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scleroderma osteolysis

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
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...
June 2, 2016: Seminars in Arthritis and Rheumatism
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
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
Shan Huang, Yan Liang, Wei Wu, Xi Fu, Lihong Liao, Xiaoping Luo
OBJECTIVE: To explore clinical, radiographical and genetic characteristics of classical Hutchinson-Gilford progeria syndrome (HGPS). METHOD: Data of a case of HGPS diagnosed at Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology was analyzed and related literature was reviewed. RESULT: At the age of 8 months, the affected-infant presented with characteristic manifestation such as short stature, low weight, frontal bossing, alopecia, prominent scalp veins, micrognathia with a vertical midline groove in the chin, sclerodermatous skin, knee joints contracture with a horse-riding stance, and limited range of movement of ankle joints...
February 2014: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
C Melenotte, P Rossi, M Reynaud Gaubert, J-R Harlé, K Aissi, Y Frances, B Granel
No abstract text is available yet for this article.
December 2013: La Revue de Médecine Interne
Emma M Johnstone, Charles E Hutchinson, Andy Vail, Aurelie Chevance, Ariane L Herrick
OBJECTIVES: Acro-osteolysis (bony resorption of the terminal digital tufts) is a well-recognized, but under-researched, manifestation of SSc. Our aim was to investigate the hypothesis that acro-osteolysis is associated with (i) the severity of digital ischaemia and (ii) the presence of calcinosis. METHODS: This was a retrospective study of 101 patients with SSc in whom hand radiographs taken between 2001 and May 2008 were available for review. These radiographs were graded for severity of acro-osteolysis on a 0-4-point scale for each finger (0 = normal bone structure, 4 = severe pencilling of the terminal phalanges)...
December 2012: Rheumatology
Mohammad Al-Haggar, Agnieszka Madej-Pilarczyk, Lukasz Kozlowski, Janusz M Bujnicki, Sohier Yahia, Dina Abdel-Hadi, Amany Shams, Nermin Ahmad, Sahar Hamed, Monika Puzianowska-Kuznicka
Mandibuloacral dysplasia (MAD) is a rare disease resulting from a mutation of LMNA gene encoding lamins A and C. The most common mutation associated with this disease is a homozygous arginine 527 replacement by histidine. Three female patients originating from two unrelated families from Northeast Egypt were examined. Their growth was retarded; they had microcephaly, widened cranial sutures, prominent eyes and cheeks, micrognathia, dental crowding, hypoplastic mandible, acro-osteolysis of distal phalanges, and joint contractures...
November 2012: European Journal of Human Genetics: EJHG
Alka Sharma, Vishal Sharma
No abstract text is available yet for this article.
February 24, 2012: New Zealand Medical Journal
Kotb Abbass Metwalley Kalil, Hekma Saad Fargalley
INTRODUCTION: Hutchinson-Gilford progeria syndrome is a rare genetic disorder. It is reported to be present in one in eight million and is characterized by severe growth failure, early loss of hair, lipodystrophy, scleroderma, decreased joint mobility, osteolysis, early atherosclerosis and facial features that resemble those of an aged person. Apart from diabetes mellitus, there are no reported abnormalities of thyroid, parathyroid, pituitary or adrenal function. Here, we report the case of a 10-year-old Egyptian child with Hutchinson-Gilford progeria syndrome and hypoparathyroidism...
2012: Journal of Medical Case Reports
Carmen Pilar Simeón-Aznar, Vicent Fonollosa-Plá, Carles Tolosa-Vilella, Gerard Espinosa-Garriga, Manel Ramos-Casals, Mercedes Campillo-Grau, Francisco José García-Hernández, María Jesús Castillo-Palma, Julio Sánchez-Román, José Luis Callejas-Rubio, Norberto Ortego-Centeno, Maria Victoria Egurbide-Arberas, Luis Trapiellla-Martínez, María Gallego-Villalobos, Luis Sáez-Comet, José Velilla-Marco, María Teresa Camps-García, Enrique de Ramón-Garrido, Eva María Esteban Marcos, Lucio Pallarés-Ferreres, Carmen Hidalgo-Tenorio, José Mario Sabio-Sánchez, Ricardo Gómez-de la Torre, Gonzalo Salvador-Cervello, Juan José Rios-Blanco, Antonio Gil-Aguado, Miquel Vilardell-Tarrés
OBJECTIVE: To investigate the incidence of clinical and immunological characteristics of a large cohort of Spanish patients with scleroderma (SSc) and identifying factors associated with particular organ manifestations assessed by a nationwide cross-sectional analysis. METHODS: We classified SSc patients in 4 subsets using a modification of LeRoy and Medsger classification that included: "prescleroderma" (pre-SSc), limited cutaneous SSc (lcSSc), diffuse cutaneous SSc (dcSSc), and SSc sine scleroderma (ssSSc)...
