The intersection of the connective tissue diseases (CTD) and the interstitial lung diseases (ILD) is complex. Although often considered as a single entity, "CTD-ILD" actually reflects a heterogeneous spectrum of diverse CTDs and a variety of patterns of interstitial pneumonia.
The evaluation of patients with CTD that develop ILD, or the assessment for underlying CTD in those presenting with presumed "idiopathic" ILD can be challenging and these evaluations can be optimized by effective multidisciplinary collaboration. When a diagnosis of CTD-ILD is confirmed, careful and thorough assessments to determine extra- versus intra-thoracic disease activity, and degrees of impairment are needed. Pharmacologic intervention with immunosuppression is the mainstay of therapy for all forms of CTD-ILD, but should be reserved only for those that demonstrate clinically significant and/or progressive disease.
The management of CTD-ILD is not yet evidence based and there is a desperate need for controlled trials across the spectrum of CTD-ILD. Non-pharmacologic management strategies and addressing comorbidities or aggravating factors should be part of a comprehensive treatment plan for individuals with CTD-ILD.
Idiopathic pulmonary fibrosis (IPF) is a complex disease that is associated with various respiratory and non-respiratory comorbidities. The most common comorbidity is cardiovascular disease (CVD), which increases in incidence with increasing duration of IPF and is associated with a higher risk of mortality. The direction of causality between CVD and IPF is unclear.
There is evidence that IPF is associated with a prothrombotic state; however, warfarin is not beneficial in IPF patients. Lung cancer is another common comorbidity, being present in more than 50% of IPF patients at 10 years after diagnosis. IPF and lung cancer share several risk factors and pathogenic pathways and also show a similar anatomic distribution; this can make radiological diagnosis difficult. As with CVD, lung cancer in IPF patients is associated with a significantly worse prognosis and treatment options are limited. Surgery, chemotherapy, and radiotherapy have all been associated with an increased risk of morbidity and mortality. However, treatment may be considered in selected patients with less advanced cancer and less advanced IPF. Emphysema may occur in patients with IPF and is believed to represent a distinct clinical syndrome, known as the combined pulmonary fibrosis and emphysema (CPFE) syndrome. CPFE has a strong male predominance, is strongly linked with smoking, and has distinct radiographic features.
CPFE is also associated with a very high frequency of pulmonary hypertension, which is associated with a poor prognosis. There are no specific treatments for CPFE and evaluation of IPF therapies in CPFE patients is urgently needed. (Sarcoidosis Vasc Diffuse Lung Dis 2015; 32; Suppl 1: 17-23).