Lung (Pulmonary) Overview

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Lung Cancer
Methotrexate Lung Complications
Pleural Effusion
Pulmonary Fibrosis
Pulmonary Hypertension
Pulmonary Veno-Occlusive Disease
Restrictive Lung Disease
Shrinking Lung Syndrome
Oxygen Therapy
Personal Stories

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.

Approximately 4% of those with localized scleroderma (such as morphea or linear or en coup de sabre) may develop respiratory symptoms, primarily restrictive pneumopathy.

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.

Symptoms of Scleroderma Pulmonary Involvement

7 Reasons You Can’t Stop Coughing. A short–term cough could be a sign of a bigger health issue that needs to be addressed by a doctor. Time Healthland, 11/10/2016.

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.

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.

Diagnosis of Scleroderma Pulmonary Involvement

Tests for Pulmonary Involvement
Diagnosis of Pulmonary Fibrosis
Diagnosis of Pulmonary Hypertension

Severity and Prognosis for Scleroderma Pulmonary Involvement

Predictors of Morbidity and Mortality
Health Related Quality of Life Issues (HRQOL)


Autoimmune Interstitial Pneumonia

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

Aspiration pneumonia occurs when vomitus or reflux gets into the lungs, causing an often deadly form of pneumonia. ISN.

Correlation with Lung Involvement
Personal Stories

Hypersensitivity Pneumonitis

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)

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)

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.

Breast Cancer
Cervical Cancer
Gastrointestinal Tract Cancer
Liver Cancer
Lung Cancer
Malignant Atrophic Papulosis/Degos
MALT Lymphoma
Medications and Cancer
Myeloma (Bone Marrow Cancer)
Non-Hodgkin's Lymphoma
Ovarian Cancer
Prostate Cancer
Thyroid Cancer
T Cell Lymphoma
Tongue and Oral Cancer
Personal Stories

Methotrexate and Pulmonary Complications

The Drug-Induced Repiratory Disease Website. List of over a hundred medications that can cause lung disease or symptoms and also shows the associated patterns. Pneumotox. (Also see Methotrexate)

Oxygen Therapy

Overview of Oxygen Therapy
Dangers of Oxygen and Flame
Custom Oxygen Tank Covers and Cases
Oxy View Glasses
Related Patient Stories

Pleural Effusion

Pleural Effusion is fluid in the lining of the lungs. In scleroderma, this is usually caused by inflammation (rather than infection.) ISN.

Personal Stories

Pulmonary Fibrosis

Pulmonary (Lung) Fibrosis is a scarring of the lungs, and is the consequence of untreated pulmonary inflammation (alveolitis). ISN.

Clinical Trials
Patient Stories

Pulmonary Hypertension

Pulmonary Hypertension (PH) is high blood pressure in the arteries which take blood between the heart and lungs. ISN.

Video Overview
Primary PH
Secondary PH
Exercised Induced PH
Disease Correlations
Research Registries
Patient Support
Personal Stories

Pulmonary Veno-Occlusive Disease

Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung arteries. MedLine Plus.

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. Medscape.

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.

Go to Aspiration Pneumonia
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