Systemic sclerosis is a multisystem disease of connective tissue that is accompanied by vasculopathy. Scleroderma, its original name, means hard skin.
Progressive systemic sclerosis (PSS) is a connective tissue disease associated with small vessel arterial vasculopathy and inflammatory and immunologic processes. The peak incidence is between 45 and 64 years of age.
Clinically, PSS is classified as diffuse or limited depending on the distribution of skin disease. PSS is considered limited when involvement is restricted to the distal extremities and face. The limited form is associated with a lower risk of visceral involvement, although pulmonary hypertension is more common.
(See the images below.)
Posteroanterior chest radiograph reveals irregular reticular opacities in the lower lobes. Two years later, the findings are more marked.
High-resolution CT of biopsy-proven nonspecific interstitial pneumonia. The image shows abnormal reticular opacities in a background of ground-glass opacity, with associated traction bronchiectasis indicating lung fibrosis.
Diffuse cutaneous systemic sclerosis (dcSSc) and limited cutaneous systemic sclerosis (lcSSc) scleroderma overlap the following syndromes
CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, skin pigmentation, telangiectasias)
Mixed connective tissue disease (controversial whether this is a separate entity or an intermediate stage in the progression to connective tissue disease)
Scleromyositis (coexisting features of dermatomyositis without features of systemic lupus erythematosus)
Polymyositis synthetase (interstitial lung disease, myositis, Raynaud phenomenon, arthritis)
Morphea (focal cutaneous scleroderma)
Chest radiography is insensitive, since findings are abnormal in only two thirds of patients with pulmonary disease.
High-resolution computed tomography (HRCT) scanning is the best imaging test for assessing the extent and severity of pulmonary disease. Reported detection of fibrosis with HRCT is 60-90%, compared with 60-100% at autopsy.
HRCT findings are abnormal in most patients with functional impairment. False-negative HRCT imaging study findings have occurred in the setting of alveolitis, as documented by Remy-Jardin et al using bronchoalveolar lavage in patients with normal pulmonary function tests.
Echocardiographic measurements of systolic pulmonary arterial pressure correlate well with right heart catheterization values.
Radiographs in women who are pregnant are limited to a single PA view to minimize radiation exposure. Shielding is used when appropriate. If HRCT examination is considered essential during pregnancy, it can be tailored to minimize radiation exposure.
A collaborative approach between pulmonologists and thoracic radiologists is important in the diagnosis and management of interstitial lung disease. Analysis of HRCT images of the chest can guide video-assisted thoracoscopic lung biopsy. An increased incidence of lung cancer is seen in patients with scleroderma, and the radiologist is involved in detection and diagnosis through CT-guided percutaneous lung biopsy.
Perform percutaneous drainage of complicated parapneumonic effusions and empyemas with CT or ultrasonographic guidance.