Arthritis More Arthritis Types & Related Conditions What Is CREST Syndrome? By Rachael Zimlich, BSN, RN Rachael is a freelance healthcare writer and critical care nurse based near Cleveland, Ohio. Learn about our editorial process Rachael Zimlich, BSN, RN Updated on September 17, 2020 Print Table of Contents View All Table of Contents Affected Systems Symptoms Causes Diagnosis Treatment Prognosis CREST syndrome is a complicated disease that affects several parts of the body. It can cause the thickening and/or hardening of the skin and internal organs. CREST is the acronym used to describe a number of conditions within the category of systemic sclerosis (scleroderma). These diseases include: Calcinosis: Calcium deposits form on skin.Raynaud's phenomemon: Hands and feet have an abnormal response to cold temperatures or anxiety. Blood vessels narrow when you are cold or stressed, turning fingers and toes white or blue before blood flow is restored and they return to a normal color.Esophageal dysmotility: The smooth muscles that line the esophagus lose normal movement and function.Sclerodactyly: Extra collagen deposits on the skin on your hands. The result is thick, tight skin on the fingers. Telangiectasia: Tiny red spots on your hands and face caused by swelling of the small blood vessels in those areas. CREST syndrome is also called limited cutaneous systemic sclerosis or limited scleroderma. Scleroderma can appear as a localized issue affecting only small areas of the body at a time (known as linear scleroderma or morphea), or as a systemic problem affecting the entire body. This rare connective tissue disease becomes more serious as it progresses to systemic disease. Barb Elkin / iStock / Getty Images Affected Systems Systemic sclerosis affects a number of body systems—not just one area. CREST syndrome is an umbrella term combining a number of specific conditions related to scleroderma, each affecting different parts of the body. The body systems most commonly affected by scleroderma are: SkinGastrointestinal tractLungsKidneysSkeletal musclesPericardium Within these systems, connective tissues are the specific areas affected. For skin, this will appear as a visible thickening or hardening. For other organs, the effect can be more difficult to see. In short, systemic sclerosis causes the lining, or "skin" of organs to harden and not function well. This dysfunction can cause major problems, such as in the esophagus, where the smooth muscles that line the organ don't contract and move food effectively. Symptoms of CREST Syndrome Symptoms of systemic sclerosis can vary based on the body system being affected. With CREST syndrome, five specific diseases are included which impact the skin, hands, feet, esophagus, and connective tissues of other internal organs. Depending on the body part affected, symptoms can include: Skin swellingItchy skinDiscoloration of the skinHair lossSkin dryness or hardeningUlcers or wounds over jointsArthritisFatigueJoint painContracturesDifficulty swallowingHeartburnCough that follows swallowingVoice hoarsenessBloatingConstipation or diarrheaFecal incontinenceBowel obstructionsShortness of breathHeart diseaseKidney dysfunction and urinary system changesNeuropathiesErectile dysfunction Systemic scleroderma, or the diseases included in CREST syndrome, are most common in females and in people of African-American descent. Causes The exact cause of CREST Syndrome or any of the disorders within this family of diseases is unknown. There is evidence that there are genetic or familial links, with clusters of these diseases present in some families. It's believed in these cases to develop as an autoimmune disease. Not all cases are inherited, though. There are also a number of viruses or environmental factors that have been linked to the development of diseases in the sclerosis family. These include: Cytomegalovirus (CMV)Epstein-Barr virus (EBV)Parvovirus B19Silica dust exposureExposure to various organic solvents and chemicalsCigarette smoking and other drug use Diagnosis Diagnosis of CREST syndrome can be complicated, and may come over time after diagnosis or a number of the conditions associated with the syndrome. A physical assessment by your doctor is the first step. Your doctor may suspect CREST syndrome if they see: Puffy or swollen fingersThickening skin on the hands, feet, face, or armsTightening of the skin around the mouthPitting in the skin on your fingertips, or wounds around your nailbedsWhite bumps on the skin that signals the accumulation of calicum, known as calcinosis cutisPain and air accumulation under the skin, also known as crepitus—particularly over joints. If your doctor suspects you have one or more of the diseases in the sclerosis family, or all the elements of CREST syndrome, they will probably send you for additional lab testing and procedures. These tests include: Complete blood count (CBC) with differential to indicate anemia or other dysfunction from malabsorption of nutrients related to decreased function in connective tissuesSerum creatinine to measure kidney functionCreatinine kinase to measure the involvement of musclesUrinalysisAntinuclear antibody (ANA) testingAntitopoisomerase I (anti-Scl-70) antibodyAnticentomere (ACA) antibodyAnti-RNA polymerase III antibodyPulmonary function testingImaging studies like a computerized tomography (CT) scan or chest X-rayDoppler echocardiography Treatment Treating CREST syndrome is complicated because the disease process affects each part of the body differently, and different organs require different management. There is no universal treatment for the conditions included in CREST syndrome, but early diagnosis is helpful. Your doctor may prescribe you immunosuppressant medications or steroids to help control the progression of the disease or limit flare-ups. Symptom management is a big part of treating CREST Syndrome. This will look different depending on which area of involvement is causing symptoms. For example, with Reynaud's, you may have to make lifestyle changes such as quitting smoking, or avoiding cold temperatures or reducing stress. When the esophagus or gastrointestinal tract is involved, laxatives may help relieve constipation, or your doctor may prescribe you medication to manage heartburn. Ask your doctor for specific advice to manage your particular symptoms. A healthy diet and exercise regimen may also help keep CREST Syndrome under control. Prognosis While limited sclerosis may cause pain or inconvenience, systemic sclerosis diseases like those in CREST syndrome are associated with a higher mortality rate. The leading cause of mortality in CREST is pulmonary hypertension. Monitoring for this condition via echocardiogram and pulmonary function testing is recommended. Prognosis for patients with severe sclerosis has improved over the last several decades, thanks to improved management of organ failure. A Word From Verywell CREST syndrome is a term used to describe a number of diseases in the sclerosis family. Skin thickening, hardening, or swelling is usually a hallmark of these diseases, and one of the first noticeable symptoms. Focus on maintaining a healthy diet and lifestyle, and develop a close relationship with your physician to help keep your sclerosis from progressing to organ failure. Was this page helpful? Thanks for your feedback! Dealing with chronic inflammation? An anti-inflammatory diet can help. Our free recipe guide shows you the best foods to fight inflammation. Get yours today! Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. What are your concerns? Other Inaccurate Hard to Understand Submit Article Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Cleveland Clinic. Scleroderma. Updated September 16, 2019. Varga J. Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults. UpToDate. Jan. 3, 2020. Stanford Health Care. Scleroderma treatments. 2020. Harvard Health. Scleroderma. December 2018.