scholarly journals PAN SCLEROTIC MORPHEA MIMICKING DIFFUSE SYSTEMIC SCLEROSIS: A CASE-REPORT

2021 ◽  
Author(s):  
Carla Baleeiro Rodrigues Silva ◽  
Rodrigo da Silveira Vasconcelos ◽  
Aline Capellato Dias Baccaro ◽  
Osvaldo Luiz Camata Junior ◽  
Giovanny Homero Jacome Verdugo ◽  
...  
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Diffuse Systemic Sclerosis

scholarly journals Combination therapy with bosentan and sildenafil for refractory digital ulcers and Raynaud’s phenomenon in a 30-year-old woman with systemic sclerosis: Case report and literature review

2019 ◽  
Vol 5 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Jan-Gerd Rademacher ◽  
Chris Wincup ◽  
Björn Tampe ◽  
Peter Korsten
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Literature Review ◽  
Combination Therapy ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Digital Ulcers ◽  
Channel Blockers ◽  
Tissue Necrosis ◽  
Diffuse Systemic Sclerosis

Background: Systemic sclerosis is a rare autoimmune disease characterized by skin and organ fibrosis, and vasculopathy. Raynaud’s phenomenon is almost universally present in systemic sclerosis and can be the most debilitating symptom. Raynaud’s phenomenon may lead to the development of digital ulcers, potentially complicated by infection, tissue necrosis, and auto-amputation. Recommended treatments have variable efficacy. Methods: We report the case of a 30-year-old woman with diffuse systemic sclerosis suffering from severe Raynaud’s phenomenon and digital ulcers with digital tissue necrosis who was treated with combination therapy of an endothelin receptor antagonist and phosphodiesterase 5 inhibitor. In addition, we reviewed the literature on the topic. Results: Previous therapy with calcium-channel blockers, intravenous iloprost, and bosentan had all failed to control symptoms. We added sildenafil in combination with bosentan and observed a rapid and sustained treatment effect. Raynaud’s phenomenon severity, number of attacks, and attack duration decreased within 2 weeks of initiating treatment. Furthermore, this resulted in the healing of established digital ulcers. Conclusion: Our case report suggests that combination therapy may be a feasible treatment for the most severely affected and refractory patients. In our literature review, we found one retrospective study and three additional cases with similarly encouraging results.

scholarly journals Cardiac metabolomics and autopsy in a patient with early diffuse systemic sclerosis presenting with dyspnea: a case report

2015 ◽  
Vol 9 (1) ◽  
Author(s):  
Tracy M Frech ◽  
Monica P Revelo ◽  
John J Ryan ◽  
Ami A Shah ◽  
Jessica Gordon ◽  
...  
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Diffuse Systemic Sclerosis

Severe ankylosing spondylitis and diffuse systemic sclerosis: case report of a genetic trap

2005 ◽  
Vol 34 (2) ◽  
pp. 145-147 ◽  
Author(s):  
C. Kayser ◽  
M. C. V. Alvarenga ◽  
A. S. Neves ◽  
M. Gerbase‐De Lima ◽  
L. E. C. Andrade
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Ankylosing Spondylitis ◽  
Diffuse Systemic Sclerosis

scholarly journals ANCA‐associated neuropathy in systemic sclerosis: A case report and review of literature

2021 ◽  
Vol 4 (2) ◽  
pp. 34-36
Author(s):  
Kayo Takenaka ◽  
Takuya Takeichi ◽  
Ryoji Nishi ◽  
Masashi Suzuki ◽  
Haruki Koike ◽  
...  
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Review Of Literature

scholarly journals Gastric Antral Vascular Ectasia and Its Clinical Correlates in Patients with Early Diffuse Systemic Sclerosis in the SCOT Trial

2013 ◽  
Vol 40 (4) ◽  
pp. 455-460 ◽  
Author(s):  
Emily W. Hung ◽  
Maureen D. Mayes ◽  
Roozbeh Sharif ◽  
Shervin Assassi ◽  
Victor I. Machicao ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Topoisomerase I ◽  
Rna Polymerase Iii ◽  
Negative Association ◽  
Upper Gastrointestinal Endoscopy ◽  
Gastric Antral Vascular Ectasia ◽  
Clinical Correlates ◽  
Vascular Ectasia ◽  
Diffuse Systemic Sclerosis ◽  
Vascular Ectasias

Objective.To describe the prevalence and clinical correlates of endoscopic gastric antral vascular ectasia (GAVE; “watermelon stomach”) in early diffuse systemic sclerosis (SSc).Methods.Subjects with early, diffuse SSc and evidence of specific internal organ involvement were considered for the Scleroderma: Cyclophosphamide Or Transplant (SCOT) trial. In the screening procedures, all patients underwent upper gastrointestinal endoscopy. Patients were then categorized into those with or without endoscopic evidence of GAVE. Demographic data, clinical disease characteristics, and autoantibody data were compared using Pearson chi-square or Student t tests.Results.Twenty-three of 103 (22.3%) individuals were found to have GAVE on endoscopy. Although not statistically significant, anti-topoisomerase I (anti-Scl70) was detected less frequently among those with GAVE (18.8% vs 44.7%; p = 0.071). Similarly, anti-RNP antibodies (anti-U1 RNP) showed a trend to a negative association with GAVE (0 vs 18.4%; p = 0.066). There was no association between anti-RNA polymerase III and GAVE. Patients with GAVE had significantly more erythema or vascular ectasias in other parts of the stomach (26.1% vs 5.0%; p = 0.003).Conclusion.Endoscopic GAVE was present on screening in almost one-fourth of these highly selected patients with early and severe diffuse SSc. While anti-Scl70 and anti-U1 RNP trended toward a negative association with GAVE, there was no correlation between anti-RNA Pol III and GAVE. Patients with GAVE had a higher frequency of other gastric vascular ectasias outside the antrum, suggesting that GAVE may represent part of the spectrum of the vasculopathy in SSc.

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