| LUNG (PULMONARY): MAIN MENU |
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| Lung (Pulmonary) Overview |
| This page was written by Shelley Ensz, and has not yet been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer. |
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| Overview of Scleroderma Lung Involvement |
| Systemic sclerosis (scleroderma)—such as limited scleroderma, CREST, or diffuse scleroderma—can cause lung problems which may include aspiration pneumonia, lung abscesses, pleural effusions, pulmonary fibrosis, pulmonary hypertension and shrinking lung syndrome. (1-5) |
| Approximately 4% of those with localized scleroderma (such as morphea or linear or en coup de sabre) may develop respiratory symptoms, primarily restrictive pneumopathy. (6) |
| Lung involvement of all types can occur alone, or as part of other illnesses, or, much less commonly, as a manifestation of scleroderma. |
| Unfortunately, systemic scleroderma is often not detected or diagnosed until the last stages of lung involvement, particularly in those who have not developed the overt and classic sign of scleroderma skin hardening, which is known as sclerodactyly. See ISN's free online PDF brochure "What is Scleroderma?" which includes a Systemic Scleroderma Symptom Checklist.) |
| Scleroderma Lung Disease. The strategy in scleroderma lung disease when disease extent is mild on high-resolution computerized tomography (HRCT) is observation. To determine who deserves procrastination and careful follow-up (limited Interstitial Lung Diseases (ILD)) and who requires therapy (extensive ILD) is a timely goal. Red Orbit. 06/03/08. (Also see: James Seibold ) |
| Systemic sclerosis and its pulmonary complications in the Netherlands An epidemiological study. This study clarifies the epidemiology of SSc in the Netherlands and confirms the frequent occurrence of pulmonary complications, based on 654 cases. M C Vonk. Ann Rheum Dis. Published Online First: 29 May 2008. |
| The natural course of progressive systemic sclerosis patients with interstitial lung involvement. Pulmonary symptoms tend to appear at a mean of 7 years after the onset of disease.The first sign of interstitial pulmonary involvement is highest during the first 15 years. Immunosuppressive treatment is not effective in preventing the development of pulmonary involvement. However, it delays the manifestation of pulmonary symptoms for nearly 4 years. PubMed. Clin Rheumatol. 2006 Apr 25. (Also see: Pulmonary Fibrosis) |
| Pulmonary involvement in systemic sclerosis (SSc): Associations with genetic, serologic, sociodemographic, and behavioral factors. Early pulmonary involvement in SSc appears to be influenced by several factors delineated by ethnicity, including racial, socioeconomic, behavioral, and serologic determinants. PubMed. Arthritis Rheum. 2007 Feb 28;57(2):318-326. (Also see: Causes of Scleroderma: Ethnicity, Race, and Geographical Regions ) |
| Organ Involvement-Induced Disability In Systemic Sclerosis (SSc) . SSc-related disability is clinically significant and is mostly related to joint, cutaneous and cardio-pulmonary involvement, as well as to the disease-induced fatigue. C. Mihai. AB0514 EULAR 2007. (Also see: Skeletal, Skin, and Cardiac ) |
| Scleroderma Webcast #1 with Dr. Art Theodore, BUMC |
 Scleroderma Webcast #1 Replay and Scleroderma Webcast Transcript (PDF). Dr. Art Theodore is a nationally recognized expert with special interests in systemic sclerosis and interstitial lung disease from Boston University Medical Campus. 10-30-07. PatientPower. (Also see: What is Scleroderma? ) |
| Scleroderma Webcast #2 with Dr. Art Theodore, BUMC |
| Scleroderma Webcast #2 Replay. Dr. Art Theodore is a nationally recognized expert with special interests in systemic sclerosis and interstitial lung disease from Boston University Medical Campus. 04-25-08. PatientPower. (Also see: What is Scleroderma? ) |
| Symptoms of Scleroderma Pulmonary Involvement |
| Exercise Performance In Systemic Sclerosis: Relationship With Disease Manifestations. Exercise performance is impaired in most SSc patients, at least in those admitted in a referral clinic. The impairment of exercise performance is correlated to lung, heart and skin involvement. L. Ruocco AB0525 EULAR 2007 (Also see: Effects of SSc, Cardiac,and Skin Involvement ) |
