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JOURNAL ARTICLE
Punthip Thammaroj, Prathana Chowchuen, Chingching Foocharoen
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...
March 1, 2024: Scientific Reports
#2
JOURNAL ARTICLE
Carles Tolosa-Vilella, Maria Del Mar Rodero-Roldán, Alfredo Guillen-Del-Castillo, Adela Marín-Ballvé, Rafael Boldova-Aguar, Begoña Marí-Alfonso, Carlos Feijoo-Massó, Dolores Colunga-Argüelles, Manuel Rubio-Rivas, Luis Trapiella-Martínez, Nerea Iniesta-Arandia, Eduardo Callejas-Moraga, Francisco J García-Hernández, Luis Sáez-Comet, Cristina González-Echávarri, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan J Ríos-Blanco, Jose Antonio Todolí-Parra, Ignasi Rodríguez-Pintó, Antonio-J Chamorro, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñoz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar
OBJECTIVES: To assess the associations and prognostic value of scleroderma patterns by nailfold videocapillaroscopy (NVC) in patients with systemic sclerosis (SSc) and cutaneous subsets. METHODS: At baseline, 1356 SSc patients from the RESCLE registry were compared according to the scleroderma pattern as Late pattern and non-Late pattern, which included Early and Active patterns. Patient characteristics, disease features, survival time and causes of death were analysed...
August 2023: Clinical and Experimental Rheumatology
#3
JOURNAL ARTICLE
Paula Song, Shufeng Li, Matthew A Lewis, David F Fiorentino, Lorinda Chung
IMPORTANCE: Degos-like lesions are cutaneous manifestations of a small-vessel vasculopathy that appear as atrophic, porcelain-white papules with red, telangiectatic borders. No study has adequately examined Degos-like lesions in patients with systemic sclerosis (SSc). OBJECTIVE: To characterize the serologic, cutaneous, and internal organ manifestations associated with Degos-like lesions in a large cohort of patients with SSc. DESIGN, SETTINGS, AND PARTICIPANTS: This retrospective cohort study involved adult patients with SSc who were seen at Stanford Rheumatologic Dermatology Clinic between January 1, 1998, and December 31, 2018...
March 1, 2023: JAMA Dermatology
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JOURNAL ARTICLE
Akash Babulal Vadher, Anindita Sinha, Shayeri Roy Choudhury, Mahesh Prakash, Muniraju Maralakunte, Tanveer Rehman, Shefali Sharma, Yashwant Kumar
OBJECTIVES: Prevalence of synovitis, tenosynovitis, erosions, acro-osteolysis and bone marrow edema in systemic sclerosis is not extensively reported. We aimed to estimate the prevalence of changes in individual joints of hands in systemic sclerosis patients. METHOD: A cross-sectional analytical study consisting of 34 adults (females, n = 32) with systemic sclerosis. Patients with clinical synovitis were excluded. All patients underwent ultrasound (US) and magnetic resonance imaging of bilateral hands...
February 2023: Journal of Scleroderma and related Disorders
#5
JOURNAL ARTICLE
Warut T Sriwong, Pattayarat Srisangwarn, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Chingching Foocharoen
BACKGROUND: Skin tightness is a hallmark of systemic sclerosis (SSc), and the fingers are an affected body part, so much so that fingerprints can be significantly affected among those with extensive skin tightness of the finger. OBJECTIVE: We aimed to compare the difference between the current and past (pre-disease onset) fingerprints of SSc patients. METHODS: We conducted a cross-sectional study among adult SSc patients who attended the Scleroderma Clinic, Khon Kaen University, between October 2019, and September 2020...
August 31, 2022: Clinical Rheumatology
#6
Mahsa Zargaran, Atiyeh Rostamian, Mahrooz Malek, Abdolrahman Rostamian
Systemic sclerosis is an autoimmune chronic sclerotic disease that can damage organs and cause serious complications for the patient such as musculoskeletal manifestations, Gastrointestinal involvement, pulmonary involvement, and renal disease. Acro-osteolysis is one of the musculoskeletal manifestations that causes corrosion of the bones in the fingertips of the hand and feet. In this paper, we have reported the rarely current evidence of severe Acro-osteolysis of the distal phalanges of the hands by radiological x-ray...
