Author Description

Patients that present with a connective tissue disease are often a challenge to diagnose and treat. A connective tissue disease is a disorder in which the tissues of the body consisting of the cells and the matrix which holds them together are disrupted. This guide will describe several multi system conditions that result from the inflammatory and immune-mediated disorder systemic lupus erythematosus and the related condition of Sjogren's syndrome. These diseases affect various tissues of the body; details of their management are discussed within, including presentation, investigation, differential diagnosis, and treatment. These diseases are multifactorial conditions associated with a complex genetic predisposition and various, not well understood, environmental triggers that induce inflammatory and immune-mediated responses directed against components of the person's own body. As a result these diseases fall under the umbrella term known as the systemic autoimmune diseases. Patients with a systemic autoimmune connective tissue disease can present with clinical symptoms and signs in one or more often, several, systems of the body. Some of these diseases affect people of certain ages and gender more often than others, including children, but this guide will focus on the management of adult patients. These diseases are more common than is generally realized, cannot be cured, are associated with considerable morbidity and still cause significant mortality. Appropriate investigations are critical to making the correct diagnosis so that treatment can be tailored to the patient's condition. It is important not only to recognize and treat the current disease activity, but also to prevent death and the development of chronic damage due to complications of the disease and the immuno-suppressive drugs used to treat it. Principles of drug treatment are discussed, but the final choice of drug and exact dosages to be used will depend on the details of the patient's condition and should be planned by a physician with relevant experience of these conditions and responsible for the management of the patient. Due to the variable severity of these diseases and their multisystem nature, patients with an autoimmune connective tissue disease may present to general practitioners, general physicians or internal medicine specialists, obstetricians, intensive care or any medical or surgical specialist. The majority of patients with these conditions are cared for long term by dermatologists, rheumatologists, and/or nephrologists, as it is the skin, joints and kidneys that are most often involved. However, it should be noted that many patients develop cardio-respiratory or infectious complications, including accelerated atherosclerosis, due to the disease and/or its treatment. Multidisciplinary care is important and should involve the full range of allied health professionals, as well as the relevant clinical specialists depending on the organs and systems involved.