Rheumatoid Arthritis Interstitial Lung Disease

Challenges in Rheumatology 66death was found to be three times higher in RA patients with ILD compared to patients without ILD; the median survival following ILD diagnosis was only 2.6 years (Bongartz et al., 2010).In general, treating connective tissue disease -associated ILD (CTD-ILD) early, with immunosuppressive medications, not only may halt ILD progression, but may improve quality of life.This may be true for RA-ILD as well, but these disease-specific data are scarce.The poor prognosis of RA patients with ILD highlights the need for clinicians to recognize the clinical features of this condition and be cognizant of its course and management. Clinical features and courseClinical detection of ILD in RA patients may be challenging.The challenge stems from the non-specific clinical features of this condition and, typically, symptoms that are often masked or manifest in an insidious manner.Patients most commonly present complaining of worsening shortness of breath on exertion and a dry cough.Since this patient population suffers from arthritis that often limits their physical activity, the presentation of exertional dyspnea may in fact already denote advanced lung disease.Moreover, since maximum ventilation at peak exercise is approximately 70% of maximum voluntary ventilation (MVV), in the absence of significant lung disease, there is significant pulmonary reserve and exercise should not be limited by dyspnea (Hansen et al., 1984).Therefore, a low clinical threshold of suspicion should be maintained with respect to complaints of dyspnea in these patients.Complaints of lower-extremity swelling, syncopal episodes, and exertional chest pain may represent an underlying pulmonary arterial hypertension with right-sided heart failure, a severe complication of ILD.Clinicians should also be mindful of the fact that RA patients, particularly elderly patients, may have other co-morbid conditions such as congestive heart failure or anemia that may present with similar symptoms.Therefore, clinicians must consider and actively search for ILD in the presence of these symptoms, rather than simply assigning them to other more common co-morbidities.Physical exam in early ILD may be normal.A small proportion of patients may have digital clubbing (Rajasekaran et al., 2001).Most commonly, RA patients with ILD will have bilateral fine crackles heard best at the end of inspiration and tend to be more prominent at the lung bases.Expiratory wheezing, bronchial or upper airway sounds are uncommon and their presence can suggest either airway disease involvement or a concomitant respiratory condition such as chronic obstructive pulmonary disease (COPD) or asthma.The presence of hypoxemia may denote a more advanced disease process.Physical exam findings of an elevated jugular venous pressure with CV waves, an abnormal hepatojugular reflux, and lower-extremity pitting edema can represent an underlying right ventricular dysfunction relating to pulmonary arterial hypertension.A parasternal heave, a prominent second heart sound, and a tricuspid regurgitation murmur on precordial exam, as well as a pulsatile liver and ascites on abdominal exam, are all findings suggestive of underlying advanced pulmonary arterial hypertension.Although uncommon in RA-ILD without concomitant COPD, clinicians should be aware of these physical findings to facilitate early detection of this serious complication.Evolving evidence points to the existence of another unique clinical entity of RA-associated ILD in which patients also present with signs and symptoms suggestive of COPD.The syndrome of combined pulmonary fibrosis and emphysema (CPFE) was first described by Cottin et al. in 2005.CPFE was characterized by the association of tobacco smoking, significant dyspnea, impaired diffusion capacity on pulmonary function test, exertional hypoxemia and www.intechopen.com

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