54 research outputs found
Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody
No full textRaiwin Suwannamajo, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Chingching Foocharoen Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, ThailandCorrespondence: Chingching Foocharoen, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand, Tel +6643363746, Email [email protected]: Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) overlap with systemic sclerosis (SSc) is uncommon. We aimed to determine the incidence of AAV and define clinical outcomes relevant to asymptomatic screening positive for ANCA in SSc after 2 years of follow-up.Patients and Methods: The study was a cohort study of 185 Thai adult SSc patients testing for ANCA and having a 2-year follow-up at the Scleroderma Clinic, Khon Kaen University, Thailand. The incidence of AAV and outcomes of those who tested positive for ANCA were evaluated.Results: A total of 185 SSc patients were tested for ANCA, of whom 21.6% were positive for either cytoplasmic ANCA, perinuclear ANCA (p-ANCA), anti-myeloperoxidase (anti-MPO), or anti-proteinase3 antibody. Only one 52-year-old female patient with dcSSc, negative for initial ANCA test, developed AAV (microscopic polyangiitis) 7 months after the first ANCA test for an incidence of AAV of 0.27 per 100-person-years (95% CI 0.01– 1.5). She was positive for p-ANCA and anti-MPO. Eight of those who had an initial test were positive for ANCA and underwent a repeated test. Only two cases persisted as positive for ANCA (1 anti-MPO and 1 anti-PR3) and had no clinicals suspicious of vasculitis. Four cases that had ANCA turned to a negative result.Conclusion: AAV is a rare complication in SSc, so ANCA may not have any role as a screening test for AAV as it cannot predict the development of AAV in SSc. We suggest testing for ANCA only in SSc patients with clinicals suspicious of AAV.Keywords: systemic sclerosis, scleroderma, anti-neutrophilic cytoplasmic antibody, ANCA- associated vasculitis, vasculiti
GI Manifestation in Scleroderma
No full textThe gastrointestinal (GI) system frequently experiences complications in systemic sclerosis. Pathological changes in the GI system can be revealed from the early onset of the disease, such as esophageal hypomotility, to the late stage of the disease, where severe complications like intestinal pseudo-obstruction may occur. Systemic sclerosis can cause pathological changes throughout the entire gastrointestinal tract. Common symptoms include dysphagia, choking, heartburn, hoarseness, abdominal distension, diarrhea, constipation, and fecal incontinence. Oropharyngeal involvement in systemic sclerosis presents as weakened pharyngeal muscles, leading to oropharyngeal dysphagia, choking, food leakage, and aspiration, attributed to the dysfunction between the pharyngeal muscles and the upper esophageal sphincter due to muscle weakness. However, weak pharyngeal muscles may also be a clinical feature of the systemic sclerosis-polymyositis overlap syndrome. Esophageal involvement is frequently observed, with conditions such as esophageal hypomotility and lower esophageal sphincter incompetence, leading to early symptoms of dysphagia, gastroesophageal reflux, and potential complications such as esophagitis, esophageal stricture, Barrett’s esophagus, and aspiration pneumonia. Furthermore, gastric involvement includes gastric antral vascular ectasia, commonly known as “watermelon stomach,” which leads to gastrointestinal bleeding and chronic anemia. Intestinal manifestations comprise malabsorption, intestinal pseudo-obstruction, and bacterial overgrowth. Additionally, wide-mouthed diverticulosis and fecal incontinence are frequently found. Liver involvement includes primary biliary cirrhosis, most prevalent in limited cutaneous systemic sclerosis or certain overlap syndromes rather than diffuse cutaneous systemic sclerosis. The severity of these changes varies among patients, ranging from asymptomatic to severe conditions, which may result in malnutrition. Severe and chronic GI involvement significantly impacts daily life, making normal eating difficult, diminishing the quality of life, and potentially causing depression. Managing patients with systemic sclerosis who have GI involvement requires a multidisciplinary approach, involving collaboration among rheumatologists, gastroenterologists, nutritionists, surgeons, and possibly psychiatrists. This teamwork is essential to achieve successful patient care outcomes
Spontaneous pneumothorax from cryptococcal pneumonia in systemic sclerosis: a case report
No full textAbstract Introduction Spontaneous pneumothorax is usually found in people with systemic sclerosis who have extensive pulmonary fibrosis with enlarged sub-pleural blebs. We report a case of spontaneous pneumothorax caused by cryptococcal pneumonia in a patient with systemic sclerosis with minimal sub-pleural emphysema. Case presentation A 49-year-old Thai man with underlying limited cutaneous systemic sclerosis presented with acute low-grade fever, progressive dyspnea and right pleuritic chest pain for five days. Our patient had pulmonary fibrosis with bronchiectasis of both lower lungs related to this underlying disease. He received only low-dose steroid therapy, without any immunosuppressant. A chest radiograph revealed right lung pneumothorax with cloudy yellow color pleural fluid. Cryptococcal pneumonia was diagnosed by positive identification of the cryptococcal antigen in the serum and pleural fluid. His symptoms improved after intercostal drainage and fluconazole therapy. Conclusion Infection can exacerbate symptoms in patients with systemic sclerosis with sub-pleural emphysema, thereby triggering a spontaneous pneumothorax. Pleural fluid--present but not initially seen because of the pneumothorax--could be a clue to a pre-existing pulmonary infection.</p
