoL) by EuroQol five-dimensions questionnaire scoring. Results. One hundred and forty-eight SSc-GERD patients were enrolled, of whom 88 had PPI-PR. Eighty cases were randomized for either domperidone (n = 38) or algycon (n = 37) therapy. The majority in both groups had the diffuse SSc subset. At the end of the study, no significant difference in symptom grading was found between groups. After treatment and compared with baseline, the severity of symptoms, frequency scale for symptoms of GERD and QoL significantly improved in both groups. Five (13.2%) and 8 (21.6%) respective cases in the domperidone and algycon groups did not respond. Conclusion. The prevalence of PPI-PR GERD is common. Domperidone and algycon are equally effective treatments in combination with omeprazole. However, ∼17% of patients were non-responsive, so the effectiveness of domperidone, algycon and PPI combination therapy should be further investigated. Trial registration: https://clinicaltrials.gov (NCT01878526).

The short-term effect of gloving in combination with Traditional Thai Massage, heat, and stretching exercise to improve hand mobility in scleroderma patients

Background: Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disorder characterized by autoimmune activation, microvascular endothelium damage, and excessive collagen proliferation. The most affected hand presents claw hand deformity and microvascular disease. Deformed hands can cause functional disability and decrease the quality of life. A daily home program can improve mobility of scleroderma patients. Objective: We sought to determine the effect of a daily home exercise program on hand mobility among scleroderma patients. Materials and Methods: This was a randomized control trial. Twenty-eight participants were divided into two groups, both of which received the same daily home treatment: Group 1 with gloves (n = 14) and Group 2 without gloves (n = 14). The 2-week daily home program combined traditional Thai massage (TTM) with stretching exercises and heat. Hand mobility was assessed using hand mobility in scleroderma (HAMIS). The study was conducted in patients who were already on vasodilator drugs. Results: Both groups showed a significant improvement in hand mobility after 2 weeks of daily home exercise program (P < 0.05). Wearing the glove, however, resulted in better thumb mobility. Conclusions: A daily home exercise program improved hand mobility among patients with scleroderma and wearing gloves may improve thumb mobility.

Survival rate among Thai systemic lupus erythematosus patients in the era of aggressive treatment

Background:  The major cause of death in systemic lupus erythematosus (SLE) is due to the disease activity itself or infection. It is uncertain whether the treatment approach during the last decade prolonged survival in SLE.