June 2022: Journal of Scleroderma and related Disorders
#7
REVIEW
Matthias Bollow
BACKGROUND: Progressive systemic scleroderma (PSS) and mixed connective tissue disease (MCTD) represent vasculitic autoimmune diseases from the group of collagenoses with manifestations in various organ systems such as the skin, the internal organs and the joints. OBJECTIVE: To present the atypical arthritis patterns of the hands in PSS and MCTD that differ from those in rheumatoid arthritis (RA) and psoriatic arthritis (PsA) in the context of clinical and serologic findings and in consideration of the classification of PSS and MCTD...
May 2021: Der Radiologe
#8
Harry Pachajoa, Angelica Claros-Hulbert, Ximena García-Quintero, Lina Perafan, Andres Ramirez, Andres F Zea-Vera
Hutchinson-Gilford progeria syndrome (HGPS) is a rare congenital disease caused by mutations in the LMNA gene. Children with HGPS are phenotypically characterized by lipodystrophy, short height, low body weight, scleroderma, reduced joint mobility, osteolysis, senile facial features, and cardiovascular compromise that usually lead to death. We aimed to describe the case of a patient who reached above-average age expectancy for children with HGPS in Latin America and describe the clinical and molecular characteristics of the patient...
2020: Application of Clinical Genetics
#9
JOURNAL ARTICLE
Antonia Valenzuela, Murray Baron, Tatiana S Rodriguez-Reyna, Susanna Proudman, Dinesh Khanna, Amber Young, Monique Hinchcliff, Virginia Steen, Jessica Gordon, Vivien Hsu, Flavia V Castelino, Sara Schoenfeld, Shufeng Li, Joy Y Wu, David Fiorentino, Lorinda Chung
OBJECTIVE: Calcinosis is a debilitating complication of systemic sclerosis (SSc) with no effective treatments. We sought to identify clinical correlations and to characterize complications and disability associated with calcinosis in a multi-center, international cohort of SSc patients. METHODS: We established a cohort of 568 consecutive SSc patients who fulfill 2013 revised ACR/EULAR criteria at 10 centers within North America, Australia, and Mexico. Calcinosis was defined as subcutaneous calcium deposition by imaging and/or physical examination, or a clear history of extruded calcium...
October 2020: Seminars in Arthritis and Rheumatism
#10
JOURNAL ARTICLE
João Dantas, Isabela Silva de Oliveira, Emanuela Pimenta da Fonseca, Mittermayer B Santiago
OBJECTIVES: We describe a case of systemic sclerosis (SS) with acro-osteolysis associated with cutaneous mucinosis, usually characterized by mucin deposition in the skin. The main differential diagnosis was multicentric reticulohistiocytosis due to the presentation of papulonodular skin lesions. MATERIALS AND METHODS: A physical examination, imaging studies and laboratory tests were performed. RESULTS: Distal bone resorption was evident on plain radiographs, and skin biopsy confirmed mucinosis...
2020: European Journal of Case Reports in Internal Medicine
#11
JOURNAL ARTICLE
Liisa Kröger, Tuija Löppönen, Leena Ala-Kokko, Heikki Kröger, Hanna-Mari Jauhonen, Kaisa Lehti, Jarmo Jääskeläinen
BACKGROUND: MONA, which stands for a spectrum of Multicentric Osteolysis, subcutaneous Nodulosis, and Athropathia, is an ultra rare autosomal recessive disorder caused by mutations in the matrix metallopeptidase 2 (MMP2) gene. To date only 44 individuals, carrying 22 different mutations have been reported. Here we report on two brothers with identical homozygous MMP2 gene mutations, but with clearly different phenotypes. METHODS: Genomic DNA was isolated from the affected brothers and the parents...
August 2019: Molecular Genetics & Genomic Medicine
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JOURNAL ARTICLE
Eda Didem Yalcin, Nihal Avcu, Serdar Uysal, Umut Arslan
OBJECTIVES: The aim of this study was to make radiomorphometric measurements on panoramic images and evaluate the radiologic findings of bones and teeth in patients with scleroderma. STUDY DESIGN: Panoramic images of 49 patients with scleroderma and 51 healthy controls were assessed. Mandibular radiomorphometric indices, including mandibular cortical index (MCI), mental index (MI), panoramic mandibular index (PMI), gonial index (GI), and antegonial index (AI), were determined...
January 2019: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
#13
REVIEW
Antonia Valenzuela, Paula Song, Lorinda Chung
PURPOSE OF REVIEW: To provide an update on the available literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc). RECENT FINDINGS: We identified observational studies that describe the frequency of calcinosis in SSc and associated clinical features; molecular studies exploring potential pathogenic mechanisms; and case reports and case series describing new diagnostic approaches and treatments...
