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Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

Han HS, Ahn GR, Kim HJ, Park KY, Li K, Seo SJ

Systemic sclerosis (SSc) is a chronic systemic disease of unknown etiology characterized by vasculopathy, excessive accumulation of extracellular matrix, and fibrosis of the skin and other internal organs. Although its...
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Pharmacological treatment of pulmonary fibrosis

Choi WI

Idiopathic pulmonary fibrosis (IPF) is a condition that has been described as alveolar collapse and thickening, which correlate with dysregulated surfactant production and injury to type 2 alveolar cells. As...
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Diagnosis of Systemic Scleroderma in a Patient with Leg Ulcer

Ju GS, Son KM, Choi WY, Cheon JS

Systemic scleroderma is an autoimmune disease that affects connective tissue in internal organs and the skin with thickening and fibrosis. When afflicting internal organs, the disease leads to reduced quality...
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The Significance of NOTCH Pathway in the Development of Fibrosis in Systemic Sclerosis

Zmorzyński S, Styk W, Filip AA, Krasowska

Systemic sclerosis (SSc) is an autoimmune disorder characterized by the fibrosis of skin, heart, lung, and kidney as well. Excessive activation of fibroblasts is associated with higher expression of Notch1...
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Posterior Reversible Encephalopathy Syndrome in a Patient with Systemic Lupus Erythematosus and Systemic Sclerosis Overlap Syndrome

Song YM

Posterior reversible encephalopathy syndrome (PRES) is characterized by neurotoxic symptoms and neuroimaging finding of reversible cerebral edema in association with various conditions including hypertension, eclampsia, and autoimmune diseases. The author...
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A Rare Case of Overlap Syndrome with Diffuse Systemic Sclerosis, Rheumatoid Arthritis, and Cutaneous Sarcoidosis

Ko KM, Moon SJ, Koh JH, Kim J, Min JK

Overlap syndrome is defined as a disease entity that satisfies the classification criteria of at least two connective tissue diseases occurring concurrently or separately in a single patient. Here, we...
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Systemic Sclerosis and Microbiota: Overview of Current Research Trends and Future Perspective

Kim S, Chun SH, Park HJ, Lee SI

The commensal microbiota contributes to the maintenance of immune homeostasis in the human body. Autoimmunity can be aggravated or alleviated by the microbiota, which affects both innate and adaptive immune...
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Imaging of Thoracic Wall Abnormalities

Semionov A, Kosiuk J, Ajlan A, Discepola

Identification of certain abnormalities of the chest wall can be extremely helpful in correctly diagnosing a number of syndromic conditions and systemic diseases. Additionally, chest wall abnormalities may sometimes constitute...
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Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease

Koo SM, Kim SY, Choi SM, Lee HK, on behalf of Korean Interstitial Lung Diseases Study Group

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial...
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Endoscopic Features of Upper Gastrointestinal Tract in Patients with Systemic Sclerosis Compared to the Healthy Control

Park JW, Kim J, Kang EA, Kim MJ, Kim JS, Lee EB

OBJECTIVE: To characterize the endoscopic features of upper gastrointestinal tract in patients with systemic sclerosis (SSc) compared with those in the healthy controls. METHODS: Data on esophagogastroduodenoscopy (EGD) in 180 patients...
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Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease

Jung JY, Lee CH, Kim HA, Choi ST, Lee JH, Yoon BY, Kang DR, Suh CH

OBJECTIVE: Pulmonary hypertension (PH) develops frequently in connective tissue diseases (CTD) and is an important prognostic factor. The aim of this study was to assess the prevalence of PH in...
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Non-cirrhotic portal hypertension in an ankylosing spondylitis patient

Park S, Lee JH, Choi JS, Kim HW, Shim BJ, Choi WK, Kim SH

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients...
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A Case of Systemic Sclerosis Manifesting as Digital Finger Ulcers and Leg Ulcers

Han B, Choi MW, Son SJ, Lee JH, Han TY

  • KMID: 2436676
  • Korean J Dermatol.
  • 2018 Mar;56(3):218-220.
No abstract available.
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Systemic Sclerosis and Pleural Effusion in a Patient with Ankylosing Spondylitis

Koo BS, Song Y, Lee S, Kim TH, Jun JB

Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by endothelial and fibroblast dysfunction, resulting in progressive fibrosis of the skin and internal organs. Ankylosing spondylitis (AS) is an arthritic...
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Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view

Koo SM, Uh ST

Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be...
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Systemic Sclerosis Associated with Non-small Cell Lung Cancer and Papillary Thyroid Cancer: Case Report and Literature Review

Kim HJ, Kim JJ, Park HJ, Kim YT

We report the case of a 52-year-old patient with rheumatic vascular disease (systemic sclerosis), non-small cell lung cancer, and papillary thyroid cancer. Malignant tumors have been described in 3-11% of...
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Long-term Outcomes of Autologous Peripheral Blood Stem Cell Transplantation for Refractory Rheumatic Diseases

Lee S, Bae SC, Jun JB, Choi CB

OBJECTIVE: We investigated the long-term outcomes of autologous peripheral blood stem cell transplantation (PBSCT) to treat refractory rheumatic diseases. METHODS: Patients who underwent PBSCT for refractory rheumatic diseases at our institution...
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Neutrophil-to-lymphocyte Ratio in Diagnosis of Systemic Sclerosis for Prediction of Interstitial Lung Disease

Jung JH, Lee YM, Lee EG, Yoo WH, Lee WS

OBJECTIVE: The neutrophil-to-lymphocyte ratio (NLR) is elevated in inflammatory diseases, but its clinical significance in systemic sclerosis (SSc) is unclear. This study evaluated NLR in diagnosing SSc and in predicting...
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Improved Gastrointestinal Involvement in Systemic Sclerosis after Immunoglobulin Treatment

Choi KJ, Hwang JH, Kim HS, Lee JS

Systemic sclerosis is an intractable clinical subset characterized by diffuse fibrosis, vasculopathy, and immune abnormalities. Diffuse cutaneous systemic sclerosis has a variety of symptoms depending on the internal organs involved,...
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Pulmonary Arterial Hypertension is Normalized Following Six Years of Inhaled Iloprost Treatment in a Patient with Systemic Sclerosis

Yoon SY, Yoo ES, Yoo EJ, Jung JY, Kim HA, Suh CH

Pulmonary arterial hypertension is a critical manifestation of systemic sclerosis (SSc) and is a main cause of death. Several treatment modalities for SSc have been identified, with effects that improve...
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