Background/Purpose: As a quality assessment tool to evaluate patient care in a general Rheumatology Clinic, we determined the frequency of screening for pulmonary hypertension and interstitial lung involvement in a subset of patients at high risk.   Involvement of the respiratory system is common in Systemic Sclerosis (SS) and Polymyositis/Dermatomyositis (PM/DM). Interstitial Lung Disease (ILD) and Pulmonary Arterial Hypertension (PAH) can lead to significant morbidity. These complications are now the leading cause of death in systemic sclerosis. The prevalence of pulmonary involvement in PM/DM is variable, but has been reported to be close to 50%. ILD is a negative prognostic factor in these patients. General medical opinion is that Pulmonary Function Tests (PFTs) are suitable as a screening tool, but that a High Resolution Computed Tomography (HRCT) scan is crucial for the initial diagnosis of ILD. In addition, an echocardiogram is recommended to evaluate for PAH. However, there are no uniform guidelines for screening and the frequency with which these studies should be repeated. Published data suggests that screening assessments are not done routinely, so to establish a baseline for quality of care,we determined the frequency of pulmonary evaluation in our clinic population.

Methods: All patients were seen in our outpatient Rheumatology clinic from January 1, 2010 to December 31, 2011. Patients were identified based on diagnosis codes, and diagnoses confirmed by chart review. Charts were reviewed to determine if screening for ILD and PAH had been performed within 24 months of the index visit. ILD was classified by FVC < 70% or suggestive findings on CT imaging. If PAH was suggested by either decreased DLCO on PFTs or increased RVSP on echocardiogram, a right heart catheterization (RHC) was needed to confirm the presence of PAH.

Results: Of the 91 patients categorized, 44 had a diagnosis of SS, while 47 carried a diagnosis of PM/DM. Of the 44 patients with SS, nearly all patients were screened for ILD with PFTs (38/44, 86%) and PAH with echocardiogram (40/44,  91%). 16 had ILD and 10 had PAH. A total of 4 patients had both ILD and PAH. All 10 patients with PAH were confirmed with RHC. All patients with ILD had a HRCT at baseline.  Of the 47 patients with PM/DM, about 61% (29/47) of the patients were screened for ILD with PFTs and just 28% (13/47) were screened for PAH with echocardiogram.  18 had ILD, while only 1 had PAH, confirmed on RHC. The 1 patient with PAH, also had ILD. All patients with ILD did have a HRCT obtained at baseline.

Conclusion: In our SS patients, 86% and 91% or our patients were screened for ILD and PAH respectively.  For patients with PM/DM, although our screening percentage for ILD was lower compared to the SS group, the high frequency of ILD noted in PM/DM patients in prior studies suggests that routine screening is warranted. Only 28% of our PM/DM patients were screened for PAH. The association of PAH with PM/DM is not as well documented, however it remains good practice that patients with progressive dyspnea despite optimal treatment for ILD, should undergo evaluation for PAH.   Future studies will assess the ongoing screening patterns, and address the rate of disease progression and the incidence of new onset ILD or PAH in this patient population.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/quality-of-care-in-patients-at-risk-for-pulmonary-involvement-assessment-of-pulmonary-screening-in-systemic-sclerosis-and-myositis-patients-in-a-general-rheumatology-clinic/