همراهي "فنوتيپ بينابيني اسكلرودرمي-شوگرن" با بيماري آديسون در يك بيمار: گزارش موردي

Occurance of scleroderma-Sjogren intermediate phenotype and Addison’s disease in a patient: case report

زمينه و هدف: آنتي‌بادي آنتي‌سانترومر با نارسايي غدد اگزوكرين مرتبط است و ممكن است يكي از علل خشكي دهان باشد. هدف از اين مقاله، معرفي يك مورد نادر فنوتيپ بينابيني اسكلرودرمي-شوگرن با آنتي‌سانترومر آنتي‌بادي مثبت در بيمار آديسون بود كه با پوسيدگي‌هاي دنداني مراجعه كرده بود. معرفي بيمار: يك زن 29 ساله مبتلا به آديسون با پوسيدگي‌هاي دنداني عود‌كننده به كلينيك دندانپزشكي مراجعه كرد. در بررسي ضمن تاييد آزمايشگاهي آديسون، خشكي دهان و چشم، رينود، رفلاكس معده به مري، چهره ماسكه و آنتي‌سانترومر مثبت يافت شد. با توجه به دارا بودن بعضي از علايم شوگرن و برخي از علايم اسكلرودرمي محدود و نه معيارهاي كامل هر كدام از اين دو بيماري، تشخيص فنوتيپ بينابيني اسكلرودرمي-شوگرن براي وي مطرح گرديد. نتيجه‌گيري: در پوسيدگي‌هاي دنداني عود‌كننده به‌ويژه در مبتلايان به يك بيماري خودايمن، آنتي‌بادي آنتي‌سانترومر مي‌تواند در شناسايي عامل خشكي دهان و نيز شناخت زودرس مراحل اوليه اسكلرودرمي يا فنوتيپ بينابيني اسكلرودرمي-شوگرن مفيد باشد.

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 anticentromere 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.