Scleroderma Types Systemic Symptoms GI

Scleroderma Esophageal (Throat) Involvement

Overview

Systemic scleroderma can affect the esophagus in many ways. It can cause Barrrett's esophagus, candida, hoarseness, dysmotility, ulcers, heartburn, and throat spasms. Regular thorough testing is necessary to ascertain the extent of involvement.

GI Manifestations With a Focus on the Esophagus: Recent Progress in Understanding Pathogenesis. The improvement of diagnostic tools for esophageal symptoms and dysfunction offer an opportunity to improve the characterization of scleroderma esophageal disease. PubMed, Curr Rheumatol Rep, 2019 Jul 3;21(8):42.

Air Esophagogram

Air in the esophagus is a very unusual x-ray finding, and particularly, when it's not associated with a fluid level. It is indicative of a stricture and should strongly suggest scleroderma.

"This radiological sign, although rare, should suggest scleroderma and may be particularly useful in patients with "systemic sclerosis sine scleroderma."⁽¹⁾ (Also see Esophagus, Gastroparesis, Scleroderma Gastrointestinal Involvement, What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis)

Barrett's Esophagus

Prevalence of Barrett's Esophagus (BE) in Female Patients With Scleroderma (SSc). The high prevalence and incidence of dysplasia found suggest that women with SSc should be included in the screening recommendations for BE. PubMed, Am J Gastroenterol, 2021 Mar 1;116(3):517-521.

Acceptability and Adequacy of a Non-endoscopic Cell Collection Device for Diagnosis of Barrett's Esophagus (BE): Lessons Learned. Diagnostic characteristics suggest that non-endoscopic assessment of BE deserves further development as an alternative to endoscopy. PubMed, Dig Dis Sci, 02/03/2021.

Carol Langenfeld: Diffuse Scleroderma People tell me that I have earned my credentials as a "certified patient" the hard way…

Don Alfera: CREST, Pulmonary Fibrosis and Sjögren's I entitled this "My Time So Far" since this has been a journey unlike any that I could have ever tried to imagine in my life…

Jerri: CREST Syndrome I was twenty-one years of age when I first noticed that my fingers, toes and lips were turning colors…

Sharon: CREST with Limited Scleroderma, Sjögren's and Pulmonary Hypertension (Australia) Day by day I discover another food I cannot eat…

Candida Esophagitis

The "Foamy" Esophagus A Radiographic Sign of Candida Esophagitis. Shows sophagrams of scleroderma patients. American Journal of Roentgenology.

Correlation with Lung Disease

Relationship Between Interstitial Lung Disease (ILD) and Oesophageal Dilatation on Chest High-Resolution Computed Tomography (HRCT) in Patients With Systemic Sclerosis: A Cross-Sectional Study. An increased oesophageal diameter ( ≥ 11 mm) on chest HRCT is associated with pulmonary and oesophageal symptoms, more severe ILD, and lower DLco. PubMed, Radiol Med, 2018 Sep;123(9):655-663. (Also see Pulmonary Fibrosis)

Clinical features of scleroderma patients with contracture of phalanges (CP). Our study suggested that the presence of CP may be a marker of oesophageal involvement, pulmonary fibrosis and heart involvement. PubMed, Clin Rheumato. (Also see Joint Contractures, Pulmonary fibrosis, Cardiac Involvement)

Dysphonia (Hoarseness, Loss of Voice)

Systemic sclerosis (scleroderma) can affect the larynx and cause dysphonia (hoarseness or loss of voice), which can be evaluated with videolaryngostroboscopy.

(Case Report) Achalasia in circumscribed scleroderma. The association of achalasia and circumscribed scleroderma could possibly be based on common autoimmune mechanisms and an analogous pathogenesis with resulting fibrosis. PubMed, Dtsch Med Wochenschr.

Dysphonia (Hoarseness, Loss of Voice) Personal Stories

Leeben: Limited Scleroderma and CREST I had a traumatic forty-five-minute intubation and landed up in the ICU with problems breathing and paralyzed vocal cords. It has been the most devastating thing for me, since the loss of of my voice has caused social isolation and dependence…

Esophageal Diseases

Gastric Juice Expression of Th-17 and T-Reg Related Cytokines in Scleroderma Esophageal Involvement. This is the first report of cytokines measurement in gastric juice of patients with SSc. PubMed, Cells, 2020 Sep 16;9(9):E2106. (Also see Cytokines)

Oesophageal dilatation on HRCT-scan of the lungs as a sign of scleroderma. Oesophageal dilatation as visible on an HRCT-scan of the chest may alert physicians to look for other signs or symptoms of systemic sclerosis (SSc) in these patients, enabling early diagnosis and specific treatment. Ann Rheum Dis.

