CREST Syndrome in Systemic Sclerosis Patients – Is Dystrophic Calcinosis a Key Element to a Positive Diagnosis?

Abstract

Carmen Bobeica,1,* Elena Niculet,1,2 Mihaela Craescu,1 Elena-Laura Parapiru,3,* Carmina Liana Musat,1,* Ciprian Dinu,4,* Iulia Chiscop,5,* Luiza Nechita,3,* Mihaela Debita,6,* Victorita Stefanescu,6,* Ioana Anca Stefanopol,1,7,* Alexandru Nechifor,3,* Ana Maria Pelin,8,* Gabriela Balan,3,9,10,* Silvia Chirobocea,11,* Claudiu Ionut Vasile,3,* Alin Laurentiu Tatu2,3,12 1Department of Morphological and Functional Sciences, Faculty of Medicine and Pharmacy, “Dunărea de Jos” University, Galați, Romania; 2Multidisciplinary Integrated Center of Dermatological Interface Research MIC-DIR (Centrul Integrat Multidisciplinar de Cercetare de Interfata Dermatologica - CIM-CID), ‘Dunărea de Jos’ University, Galați, Romania; 3Clinical Medical Department, Faculty of Medicine and Pharmacy, Dunărea de Jos University, Galați, Romania; 4Dental Department, Faculty of Medicine and Pharmacy, Dunărea de Jos University, Galați, Romania; 5Clinical Surgical Department, Faculty of Medicine and Pharmacy, “Dunărea de Jos” University, Galați, Romania; 6Medical Department, Faculty of Medicine and Pharmacy, Dunărea de Jos University, Galați, Romania; 7Department of Pediatrics, Clinical Emergency Hospital for Children “Sf. Ioan”, Galati, Romania; 8Department of Pharmaceutical Sciences, Faculty of Medicine and Pharmacy, “Dunărea de Jos” University, Galați, Romania; 9Department of Gastroenterology, “Sf. Apostol Andrei” County Emergency Clinical Hospital, Galați, Romania; 10Research Center in the Field of Medical and Pharmaceutical Sciences, “Dunărea de Jos” University, Galați, Romania; 11Department of Neurology, Municipal Emergency Hospital, Moinești, Romania; 12Dermatology Department, “Sf. Cuvioasa Parascheva” Clinical Hospital of Infectious Diseases, Galați, Romania*These authors contributed equally to this workCorrespondence: Elena Niculet; Mihaela Craescu, Department of Morphological and Functional Sciences, Faculty of Medicine and Pharmacy, “Dunărea de Jos” University of Galați, 35 Alexandru Ioan Cuza Street, Galați, 800008, Romania, Tel [phone], Email [email protected]; [email protected]: CREST syndrome is a clinical entity associated with systemic sclerosis, which meets at least three of the five clinical features: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. Three of these clinical features (Raynaud’s phenomenon, sclerodactyly and esophageal dysmotility) are often present in classical subsets of SSc: limited and diffuse, and their presence in association does not define CREST syndrome. Calcinosis seems to be less common in SSc and its association with other clinical features is characteristic of CREST syndrome. Therefore, it can be appreciated that calcinosis is the key element of CREST syndrome.Methods: This study included a number of 37 candidates with SSc, diagnosed with the help of the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2013 criteria.Results and Discussions: These three elements (calcinosis, Raynaud’s phenomenon, esophageal dysmotility) were recorded both in the limited subset of SSc, but especially in the subset of diffuse SSc, contrary to the data in the literature.Conclusion: We appreciate that CREST syndrome is a clinical entity that can overlap with both subsets of SSc. Given the divergent views of the authors on the classification of CREST syndrome, future studies may contribute to a reassessment of SSc classification.Keywords: systemic sclerosis, dystrophic calcinosis, CREST syndrome, telangiectasia, Raynaud’s phenomenon, sclerodactyly

Keywords

• systemic sclerosis
• dystrophic calcinosis
• crest syndrome
• telangiectasia
• raynaud's phenomenon
• sclerodactyly