CREST Syndrome – What is it, Causes, Symptoms and Treatments!

CREST Syndrome – What it is, Causes, Symptoms and Treatments  best suited for this condition. In addition,  CREST Syndrome , initially described as a variant of Systemic Sclerosis, with a more indolent and limited evolution, is characterized by calcinosis, Raynaud’s phenomenon, esophageal motility alteration, sclerodactyly and telangiectasias.

Studies have shown that, although with a slow clinical evolution, visceral abnormalities are not as rare as previously thought. Pulmonary alterations are frequent, namely pulmonary hypertension, which is a major cause of morbidity and mortality in this CREST Syndrome .

CREST Syndrome is the  former name of so-called limited cutaneous systemic sclerosis. In contrast to diffuse scleroderma, limited scleroderma is restricted to the fingers or distal limbs (no trunk involvement) and has a more benign and painless course. So, check out more about CREST Syndrome – What it is, Causes, Symptoms and Treatments:

Causes of CREST  Syndrome: Crest Syndrome is a set of conditions of as yet unknown cause, characterized by hardening of the tissues of the face and limb joints, causing hands bent like claws to look and adopt an expressionless face appearance due to stiffness.

Signs and Symptoms of CREST Syndrome:  The evolution is uncertain and depends on the fibrotic involvement of each affected organ, although it is not a progressive systemic sclerosis that usually has a worse evolution.

 Raynaud’s Phenomenon : This is a condition in which the small blood vessels in the hands or feet react in response to cold or anxiety . Initially they are white and cold, then they become bluish. With the return of the blood flow, they return to their normal color, they become reddish. In the most severe cases the tissues of the fingers can be damaged, leading to ulcers, scarring or gangrene.
Esophageal Dysfunction: This occurs when the smooth muscle of the esophagus loses normal movement. At the top of it, the result can lead to swallowing difficulties, while at the bottom, the problem can cause chronic heartburn or inflammation.
Sclerodactyly:results from excess collagen deposits within layers of the skin making it thick and tight in the fingers. This problem makes it more difficult to bend or straighten the fingers. The skin may also appear shiny and dark with hair loss .
Telangiectasia: These are small red spots on the hands and face caused by the swelling of small blood vessels. While not painful, blemishes can create cosmetic problems.

Treatment For CREST Syndrome:  Treatment for Crest Syndrome  is based on the use of supportive measures. Raynaud’s phenomenon can be controlled by vasodilators, especially with calcium channel blockers . Gastroesophageal reflux disease should be treated with proton pump inhibitors. To control renal crisis, ACE inhibitors are recommended.

Lung lesions, such as acute interstitial pneumonitis, can be controlled with the use of glucocorticoids and immunosuppressive drugs, such as azathioprine or cyclophosphamide. The D-penicilamide regimen is widely used in patients with cutaneous and pulmonary involvement.

D-penicillamine is applied, but symptomatic drugs are usually used for each affected organ. Interferon B1b has been used for a few years with great hope. The evolution is quite unpredictable, depending on the involvement of each organ.