Background/Purpose:

Systemic Sclerosis (SSc) is a rare heterogeneous autoimmune connective tissue disease.Pulmonary involvement is the major cause of mortality and morbidity in Systemic sclerosis. The aim of the study is to provide a single center experience of SSc patients diagnosed at a tertiary care center over a continuous period of 10 years, in regards to the extent and characteristics of interstitial lung disease at the time of initial presentation

Methods:

Retrospectively reviewed the charts of all the SSc patients who presented to The University of Connecticut Health Center Rheumatology clinic for the first time from April,2004 to March, 2014. All patients fulfilled the 2013 ACR/EULAR classification criteria for SSc, applied retrospectively at the time of chart review. Information about patient’s demographics, laboratory, imaging studies and procedures including chest x- ray (CXR), high resolution CT (HRCT) of lung , pulmonary function tests ( PFT’s) and esophageal transit time (ETT) done at the time of their initial presentation were collected

Results:

128 patients were identified with either limited cutaneous (111) or diffuse cutaneous (17) variant SSC, of whom 120 (94%) were female and 95 (74%) were white. SCL-70 was positive in 18% and anti-centromere antibody was positive in 32% of patient’s. Complete report of the PFT’s was available in 104 patients. FVC was less than 70% in 20 (19%) patients. Of these, 4 had normal HRCT of the lung and 16 patients had interstitial changes. DLCO was less than 70% in 16 of the 20 patients with abnormal FVC. Of the remaining 84 patients with normal FVC, 10 (12%) patients had abnormal CXR and of the 62 patients with both normal FVC and normal CXR, 15 (24%) patients had an abnormal HRCT.

 Among the 122 patients who had HRCT lung, 52 (43%) had abnormal HRCT including the 18 patients with only minimal ground glass changes and 34 patients with honeycombing and or fibrosis mostly limited to the subpleural space . Among the 52 patients with abnormal HRCT 28 (54%) patients had either an abnormal CXR or FVC. The remaining 24 patients with an abnormal HRCT had a normal FVC and CXR which accounts for 20% of total patients.

Of the 52 patients with abnormal HRCT lung, ETT was normal in 16 (30%) patients and abnormal in 35 (67%) patients.  Of the 70 patients with normal HRCT, ETT was normal in 31(44%) patients and abnormal in 36 (51%) patients. No statistically significant difference was seen in the incidence of ETT in people with and without HRCT lung abnormalities (2 tailed p value was 0.129 using Fisher’s exact test)

Conclusion:

Majority of patients presenting to University of Connecticut Health Center had limited cutaneous variant of SSc .Interstitial lung disease as diagnosed with HRCT lung was seen in more than 40% of patients with Systemic Sclerosis at the time of diagnosis. More than one third of these patients had only minimal ground glass opacities. Even though HRCT lung was abnormal in one fourth of the patients with normal FVC and chest x-ray, none of the patients had ILD severe enough to require treatment. Esophageal dysmotility is seen more often in patients with HRCT lung abnormalities but it was not statistically significant.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-involvement-in-systemic-sclerosis-at-initial-presentation-a-single-center-experience/