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

Abstract

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, esophageal dysmotility, pulmonary fibrosis, and heart failure.

Materials and Methods: A 71-year-old female was hospitalized with ischemic and cyanotic fingertips in the Department of Rheumatology, St. Marina University 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 that were included in her medical records were type two diabetes, hypertension, and past history of endometrial cancer. The physical exam 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) (+++).

Results: The following medications were administered: methylprednisolone 40 mg intravenous, ranitidine 150 mg 2х1 per os, pulse therapy with cyclophosphamide amp 1000 mg in 500 mL saline, then followed by another bank of 500 mL saline, metoclopramide 10 mg - 1 ampoule intravenously, and heparin 2x500E intravenous.

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