Does CREST syndrome affect the lungs?
Twenty-one percent of patients developed primary lung carcinomas. The CREST syndrome is unique in the spectrum of pulmonary alterations seen in progressive
We report an uncommon case of generalized morphea associated with pulmonary interstitial fibrosis, pulmonary arterial hypertension, and positive Th/To antibodies.
CREST syndrome is a type of scleroderma that affects the skin, small blood vessels, joints, lungs, and other organs. It can cause symptoms such as Raynaud's phenomenon, swelling and stiffness in the joints, thickening and hardening of the skin, difficulty swallowing, and other symptoms.
- Interstitial lung disease.
- Pulmonary vascular disease. Pulmonary hypertension. Thromboembolic disease. Pulmonary capillary hemangiomatosis and veno-occlusive disease.
- Pleural effusion.
- Spontaneous pneumothorax.
- Recurrent aspiration.
- Drug-associated pneumonitis.
- Airways disease.
- Lung cancer.
The leading cause of death in patients with scleroderma is lung fibrosis, according to Mouawad – approximately 40% of those patients will not survive past 10 years. The Food and Drug Administration has approved new drugs for lung fibrosis in these patients.
The most commonly used test to determine this is a high resolution CT scan of the lungs (HRCT).
Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure.
- Calcinosis: Calcium skin deposits.
- Raynaud's phenomenon: A condition in which the blood vessels of the fingers and toes spasm when triggered by factors, such as cold, stress or illness. ...
- Esophageal dysfunction: Problems with the esophagus.
- Sclerodactyly: Skin damage on fingers.
In the typical course of limited scleroderma, the patient first notices Raynaud phenomenon. Over time (usually years), fingers become puffy, then the skin thickens slowly. Internal organ manifestations are delayed for many years.
People with limited CREST scleroderma can usually expect a favorable outlook and normal lifespan if the disease affects only the hands and face. The course of this type of scleroderma still tends to be slowly progressive and, in some cases, may affect internal organs.
Can scleroderma make it hard to breathe?
Scleroderma can also cause the blood pressure to increase in the circulation that goes between the heart and the lungs. This is called pulmonary hypertension. In addition to shortness of breath, pulmonary hypertension can also cause excess fluid in the legs, feet and sometimes around the heart.
With the unusual radiologic manifestation, scleroderma should be also considered in the etiology of multipl pulmonary nodules.
Scleroderma is a severe systemic collagen vascular disease of unknown cause characterized by marked vascular and connective tissue abnormalities. The lungs are commonly involved in scleroderma, ranking only behind the skin, the peripheral vasculature, and the esophagus in frequency of organ involvement.
Scleroderma-associated interstitial lung disease, or “SSc-ILD”, is a chronic lung disease in which scar tissue (“fibrosis”) and/or inflammation builds up in the walls of the air sacs of the lungs in a person with a diagnosis of scleroderma (also called “systemic sclerosis” or “SSc”).
Alternative initial pharmacologic therapy — Tocilizumab, cyclophosphamide, and azathioprine are alternative initial immunosuppressive agents for patients with SSc-ILD. Compared with placebo, tocilizumab has been shown to reduce lung function decline.
People who have localized scleroderma may live an uninterrupted life with only minor symptom experiences and management. On the other hand, those diagnosed with an advanced and systemic version of the disease have a prognosis of anywhere from three to 15 years.
The prognosis of CREST syndrome is relatively good with a long lasting disease duration (>10 years). Two complications are seldom associated with CREST syndrome: digital gangrene with finger losses and pulmonary hypertension (3 to 14% of CREST syndrome).
End stage lung disease was defined as pulmonary hypertension requiring continuous ambulatory iloprost, or pulmonary fibrosis requiring continuous oxygen, or death from a scleroderma related lung disease.
The most commonly used treatment for the lungs is a combination of low-dose steroids (usually 10 mg once daily) together with an immunosuppressant drug. The immunosuppressants that are most frequently used for scleroderma, and both of which come in tablet form, are azathioprine and mycophenolate.
Symptoms of scleroderma associated ILD may include: Chronic coughing that is usually dry. Worsening cough is often the first sign of ILD in scleroderma.
What diseases do most people with scleroderma develop?
What complications can scleroderma cause? People with scleroderma are much more likely to have two other conditions: Raynaud's syndrome and Sjögren's syndrome. Reynaud's syndrome affects small blood vessels in your fingers and toes (your digits).
The most common symptoms of pulmonary sarcoidosis are shortness of breath, which often gets worse with activity; dry cough that will not go away; chest pain; and wheezing. Treatment is generally done to control symptoms or to improve the function of organs affected by the disease. Steroids are often used.
Renal involvement is responsible for half of all scleroderma-related deaths in patients with widespread skin changes, while patients with sclerodactyly alone do not tend to develop any type of renal disease. The mortality in patients with limited skin involvement results from cardiac, pulmonary, and GI causes.
A subtype of limited scleroderma is also known as CREST syndrome. CREST is an acronym for its most prominent clinical features: Calcinosis: calcium deposits under the skin and sometimes in tissues.
If you suffer from CREST syndrome and are no longer able to work because of the effects of your symptoms on your daily functioning, you may be eligible to receive Social Security disability benefits, either through SSDI or SSI.