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DIAGNOSIS:Scleroderma
The images shown reveal subpleural and basal interlobular septal thickening, ground glass opacities and interstitial fibrosis with honeycombing. These features are typically seen with idiopathic pulmonary fibrosis, either with UIP (usual interstitial pneumonia) or NSIP (non-specific interstitial pneumonia with fibrosis). In addition, there is dilatation of lower esophagus, which suggests the diagnosis of scleroderma.
Scleroderma (also called “systemic sclerosis”) is a chronic autoimmune disorder. It is a poorly understood illness that causes widespread hardening of the skin, especially on the hands and face. It may also damage the lungs, heart, kidneys, digestive tract, muscles, and joints
Types of Pulmonary Involvement
About 10% to 15% of people with scleroderma develop severe lung disease during the course of their illness.
Two main clinical manifestations of lung involvement in scleroderma are:
1. Interstitial lung disease (also called fibrosing alveolitis or pulmonary fibrosis)
2. Pulmonary vascular disease, leading to pulmonary hypertension.
Fibrosing alveolitis occurs in more than three-quarters of patients with scleroderma, and vascular disease in at least 10 percent and possibly many more. The incidence of pulmonary hypertension has varied from 10 to 80 percent. It occurs most frequently as an isolated pulmonary complication, but can be secondary to pulmonary fibrosis. Pulmonary hypertension is typically subclinical for many years until the on-going arterial damage results in the new onset of dyspnea. There are also a number of less common manifestations of pulmonary involvement in scleroderma. These may include pleural disease, aspiration pneumonia, bronchiectasis, spontaneous pneumothorax, drug-associated pneumonitis and neoplasm.
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PubMed References for Scleroderma
At any given time this link will provide references from Pubmed, including the most recent updates. Retrievals are designed to include articles available free and also Indian studies. For Indian articles, in case there is no link to the full article,you could check out the archives from the journal's website, through this list.
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