Lung (Pulmonary) Overview |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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Overview of Scleroderma Lung Involvement |
| Systemic sclerosis (scleroderma) can affect the skin and/or internal organs. One of the ways scleroderma can affect the lungs is by causing pulmonary fibrosis, which is a scarring of the lungs. Lung involvement is the second most common complication of systemic scleroderma, eventually occurring in about 70%, and, unfortunately, it is the most common cause of death. |
| 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. Pulmonary involvement is second in frequency only to esophageal involvement as a visceral complication of systemic sclerosis (SSc) and has surpassed renal involvement as the most common cause of death. Le Pavec J. Clin Rev Allergy Immunol. 2010 Jan 12. (Also see: Esophageal Involvement) |
Symptoms of Scleroderma Pulmonary Involvement |
| Scleroderma Lung-Associated Cough: More Than Meets the Eye? The loss of improvement in cough frequency in parallel with the loss of the physiologic benefit at 24 months suggests that cough may be more than just a nuisance symptom and may be related to inflammation and fibrosis. CHEST Journal, September 2012, Vol 142, No. 3. |
| Dyspnea-related anxiety: The Dutch version of the Breathlessness Beliefs Questionnaire Dyspnea-related anxiety may lead to reduced quality of life and functional disability through fearful avoidance of dyspnea-evoking activity. Steven De Peuter, Chronic Respiratory Disease December 20, 2010. (Also see: Anxiety) |
| Exercise Performance In Systemic Sclerosis: Relationship With Disease Manifestations. Exercise performance is impaired in most SSc patients, and is correlated to lung, heart and skin involvement. L. Ruocco AB0525 EULAR 2007. (Also see: Effects of SSc, Cardiac,and Skin Involvement) |
| 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. ISN. |
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Autoimmune Interstitial Pneumonia |
| Undifferentiated Connective Tissue Disease (UCTD) presenting with prevalent interstitial lung disease: case report and review of literature. The diagnosis of UCTD should be considered when NSIP (nonspecific interstitial pneumonia) is diagnosed even in cases with evident first clinical manifestations of severe respiratory dysfunction. Diagn Pathol. 2011 Jun 7. (Also see: UCTD) |
Lung Cancer |
| Scleroderma and Cancer. Systemic sclerosis (scleroderma) may be associated with an increased incidence of cancer, including breast cancer, B-cell lymphoma, lung cancer and tongue cancer. ISN. |
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Methotrexate and Pulmonary Complications |
| Drug-induced interstitial lung disease: mechanisms and best diagnostic approaches. Drug-induced interstitial lung disease (DILD) is not uncommon and has many clinical patterns, ranging from benign infiltrates to life-threatening acute respiratory distress syndrome. Respiratory Research 2012, 13:39. (Also see: Methotrexate) |
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.) 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). 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. 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. |
Restrictive Lung Disease |
| Restrictive Lung Disease. Restrictive lung diseases are characterized by reduced lung volume, either because of an alteration in lung parenchyma or because of a disease of the pleura, chest wall, or neuromuscular apparatus. In physiological terms, restrictive lung diseases are characterized by reduced total lung capacity (TLC), vital capacity, or resting lung volume. eMedicine. |
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) |
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. |
