Methotrexate Lung Complications
Pulmonary Veno-Occlusive Disease
Restrictive Lung Disease
Shrinking Lung Syndrome
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.
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.)
Dysregulation of angiogenic homeostasis in systemic sclerosis (SSc). Increased serum endostatin is associated with skin sclerosis severity and pulmonary fibrosis and favors SSc disease progression. PubMed, International Journal of Rheumatic Diseases, 2 Jul 2013. (Also see Common Medical Tests for Scleroderma and Prognosis)
Scleroderma (SSc) lung disease. Interstitial lung disease (ILD) and Pulmonary Hypertension (PH), are the leading causes of death in SSc patients and so early recognition of lung involvement is essential for the care of these patients. Eur Respir Rev, 03/01/2013.
Thoracic Manifestations of Connective Tissue Disease. The collagen vascular diseases that most commonly involve the lung are rheumatoid arthritis, progressive systemic sclerosis, systemic lupus erythematosus, polymyositis and dermatomyositis, mixed connective tissue disease, and Sjögren syndrome. RadioGraphics, January 2012, pp. 32, 33-50.
The global peripheral chemoreflex drive (GPCD) in patients with systemic sclerosis (SSc): a rebreathing and exercise study. The GPCD was diminished in dcSSc patients, suggesting an altered control of breathing and its assessment may help the clinician to better understand reported exercise intolerance and exertional dyspnea in dcSSc patients. PubMed, QJM, 2014 Jul 13.
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, SeptemberSeptember 2012, Vol 142, No. 3.
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
Tests for Pulmonary Involvement
|Diagnosis of Pulmonary Fibrosis
Diagnosis of Pulmonary Hypertension
Predictors of Morbidity and Mortality
|Health Related Quality of Life Issues (HRQOL)|
Scleroderma–related Interstitial Lung Disease (SSc–ILD) Not Linked to Idiopathic Interstitial Pneumonia. The study confirmed that SSc–ILD and idiopathic interstitial pneumonia are different diseases, not sharing a genetic basis. Scleroderma News, 02/02/2016. (Also see Pulmonary Fibrosis Research)
An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. This task force proposes the term “interstitial pneumonia with autoimmune features” (IPAF) and offers classification criteria. European Respiratory Journal, 07/09/2015. (Also see UCTD)
Aspiration pneumonia occurs when vomitus or reflux gets into the lungs, causing an often deadly form of pneumonia. ISN.
Correlation with Lung Involvement
He withered away for 7 years. Doctors didn’t realize his passion was killing him. Researchers have found a rare cause for serious lung inflammation:moldy bagpipes. Washington Post, 08/23/2016.
Solitary organizing pneumonia (SOP) in systemic sclerosis (SSc) mimicking lung adenocarcinoma. SOP is often misdiagnosed and removed surgically as it is rare and difficult to distinguish from lung carcinoma. SOP should be considered when a solitary nodular lung lesion is noted in a patient with SSc. PubMed, Arthritis Rheumatol, 2014 Apr 22. (Also see Cancer and Scleroderma)
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.
Gastrointestinal Tract Cancer
Malignant Atrophic Papulosis/Degos
|Medications and Cancer
Myeloma (Bone Marrow Cancer)
T Cell Lymphoma
Tongue and Oral Cancer
|Overview of Oxygen Therapy
Dangers of Oxygen and Flame
Custom Oxygen Tank Covers and Cases
|Oxy View Glasses
Related Patient Stories
Pleural Effusion is fluid in the lining of the lungs. In scleroderma, this is usually caused by inflammation (rather than infection.) ISN.
Pulmonary (Lung) Fibrosis is a scarring of the lungs, and is the consequence of untreated pulmonary inflammation (alveolitis). ISN.
Pulmonary Hypertension (PH) is high blood pressure in the arteries which take blood between the heart and lungs. ISN.
Exercised Induced PH
Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung arteries. MedLine Plus.
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. Medscape.
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.
Reading Voices of Scleroderma Books: Diana Kramer.
Sharing Scleroderma Awareness Bracelets: Deb Martin, Brenda Miller, Vickie Risner.
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