| Exhaled Nitric Oxide (NO) Levels in Systemic Sclerosis (SSc) With and Without Pulmonary Involvement. Our results indicate that exhaled air NO concentrations are lower in SSc patients with lung involvement than in those without, and that SSc patients without ILD or PH have higher exhaled NO values than healthy subjects. Chest. 2007; 132:575-580. |
| Shortness of Breath This worrisome symptom has many acute and chronic causes. Follow this flowchart for more information about the diseases in which shortness of breath occurs. familydoctor.org |
| 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 |
| Diagnosis of Scleroderma Pulmonary Involvement |
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| Severity and Prognosis for Scleroderma Pulmonary Involvement |
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| Aspiration Pneumonia |
| Aspiration pneumonia occurs when vomitus or reflux gets into the lungs, causing an often deadly form of pneumonia. Anyone (with or without scleroderma) can get aspiration pneumonia. It often occurs due to esophagus problems. (See: Aspiration Pneumonia and Heartburn/Reflux) |
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| Autoimmune Interstitial Pneumonia |
| Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. In the presentation of an idiopathic interstitial pneumonia, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis.. PubMed. Chest. 2006 Oct;130(4):976-81. (Also see: Diffuse Without Skin Involvement and Pulmonary Involvement ) |
| Role of Endothelial Damage in the Pathogenesis of Interstitial Pneumonitis in Patients with Polymyositis and Dermatomyositis. Our data suggest that endothelial damage might play an important role through the production of fibrosis-enhancing factors such as TGF-ß or ET-1 in PM/DM. J Rheumatol 2006 May;33:903-6. (Also see: Dermatomyositis and Polymyositis) |
| Small cell lung cancer associated with systemic sclerosis. A 51-year-old woman developed small cell lung cancer (SCLC). She was a non-smoker and had interstitial pneumonia associated with systemic sclerosis (SSc). Since SCLC is very rare in non-smokers, these findings suggest a positive association between SCLC and interstitial pneumonia associated with SSc. PubMed. Intern Med. 2005 Apr;44(4):315-8. (Also see: Scleroderma and Cancer) |
| Idiopathic non-specific interstitial pneumonia: as an "autoimmune interstitial pneumonia". Since generalized symptoms were not observed in patients with idiopathic NSIP, and clinical and pathological features were identical to NSIP with several autoimmune disorders, we postulate new clinical entities of "autoimmune interstitial pneumonia" in cases without underlying diseases. PubMed. Respir Med. 2005 Feb;99(2):234-40. |
| Lung Cancer |
| Systemic sclerosis (scleroderma) may be associated with an increased incidence of cancer, including breast cancer, B-cell lymphoma, lung cancer and tongue cancer. (See: Scleroderma and Cancer) |
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| Oxygen Therapy |
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| Pleural Effusion |
| Pleural Effusion is fluid in the lining of the lungs. In scleroderma, this is usually caused by inflammation (rather than infection.) Sometimes there are no symptoms, and it is just discovered on a chest x-ray. The classic symptom is sharp chest pain, which is worsened by taking a deep breath. Other symptoms include shortness of breath, cough, hiccups, rapid breathing, abdominal pain, and joint stiffness. ISN. |
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| Pulmonary Fibrosis |
| Pulmonary (Lung) Fibrosis is a scarring of the lungs, and is the consequence of untreated pulmonary inflammation (alveolitis). It is often also referred to as interstitial lung disease. Many other diseases can also cause pulmonary fibrosis. ISN. |
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| Pulmonary Hypertension |
| Pulmonary Hypertension (PH) is high blood pressure in the arteries which take blood between the heart and lungs. It can be caused by scleroderma and also by other diseases. ISN. |
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| Pulmonary Veno-Occlusive Disease |
| Pulmonary veno-occlusive disease in a patient with scleroderma and the CREST syndrome. Clinical signs of pulmonary hypertension without evidence of left ventricular failure may give rise to suspicion of the disease, and high-resolution CT of the lungs with relatively specific findings can be helpful. PubMed. Tidsskr Nor Laegeforen. 2004 Jan 9;123(23):3391-2. |
| Shrinking Lung Syndrome |
