This Form of Rare Interstitial Lung Disease is Bad News for Scleroderma Patients

According to a story from Scleroderma News, a recent study has recommended that scleroderma patients with a rare, poorly understood form of interstitial lung disease (ILD) should receive regular monitoring and check ups from their doctor. The type of ILD in question is called pleuroparenchymal fibroelastosis (PPFE), and it is associated with a poor prognosis in scleroderma. ILDs are characterized from other types of lung diseases because they cause scarring of the lung tissue.

About Scleroderma

Scleroderma, which is also referred to as systemic sclerosis, describes a group of autoimmune diseases that can cause system-wide effects in the most severe cases. The mechanism of this disease is believed to be an autoimmune response in which the immune system mistakenly attacks body tissue. Some factors that may contribute to triggering the autoimmune response include mutations of the HLA genes and exposure to certain materials, such as certain solvents, white spirits, ketones, and silica. Symptoms are broad ranging and systemic, including kidney failure, erectile dysfunction, fatigue, stroke, headaches, facial pain, congestive heart failure, skin abnormalities, high blood pressure, chest pain, indigestion, and many more. Treatments are varied and depend on the symptoms, but most patients take medications in an attempt to suppress the autoimmune response. In severe cases, life expectancy is around 11 years from onset. To learn more about scleroderma, click here.

About Pleuroparenchymal Fibroelastosis (PPFE)

PPFE was only just discovered a few years ago. It is a progressive disease that primarily affects the upper sections of the lungs. The visceral pleura, which is a thin membrane on that surrounds the lungs, becomes thickened and scarred. As the appearance of lesions associated with PPFE is a bad sign for scleroderma patients, the researchers emphasize that more study of the disease in this group of patients is critical and that doctors should be vigilant about identifying PPFE because of its negative impact on patient prognosis.

An evaluation of scleroderma patients saw that those with PPFE were more likely to be older and often had a lower body mass index. These patients saw their lung function decline more rapidly than patients without the illness and were more likely to have other lung-related problems.

Check out the original study here.


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