Severe systemic scleroderma with multiple organ involvement in a 45-years-old patient

Violeta Diana Oprea,Grigore Balosin, ,Department Of Cardiology, “Sf Maria” Hospital, Bucharest, Romania ,Department Of Plastic Surgery, “Sf Maria” Hospital, Bucharest, Romania ,Ruxandra Ionescu,Violeta Claudia Bojinca,Ruxandra Nicoleta Ciofu

doi:10.37897/rjr.2021.1.5

Abstract

Systemic scleroderma has inflammation and fibrosis playing a crucial role and eventually leading to severe functional failure and damage of multiple organs. Three primary mechanisms are mainly involved in the pathogenesis of scleroderma: vascular anomalies, excess fibrosis, and autoimmune phenomenon. The present case study shows a severe cardiac dysfunction with low cardiac output syndrome (LCOS) with cardiac, hepatic and renal impairment, peripheral ischemia, neurologic symptoms associated or even aggravated by a probable severe toxicity to azathioprine therapy. Currently there is no cure for systemic sclerosis, the aims of treatment are- as recommended also by EULAR: to relieve symptoms, to prevent the condition from progressing, as much as possible, to detect and treat complications early and to minimize any disability.

Highlights

Being named from Greek words “sclero” (“hard”) and “dermis” (“skin”), scleroderma is a connective tissue disorder with 2 primary types: a localized one, where skin is mainly affected- and bones and muscles may be involved, and the systemic sclerosis, which can affect multiple organs and systems: heart, digestive tract, respiratory system, kidneys
Most pathogenesis studies emphasized on a complex role of genetics (OX40L gene polymorphism correlates with systemic scleroderma) and external environmental factors in the abnormal activation of the immune system causing tissue injury and blood vessel impairment determining accumulation of excess collagen in the intercellular space [3,4,5]
Cardiac failure was a severe clinical manifestation being caused by myocardial involvement- common in systemic sclerosis, which is considered to be related mainly to abnormal vasoreactivity and microvascular injuries such as transient coronary artery spasm leading to repeated focal ischemia

Summary

Introduction

Being named from Greek words “sclero” (“hard”) and “dermis” (“skin”), scleroderma is a connective tissue disorder with 2 primary types: a localized one, where skin is mainly affected- and bones and muscles may be involved, and the systemic sclerosis, which can affect multiple organs and systems: heart, digestive tract, respiratory system, kidneys.

Results

Conclusion