| Shrinking lung syndrome and systemic autoimmune disease. Shrinking lung syndrome is rare but must be considered in patients with autoimmune disease and dyspnea. The diagnosis can be difficult because of clinical, pathological and functional features which are controversial. PubMed. Rev Med Interne. 2004 Jan; 25(1): 83-90. |
| Research on Scleroderma Pulmonary Involvement |
| Pulmonary-renal syndrome (PRS) in systemic sclerosis: a report of three cases and review of the literature. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy. PubMed. Mod Rheumatol. 2007;17(1):37-44. (Also see: Renal Involvement, Medications, and Clinical Trials: Negative Results ) |
| Mixed connective tissue disease (MCTD): a case with scleroderma renal crisis following abortion. Immune response to U1-ribonucleoprotein is the defining serological feature of MCTD. We report a case of MCTD with pulmonary involvement that developed scleroderma renal crisis after an abortion. PubMed. Clin Rheumatol. 2006 Nov 21. (Also see: MCTD and Renal Involvement ) |
| Increased Alveolar Concentration of Exhaled Nitric Oxide is Associated with Lung Disease in Systemic Sclerosis. Alveolar concentration of exhaled nitric oxide (NO) is markedly increased in patients with systemic sclerosis (SSc) as compared with healthy controls and asthmatic patients, perhaps from alveolar inflammation in SSc. K. Tiev. FRI0154 EULAR 2005. |
| Correlation of the degree of dyspnea with health-related quality of life, functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: Results from the scleroderma lung study. The SF-36 (Short Form) complements the BDI and VAS scores for breathing in scleroderma lung disease and is variably correlated with results of pulmonary function tests, suggesting that the SF-36 should be included as an outcome measure in intervention trials in this population. PubMed. Arthritis Rheum. 2005 Feb;52(2):592-600. |
| Identification of Inflammatory Cytokines Profile in Bronchoalveolar Lavage Fluid (BAL) of a Group of Patients with Local (SSL) and Diffuse (SSD) Systemic Scleroderma. These findings show an inflammatory profile in SS significantly different from healthy controls and a tendency of SSD to have higher levels of inflammatory cytokines than SSL which could reach statistical significance with a higher sample of patients. L. M. Avila. AB0120 EULAR 2004. |
| Esophageal Dysfunction in Patients with Definite and Early Systemic Sclerosis. Esophageal manometry can detect abnormalities in a sizeable proportion of patients with early SSc not fulfilling the ACR criteria, including asymptomatic patients. The correlation between LES (lower esophageal sphincter) abnormalities and FVC (Forced Vital Capacity) suggests a possible causal relationship between these disease manifestations. P. Airo'. FRI0285 EULAR 2004. (Also see: Esophageal Involvement) |
| References |
| (1) Pleuropulmonary Manifestations of Collagen Vascular Disease by Gordon L. Weisbrod, M.D., F.R.C.P.C. of The University of Toronto. |
| (2) "Pulmonary and Cardiac Involvement in Scleroderma" by Barbara White, M.D., Professor of Medicine, University of Maryland, Baltimore, Maryland in the SF Newsline, Vol. 1 - No. 3, Summer 1998. |
| (3) Pulmonary Disorders The Merck Manual |
| (4) Scleroderma Lung Disease: A Common Phenomenon in a Rare Disease (This article used to be on Medscape.com.) Note: Medscape requires (free) sign up and password. You do not need to be a medical professional to use Medscape. However this makes for slower access to the site for first-time users. |
| (5) Shrinking lung syndrome and systemic autoimmune disease. Shrinking lung syndrome is rare but must be considered in patients with autoimmune disease and dyspnea. The diagnosis can be difficult because of clinical, pathological and functional features which are controversial. PubMed. Rev Med Interne. 2004 Jan; 25(1): 83-90. |
| (6) Is Juvenile Localized Scleroderma really "LOCALIZED"? One fourth of JLS patients in this data series presented various kind of extra-cutaneous manifestations, sometimes with multiorgan involvement. For this reason, the term "localized" is somehow inappropriate. 688 patients with JLS entered the study. 177 (25.7%) presented various non-cutaneous manifestations that included osteoarticular 48%, neurological 14.7%, ocular 13%, vascular 10%, gastrointestinal 5.6%, respiratory 3.9%, cardiac 2,8%, renal involvement 2.3%. Respiratory symptoms, reported in 7 patients, were essentially due to restrictive pneumopathy. Francesco Zulian. ACR Conference Oct. 2003. (Also see: Types of Scleroderma and Linear) |
