Very early and early systemic sclerosis: An update.

[Very early and early systemic sclerosis: An update]. Rev Med Interne. 2019 Apr 10;: Authors: Michaud M, Catros F, Ancellin S, Gaches F Abstract Classification criteria for systemic sclerosis evolved over the last three decades, allowing an earlier classification. In the late 2000s, the EULAR Scleroderma Trials and Research Group validated very early and early systemic sclerosis criteria. Raynaud phenomenon, anti-nuclear antibody positivity and the puffy fingers are "Red flags" that must lead to refer the patient to a specialist and benefit from a capillaroscopy and the specific autoantibodies. At the stage of very early systemic sclerosis, pulmonary, cardiac and digestive involvements may be present and must be screened. Herein, we detail very early and early systemic sclerosis criteria, as well as the predictive factors of evolution towards a systemic sclerosis. PMID: 30981561 [PubMed - as supplied by publisher]
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research

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We report a 34-year-old woman with a medical history of asthma and Raynaud's phenomenon, presented for the evaluation and management of multiple erythematous hyperpigmented annular plaques reportedly developed after taking trimethoprim/sulfamethoxazole. An initial skin biopsy showed findings supportive of a drug eruption. She was treated with oral prednisone and achieved some improvement. She presented 1 year later with enlargement of the plaques and emergence of new lesions. Skin biopsies revealed an unremarkable epidermis with marked fibrosis of the mid-to-deep dermis with sparing of the papillary dermis, and superficial...
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Brief Report Source Type: research
CONCLUSIONS: Overall, 50% of dermatoscopic images were classified as non-specific and 30% were classified as non-interpretable in RP patients. However, all images classified by dermatoscopy as "normal" or as overt "scleroderma" pattern were confirmed by concomitant NVC analysis. These findings demonstrate tenuous promise for dermatoscopy as a tool for the initial screening of nailfold capillaries in RP. Further regular work up with NVC is needed to further clarify non-interpretable and non-specific findings possibly related to non-scleroderma patterns. PMID: 32865167 [PubMed - in process]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
You are consulted to evaluate a 56 ‐year‐old woman with known Raynaud's phenomenon, finger swelling of several; months’ duration, and new hypertension with a blood pressure of 160/100 mm/Hg. She also reports progressive shortness of breath. Physical examination reveals telangiectasias, sclerodactyly, and proximal skin sclerosis (thick shiny skin on the chest and upper arms), and bibasilar crackles are found on chest examination. Laboratory tests reveal evidence of microangiopathic hemolytic anemia, thrombocytopenia, and elevation of the serum creatinine level (previously normal), and chest computed tomography sho...
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Expert Perspectives On Clinical Challenges Source Type: research
ConclusionsOur results confirm the efficacy and safety of autologous fat grafting for the treatment of skin complications and digital ulcers due to systemic sclerosis. In addition, the patients ’ subjective well-being improved.Level of evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authorswww.springer.com/00266.
Source: Aesthetic Plastic Surgery - Category: Cosmetic Surgery Source Type: research
As lack of awareness of rare diseases (RDs) among healthcare professionals results in delayed diagnoses, there is a need for a more efficient approach to RD training during academic education. We designed an e...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Review Source Type: research
In this study, we investigated the correlation of NVC patterns and DTM variables in SSc patients. METHODS: Patients with SSc by 2013 ACR/EULAR criteria that were consented into the clinical care registry had NVC and DTM performed. For NVC, the number of capillaries (density), measurement of apical diameter (dimension), presence or absence of hemorrhages and number of abnormal shapes were assessed to categorize three different qualitative patterns: 'early', 'active' and 'late'. For DTM, Doppler ultrasound hyperemic, low frequency, blood velocity of radial artery and fingertip vascular function was assessed and a vascul...
Source: J Rheumatol - Category: Rheumatology Authors: Tags: J Rheumatol Source Type: research
Authors: Bobeică C, Laurențiu Tatu A, Crăescu M, Solovăstru L Abstract Systemic sclerosis (SSc) is a collagenosis with insufficiently known etiopathogenesis, characterized by microvasculopathy and excessive fibrosis in the context of an autoimmune disorder. The incompletely elucidated pathogenesis and limited therapeutic options, disabling aspects, skin lesions and pain determine important functional and psychological deficiencies which affect the quality of life. It is imperative to observe and correlate individual clinical and paraclinical data to optimize disease management. A group of 22 patients diagnosed ...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research
The authors have identified an error in thier manuscript and have incorrectly referred to the overall DISCERN score for SSc and RP as 2.21 and 1.99, respectively, in the abstract and discussion. The correct DISCERN quality scores are listed in Table 2.
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
CONCLUSIONS: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights on the SSc natural history and main prognostic factors. PMID: 32301427 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
We reported that a systemic sclerosis (SSc) or scleroderma-like capillaroscopic pattern is common in patients with Raynaud phenomenon, and can be frequently observed in patients with connective tissue diseases (CTD) other than SSc. PMID: 32295851 [PubMed - as supplied by publisher]
Source: J Rheumatol - Category: Rheumatology Authors: Tags: J Rheumatol Source Type: research
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