Clinical course and factors associated with progressive acro-osteolysis in early systemic sclerosis: a retrospective cohort study
No full textAbstract To examine clinical course of early systemic sclerosis (SSc) and identify factors for progression of acro-osteolysis by a retrospective cohort study. Dual time-point hand radiography was performed at median interval (range 3.0 ± 0.4 years) in 64 recruited patients. Progressive acro-osteolysis was defined as the worsening of severity of acro-osteolysis according to rating scale (normal, mild, moderate, and severe). Incidence of the progression was determined. Cox regression was analyzed for the predictors. A total of 193.6 per 100 person-years, 19/64 patients had progressive acro-osteolysis with incidence of 9.8 per 100-person-years (95% CI 6.3–15.4). The median time of progressive acro-osteolysis was 3.5 years. Rate of progression increased from 1st to 3rd years follow-up with the progression rate at 1-, 2- and 3-years were 0, 2.0 and 18.3%, respectively. Patients with positive anti-topoisomerase I tended to have more progressive acro-osteolysis but no significant predictors on Cox regression. 44%, 18%, and 33% of who had no, mild, and moderate acro-osteolysis previously developed progression and 10 turned to be severe acro-osteolysis. In conclusion, the incidence of progressive acro-osteolysis was uncommon in early SSc but the rate of progression was pronouncedly increasing after three years follow-up. A half of the patients progressed to severe acro-osteolysis
Erratum to: Spontaneous skin regression and predictors of skin regression in Thai scleroderma patients
No full textThe prevalence of median neuropathy at wrist in systemic sclerosis patients at Srinagarind Hospital
No full textPrognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in Thailand
No full textIncidence and prevalence of idiopathic inflammatory myopathies in Thailand from the Ministry of Public Health data analysis
No full textAbstract The epidemiology of idiopathic inflammatory myopathies (IIMs) varies by country. Investigating the epidemiological profile among Thai IIMs could help to inform public health policy, potentially leading to cost-reducing strategies. We aimed to assess the prevalence and incidence of IIM in the Thai population between 2017 and 2020. A descriptive epidemiological study was conducted on patients 18 or older, using data from the Information and Communication Technology Center, Ministry of Public Health, with a primary diagnosis of dermatopolymyositis, as indicated by the ICD-10 codes M33. The prevalence and incidence of IIMs were analyzed with their 95% confidence intervals (CIs) and then categorized by sex and region. In 2017, the IIM cases numbered 9,074 among 65,204,797 Thais, resulting in a prevalence of 13.9 per 100,000 population (95% CI 13.6–14.2). IIMs were slightly more prevalent among women than men (16.8 vs 10.9 per 100,000). Between 2018 and 2020, the incidence of IIMs slightly declined from 5.09 (95% CI 4.92–5.27) in 2017 and 4.92 (95% CI 4.76–5.10) in 2019 to 4.43 (95% CI 4.27–4.60) per 100,000 person-years in 2020. The peak age group was 50–69 years. Between 2018 and 2020, the majority of cases occurred in southern Thailand, with incidence rates of 7.60, 8.34, and 8.74 per 100,000 person-years. IIMs are uncommon among Thais, with a peak incidence in individuals between 60 and 69, especially in southern Thailand. The incidence of IIMs decreased between 2019 and 2020, most likely due to the COVID-19 pandemic, which reduced reports and investigations
The prevalence of median neuropathy at wrist in systemic sclerosis patients at Srinagarind Hospital
No full textObjectives: To determine the prevalence and factor related with median neuropathy at wrist (MNW) in systemic sclerosis patients. Study Design: Cross-sectional study. Setting: Srinagarind Hospital, Khon Kaen, Thailand. Participants: Systemic sclerosis patients who attended the Scleroderma Clinic, Srinagarind Hospital. Materials and Methods: Seventyfive systemic sclerosis patients were prospectively evaluated by questionnaire, physical examination, and electrodiagnostic study. The questionnaire consisted of the symptoms, duration, and type of systemic sclerosis. The physical examination revealed skin score of systemic sclerosis, pinprick sensation of median nerve distribution of both hands, and weakness of both abductor pollicis brevis muscles. The provocative test which were Tinel′s sign and Phalen′s maneuver were also examined. Moreover, electrodiagnostic study of the bilateral median and ulnar nerves was conducted. Results: The prevalence of MNW in systemic sclerosis patients was 44% - percentage of mild, moderate, and severe were 28%, 9.3%, and 6.7%, respectively. The prevalence of asymptomatic MNW was 88%. There were no association between the presence of MNW and related factors of systemic sclerosis. Conclusions: MNW is one of the most common entrapment neuropathies in systemic sclerosis patients. Systemic sclerosis patients should be screened for early signs of MNW
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