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Progressive systemic sclerosis with multiple comorbidities in a 71-year old female

Ilia-Presian Georgiev, Emran Lyutfi, Georgi Stefanov, Meliz Gursel, Tanya Shivacheva


Introduction: Progressive systemic sclerosis (PSS) is a connective tissue disorder with unknown eti­ology, characterized by increased collagen deposition leading to fibrosis, skin thickening and oth­er pathological changes in organs. Females constitute at least two thirds of all the cases. The disease commonly occurs in women aged 30-40 years. Other features of the disease include Raynaud`s phe­nomenon, telangiectasia, calcinosis, myositis, arthritis, tenosynovitis, renal failure, oesophageal dys­motility, pulmonary fibrosis and heart failure.

Materials and methods: A 71-years old female was hospitalized with ischemic and cyanotic finger­tips in the Department of Rheumatology, Saint Marina Hospital, Varna, Bulgaria. She complained of pain in both hip joints when walking, erythema in the region of the distal phalanges of the hands and feet with individual ulcerations on the fingertips. Raynaud›s phenomenon was positive. Other comorbidities which were included in her medical records were type two diabetes, hypertension and past history of endometrial cancer.

Results: Physical examination revealed sclerodactyly, cutaneous sclerosis of hands and feet and mi­crostomia. Laboratory tests revealed high levels of very low density lipoprotein (4.9) and high level of blood sugar (7.2). Immunology revealed high levels of antinuclear antibodies (+++), anti-centromere protein B (++), anti-Sjögren`s syndrome-A (Ro52) (+++). The following medications were adminis­tered: Methylprednisolone 40mg intravenous, Ranitidine 150mg 2Ñ…1 per os, pulse therapy with Cy­clophosphamide amp 1000mg in 500ml Physiological serum, then followed by another bank of Phys­iological serum 500ml, Metoclopramide 10mg - 1 ampulla intravenous and Heparin 2x500E intrave­nous.

Conclusions: The occurrence of the disease in such an advanced age is unlikely, while the risk of dia­betes and neoplasia increases exponentially with age. The uniqueness of the case herein lies with its late presentations, which could easily be misinterpreted as a complication of her concomitant diseas­es.


rheumatology; progressive systemic sclerosis; Raynaud`s phenomenon; microstomia



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