November 2018: Current Opinion in Rheumatology
#14
JOURNAL ARTICLE
Milad Hosseinialhashemi, Babak Daneshfard, Omid Keshavarzian
No abstract text is available yet for this article.
January 2017: Iranian Journal of Public Health
#15
JOURNAL ARTICLE
Wan-Sun Choi, So-Yeon Park, Yoon-Seong Ban, Joo-Hak Kim
Acro-osteolysis is a rare disease characterized by bone resorption involving the distal phalanges of the hand. We present a unique case of progressive acro-osteolysis of the distal phalanges and articular calcifications in a patient with scleroderma. The calcified deposit in a proximal interphalangeal joint was excised under local anesthesia. The medical treatment was arranged under the supervision of a rheumatologist.
January 2017: Acta Orthopaedica et Traumatologica Turcica
#16
JOURNAL ARTICLE
Sujith V Cherian, Elena Thampy
No abstract text is available yet for this article.
July 2016: Indian Journal of Medical Research
#17
JOURNAL ARTICLE
Shantanu Dixit, Chaithra Kalkur, Atul P Sattur, Michael M Bornstein, Fred Melton
BACKGROUND: Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable...
October 24, 2016: Journal of Medical Case Reports
#18
JOURNAL ARTICLE
Antonia Valenzuela, Murray Baron, Ariane L Herrick, Susanna Proudman, Wendy Stevens, Tatiana S Rodriguez-Reyna, Alessandra Vacca, Thomas A Medsger, Monique Hinchcliff, Vivien Hsu, Joy Y Wu, David Fiorentino, Lorinda Chung
OBJECTIVES: We sought to identify the clinical factors associated with calcinosis in an international multicenter collaborative effort with the Scleroderma Clinical Trials Consortium (SCTC). METHODS: This is a retrospective cohort study of 5218 patients with systemic sclerosis (SSc). Logistic regression was used to obtain odds ratios (OR) relating calcinosis to various clinical features in multivariate analyses. RESULTS: A total of 1290 patients (24...
December 2016: Seminars in Arthritis and Rheumatism
#19
JOURNAL ARTICLE
Carles Tolosa-Vilella, Maria Lluisa Morera-Morales, Carmen Pilar Simeón-Aznar, Begoña Marí-Alfonso, Dolores Colunga-Arguelles, José Luis Callejas Rubio, Manuel Rubio-Rivas, Maika Freire-Dapena, Alfredo Guillén-Del Castillo, Nerea Iniesta-Arandia, Maria Jesús Castillo-Palma, Marivi Egurbide-Arberas, Luis Trapiellla-Martínez, José A Vargas-Hitos, José Antonio Todolí-Parra, Mónica Rodriguez-Carballeira, Adela Marin-Ballvé, Xavier Pla-Salas, Juan José Rios-Blanco, Vicent Fonollosa-Pla
OBJECTIVE: Digital ulcers (DU) are the most common vascular complication of systemic sclerosis (SSc). We compared the characteristics between patients with prior or current DU with those never affected and evaluated whether a history of DU may be a predictor of vascular, organ involvement, and/or death in patients with SSc. METHODS: Data from SSc patients with or without prior or current DU were collected by 19 referral centers in an ongoing registry of Spanish SSc patients, named Registro de ESCLErodermia (RESCLE)...
October 2016: Seminars in Arthritis and Rheumatism
#20
REVIEW
Nicola Ughi, Simon A Hervey, Roberta Gualtierotti, Zeni Silvana, Ariane L Herrick, Francesca Ingegnoli, Pierluigi Meroni
OBJECTIVES: The sparing effect of hemiplegia in rheumatic diseases has been described, but reports on systemic sclerosis (SSc)-spectrum disorders are unusual. SSc-spectrum disorders are complex diseases of unknown origin characterized by multisystem involvement, skin and organ fibrosis, microvascular alterations, and immunologic abnormalities. We describe two cases of patients with hemiplegia who developed Raynaud׳s phenomenon and skin fibrosis of the non-paretic limb. METHODS: Clinical, laboratory, and investigation findings of two cases with hemiplegia who developed scleroderma spectrum disorders of the non-paretic limb are presented...
April 2015: Seminars in Arthritis and Rheumatism
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