CASE 9074 Published on 17.03.2011

Scleroderma: A focus on pulmonary findings

Section

Chest imaging

Case Type

Clinical Cases

Authors

Kalra V1, Mirza K2, Shin MS3

[1] Yale University; [2]Ross University School of Medicine; [3]Chest Radiology, Yale University.

Patient

58 years, female

Categories
Area of Interest Oesophagus, Lung ; Imaging Technique Digital radiography, CT, CT-High Resolution
Clinical History
A 58-year-old woman with chronic dyspnoea and reflux undergoes evaluation for worsening shortness of breath. The patient has a chronic medical condition and is on immunosuppressive medication. Chest radiograph, CT, and high-resolution CT (HRCT) are performed.
Imaging Findings
The chest radiograph shows increased interstitial markings and decreased lung volumes. Associated esophageal dilation with an air-fluid level, as seen in this case (Fig. 1), suggests the diagnosis of scleroderma. Colonic dilation, an additional manifestation of the disease's smooth muscle involvement, is also seen (Fig 1). Although not appreciated on the chest radiograph, pulmonary arterial dilation from pulmonary hypertension is seen on CT (Fig. 2b).

HRCT shows the classic pulmonary findings in scleroderma, which cosist of a non-specific interstitial pneumonia (NSIP) pattern. These findings are most apparent in the lower lobes and include reticulonodular interlobular septal thickening, subpleural ground-glass opacities, and traction bronchiectasis (Fig. 3a-c). There is notable absence of pleural-based honeycombing, which is seen in usual interstitial pneumonia (UIP) / idiopathic pulmonary fibrosis (IPF), and not NSIP. Predominant involvement of the lower lobes, with extensive volume loss, can be appreciated on the coronal reformat (Fig. 4). Esophageal dilation is also noted (Fig. 2a, b and Fig 3c).
Discussion
Scleroderma is a collagen vascular disease of unknown etiology that leads to inflammation and fibrosis in multiple organ systems including the skin, GI tract, heart, and lungs. Pathogenesis involves inflammatory mediators from alveolar macrophages causing fibrotic changes in the lung. Similar to other immune-mediated diseases, it presents most commonly in middle-aged women. Two main variants are the limited and systemic forms, with the limited form obsoletely described as CREST syndrome (Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) [1]. Clinical pulmonary findings include dyspnea on exertion and dry cough.

The most common radiographic findings in scleroderma are those of the respiratory tract. Interstitial lung disease occurs in approximately 80% of patients and pulmonary hypertension occurs in up to 50% of patients [2]. Pulmonary radiographic findings include a basilar irregular reticular or reticulonodular pattern with interlobular septal thickening, ground-glass opacities, traction bronchiectasis, and pleural thickening. High resolution CT (HRCT) in scleroderma more closely resembles non-specific interstitial pneumonia (NSIP) with pulmonary fibrosis that is less coarse with more ground glass opacification compared to usual interstitial pneumonia (UIP) / idiopathic pulmonary fibrosis (IPF). Scleroderma rarely has multiple subpleural cysts termed “honeycombing” or findings of upper lobe fibrosis characteristic of UIP. Histopathologic and survival data in scleroderma patients with pulmonary findings also more closely resemble those seen in patients with idiopathic NSIP rather than UIP/ IPF. [3]

HRCT is superior to chest radiograph in detecting pulmonary findings; however, the degree of fibrosis does not correlate to clinical presentation [4]. There is asymptomatic esophageal dilatation in 80% of cases and mediastinal adenopathy in 60% [5]. Subpleural cysts, seen in the lower lobes, can cause spontaneous pneumothorax. Alveolar infiltrates are occasionally seen and can be secondary to aspiration from esophageal dysmotility.

Differential diagnosis includes other collagen vascular diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis, Sjogren's syndrome, dermatomyositis-polymyositis, and mixed connective tissue disease. Lung disease secondary to rheumatoid arthritis is associated with air trapping, mosaic perfusion, and cavitating subpleural rounded opacities. Lung disease associated with SLE is more frequently seen with pleural and pericardial effusions. Less likely differentials include asbestosis which would have calcific pleural plaques with less ground-glass opacities and bronchiectases; or drug-toxicity which would have more ground-glass opacities [6].

Clinical diagnosis is supported with serologic markers of specific autoantibodies, bronchoalveolar lavage, and rarely, lung biopsy. Patients with scleroderma have increased risk of lung cancer [7]. The disease progresses towards fibrosis and respiratory failure. Steroids and other immunosupressants are used to manage disease progression which may lead to further pulmonary complications of drug-toxicity and opportunistic infections. Pulmonary disease has become the leading cause of death in scleroderma patients with the advent of improvements in the treatment of renal complications [8].

Teaching Point:
Radiographic findings of a dilated esophagus and HRCT findings consistent with an NSIP pattern are suggestive of scleroderma.
Differential Diagnosis List
Scleroderma, Systemic sclerosis
Systemic lupus erythematosus (SLE)
Rheumatoid arthritis
Sjogren`s syndrome
Dermatomyositis-Polymyositis
Mixed connective tissue disease
Nonspecific interstitial pneumonitis (NSIP)
Usual interstitial pneumonia (UIP)
Asbestosis
Drug-toxicity
Final Diagnosis
Scleroderma, Systemic sclerosis
Case information
URL: https://www.eurorad.org/case/9074
DOI: 10.1594/EURORAD/CASE.9074
ISSN: 1563-4086