A case-control study of the pathology of esophageal disease in systemic sclerosis (scleroderma). The loss of circular and longitudinal smooth muscle in the distal scleroderma oesophagus may represent loss of normal neural function followed by secondary tissue atrophy, or may be a primary smooth muscle lesion. PubMed, Gut.

Chest Pain, Chronic Many different types of problems can cause discomfort, shortness of breath, pain with swallowing, and many other symptoms in the chest area. This chart may help you pinpoint your problem as you confirm your symptoms. familydoctor.org

Esophageal Motility Disorders

Abnormal Propulsion of Food. The movement of food from mouth to stomach requires normal and coordinated action of the mouth and throat, propulsive waves of the esophagus, and relaxation of the sphincters. A problem with any of these functions can cause difficulties. Merck.

Esophageal Motility Stories

Amanda: Diffuse Scleroderma Systemic Sclerosis I am thirty-nine years old and was diagnosed with diffuse scleroderma systemic sclerosis in August 2007…

Annette G: MCTD, Systemic Scleroderma, Chronic Kidney Disease, CFS, Fibromyalgia, Trigeminal Neuralgia, TMJ People think that because I am a nurse I have all the answers. Well guess what? I don't…

Daphne M: Daughter of Limited Systemic Scleroderma Patient She has had symptoms of reflux, which has caused her to throw up every night for over ten years. Her hands turn blue and are very sensitive to hot and cold. She has been developing ulcers on her fingers too. She was admitted to CCU last week with difficulty swallowing and breathing…

Dawn M: Linear/Systemic Scleroderma My family and I were informed by the doctors, that the localized/linear form of scleroderma that I was diagnosed with, would never progress into the potentially fatal, systemic form…

Mars: Scleroderma with Full Gastrointestinal Involvement I finally started total parenteral nutrition (TPN, or tubal feeding) in May 2010 and it was an absolute success…

Sonya D: Surviving Daughter of Systemic Scleroderma Patient (Portugal) She had difficulty eating, drinking and digesting her food, but yet doctors had no clue…

Esophageal Spasm

Esophageal spasms. Esophageal spasms are an uncoordinated series of muscle contractions that prevent food from traveling properly from your esophagus to your stomach. These spasms can be very painful. Chest pain is a common symptom of esophageal spasm. Mayo Clinic.

Esophageal Stricture

Esophageal Stricture is an abnormal narrowing of the esophagus.

See Medical Tests: Esophageal Dilation

Esophageal Stricture Personal Stories

Judy R. Thompson Devlin: Diffuse Scleroderma with CREST He said 1 in 200 people get a disease in their lifetime, and that scleroderma was like having 20-30 diseases at once…

Lorrie: Limited Scleroderma/CREST At first I thought, "Oh my goodness! I am going to die." But my doctor assured me that I could very likely live to be an old woman…

Lynsey: Daughter of Patient with Scleroderma and Lupus My mother has had all but four fingers amputated, all of her toes on her left foot and her right leg up to her knee has been amputated…

Esophageal Ulcer

Esophageal Ulcer is an open sore in the esophagus.

A case-controlled study of the pathology of esophageal disease in systemic sclerosis (scleroderma). The loss of circular and longitudinal smooth muscle in the distal scleroderma esophagus may represent loss of normal neural function followed by secondary tissue atrophy, or may be a primary smooth muscle lesion. PubMed, Gut.

Gastric MALT Lymphoma

Systemic sclerosis and gastric MALT lymphoma. MALT (mucosa-associated lymphoid tissue) lymphomas are usually confined to the gastric wall. Gastric MALT lymphomas have a well-established association with H. pylori infection. Eradication of H. pylori is the mainstay of treatment. PubMed, Joint Bone Spine. (Also see Scleroderma and Cancer)

Reflux (Heartburn)

Reflux (heartburn) occurs when acid from the stomach backs up into the esophagus (throat). See Reflux (Heartburn) by ISN.

Hoarseness:Reflux (heartburn) is a frequent cause of dysphonia (hoarseness or loss of voice) in scleroderma.

References

(1) Air esophagogram and intestinal pseudoocclusion in a patient with scleroderma. J Rheumatol, Kraus A, Alarcón-Segovia D, Huddinge, University Hospital, Sweden

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