June 2012: Seminars in Arthritis and Rheumatism
Didem Arslan Tas, Eren Erken, Hakan Sakalli, A Eftal Yucel
Articular symptoms are common in SSc and joint pain is a frequent presenting feature of this disease. Hand involvement is often the first clinical manifestation of SSc and could be resulted from fibrosis or synovitis or an overlap syndrome with rheumatoid arthritis (RA); though, the latter is a controversy in practice. To define the clues when identifying the nature of the hand arthropathy in SSc. In order to determine the hand arthropathy, serological tests, hand radiography, finger-to-palm (FTP) distance and other clinical features, disease activity and functional scoring parameters were assessed...
November 2012: Rheumatology International
J Avouac, G Mogavero, H Guerini, J L Drapé, A Mathieu, A Kahan, Y Allanore
OBJECTIVE: To examine the outcomes of hand radiographic x-rays in patients with systemic sclerosis (SSc) and to identify risk factors for the progression of hand radiographic lesions in a prospective cohort. METHODS: Dual time-point x-rays were systematically performed after a median interval of 5 years (range 4-7 years) in 103 consecutively recruited patients with SSc. Univariate and multivariate Cox proportional hazards models evaluated predictors of progression of hand radiographic lesions...
April 2011: Annals of the Rheumatic Diseases
Thirthar Palanivelu Vetrichevvel, Lourdhurajan Renita, Subramanyam Shobana, Sankarasubramanian Anandan
No abstract text is available yet for this article.
August 2010: International Journal of Dermatology
Sophie Koutaissoff, Marie Vanthuyne, Vanessa Smith, Ellen De Langhe, Geneviève Depresseux, Rene Westhovens, Filip De Keyser, Jacques Malghem, Frédéric A Houssiau
OBJECTIVE: To define the burden of hand radiological damage in systemic sclerosis (SSc) patients, compared with a control group. METHODS: Both hands of 167 SSc patients and 168 hands (82 right and 86 left) of age- and gender-matched controls were imaged by conventional radiograph. Two musculoskeletal radiologists semiquantitatively scored the following lesions: tuft acro-osteolysis, tuft calcinosis, joint space narrowings, marginal erosions, surface erosions, collapse arthropathies, periarticular calcifications, and juxta-articular osteoporosis, at the following areas: tufts, distal interphalangeal, proximal interphalangeal, metacarpophalangeal, carpal, and first carpometacarpal joints...
April 2011: Seminars in Arthritis and Rheumatism
M Marcucci, N Abdala
OBJECTIVES: The aim of this study was to investigate the relationship between mandibular osteolysis and the T1 and T2 tissue signal strength and morphology of the masseter muscle in patients with systemic sclerosis (SS). METHODS: All patients with SS underwent panoramic radiography to detect mandibular osteolysis. MRI of the masseter was performed on 15 patients with SS and 10 healthy control subjects. RESULTS: All patients presented mean T1 values that were statistically equal...
December 2009: Dento Maxillo Facial Radiology
M J Saavedra, C Ambrosio, A Malcata, M Matucci-Cerinic, J A P Da Silva
A case of exuberant acroosteolysis and subcutaneous tissue calcinosis in the absence of skin involvement is presented. Different hypotheses are discussed following the clinical unfolding of the case in practice.
May 2009: Clinical and Experimental Rheumatology
Marcelo Marcucci, Nitamar Abdala
Systemic sclerosis (SS) is an autoimmune disease with great repercussions on the hard and soft tissues of the orofacial region. The aim of this study was to investigate the relationship between mandibular osteolysis and mouth opening measurements, duration of disease and presence/absence of teeth. Twenty-five subjects were selected: 15 diagnosed with systemic sclerosis and 10 healthy controls. The SS patients were grouped according to the presence (group I) or absence (group II) of mandibular osteolysis. The healthy subjects served as the control group (III)...
January 2009: Brazilian Oral Research
Glen A Toomayan, Emily N Vinson
No abstract text is available yet for this article.
March 2009: Arthritis and Rheumatism
Yolanda Braun-Moscovici, Daniel E Furst, Doron Markovits, Alexander Rozin, Philip J Clements, Abraham Menahem Nahir, Alexandra Balbir-Gurman
OBJECTIVE: .Sclerodactyly with acroosteolysis (AO) and calcinosis are prominent features of systemic sclerosis (SSc), but the pathogenesis of these findings is poorly understood. Vitamin D and parathyroid hormone (PTH) have a crucial role in bone metabolism and resorption and may affect AO and calcinosis. We assessed vitamin D and PTH in patients with SSc. METHODS: Medical records of 134 consecutive patients with SSc (American College of Rheumatology criteria) followed at the rheumatology department during the years 2003-2006 were reviewed for clinical assessment, laboratory evaluation [including 25(OH) vitamin D, calcium, phosphorus, alkaline phosphatase, PTH, creatinine, and albumin]; imaging data confirming AO and/or calcinosis...
November 2008: Journal of Rheumatology
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