ABSTRACT
Introduction Pulmonary involvement is remarkably common1,2 in patients with connective tissue disorders and patients who present with respiratory symptoms pose a considerable challenge to the physician. Lung disease is now the most frequent cause of death in such patients. Hence, accurate evaluation of the lungs has become an increasingly important component of clinical practice. The growth in information technology has meant that patient awareness about lung disease and the expectations that disease will be treated have also increased. The result is that sensitive screening procedures, such as high-resolution computed tomography (HRCT), are often brought to bear and, perhaps not surprisingly, ‘early’ disease is frequently detected. The decision of whether to treat then becomes a problem with which the physician must grapple. Another difficulty is the plethora of different yet non-specific interstitial processes which overlap but may have different outcomes to those seen in the idiopathic interstitial pneumonias.3,4
The clinical evaluation is further complicated by the variable presence of pulmonary vascular disease, airways disease, extra-thoracic pulmonary restriction, lung disease due to treatment of the underlying rheumatological disorder, and even possible cardiac involvement. In some connective tissue disorders, most notably rheumatoid arthritis, it is also known that different disease processes often coexist in an individual patient, confounding the quantification of the functional severity of disease.
