Occurance of Scleroderma-Sjogren Intermediate Phenotype and Addison’S Disease in a Patient: Case Report



Ahmadi E¹ ; Fallahi S^{2, 3} ; Jalalian B¹ ; Shakib PA⁴
Source: Tehran University Medical Journal Published:2018

Abstract

Background: Association of Addison's disease with connective tissue diseases such as scleroderma and Sjogren have been rarely reported. Anti-centromere antibody (ACA) has been associated with exocrine gland dysfunction in anti-Ro, anti-La negative Sjogren’s syndrome and may be one of the causes of xerostomia in community. The purpose of this article was to introduce a rare case of scleroderma-Sjogren intermediate phenotype with positive anti-centromere antibody in a known case of Addison’s disease admitted for dental caries and xerostomia. Case Presentation: A 29-year-old woman with Addison’s disease referred to a dental clinic due to recurrent dental caries. Addison’s disease was confirmed by low basal serum cortisol level and unresponsive serum cortisol level to adrenocorticotropin hormone (rapid ACTH stimulation test). Signs of xerostomia, xerophthalmia, Raynaud’s phenomenon, gastro-esophageal reflux, masked face, osteoporosis, positive anti-centromere antibody, negative anti-Ro and anti-La antibodies and failure to match the pathology of the minor salivary gland of lip with Sjogren's disease were found. The diagnosis of scleroderma-Sjogren intermediate phenotype was raised with considering some of the symptoms of scleroderma and Sjogren and not the exact classification criteria for each of these two diseases. Hydroxychloroquine, fluoride and Biotene® mouthwash (Laclede, Inc., CA, USA) (oral moisturizing saliva), chewing gum containing xylitol plus artificial tear droplet was prescribed. Drinking plenty of fluids was recommended. Due to gastroesophageal reflux and osteoporosis, Pantoprazole and CinnoPar® (Cinnagen, Iran) (parathyroid hormone analogue) plus calcium and vitamin D supplements was administered. Regarding adrenal insufficiency, Prednisolone and Fludrocortisone were continued. Conclusion: For evaluation of recurrent dental caries, especially in patients with autoimmune disease, anti-centromere antibody may be useful to identify the cause of dry mouth, as well as early detection of limited scleroderma or scleroderma-Sjogren intermediate phenotype. © 2018, Tehran University of Medical Sciences. All rights reserved.