Scleroderma Types Systemic Symptoms Lungs

Lung (Pulmonary) Overview

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.)

Symptoms of Scleroderma Pulmonary 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

Severity and Prognosis for Scleroderma Pulmonary Involvement

Pneumonia

Autoimmune Interstitial Pneumonia

Clinicopathological characteristics of lung cancer in patients with systemic sclerosis (SSc). A high proportion of patients had SSc-interstitial lung disease, including non specific interstitial pneumonia and usual interstitial pneumonia. PubMed, Clin Respir J, 08/08/2020. (Also see Cancer)

Clinical characteristics of autoimmune rheumatic disease–related organizing pneumonia (AIRD–OP). Compared with cryptogenic organizing pneumonia patients, AIRD–OP patients are characterized with occult onset but more severe lung involvement and higher recurrence rate. PubMed, Clin Rheumatol, 2018 Apr;37(4):1027-1035.

Aspiration Pneumonia

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

Hypersensitivity Pneumonitis

A systematically derived exposure assessment instrument for Chronic Hypersensitivity Pneumonitis (CHP). This Delphi survey provides items that interstitial lung disease experts agree are important to ask in all patients presenting with suspected CHP and provides basis for a systematically derived CHP exposure assessment instrument. PubMed, Chest, 01/17/2020.

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. (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.

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

Pleural Effusion

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

Pulmonary Fibrosis

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

Pulmonary Hypertension

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

Pulmonary Veno-Occlusive Disease

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

Poor scleroderma-pulmonary hypertension-interstitial lung disease (SSc-PH-ILD) Prognosis May be Linked to Pulmonary Veno-occlusive Disease (PVOD). A high prevalence of PVOD SSc patients with pulmonary hypertension associated with interstitial lung disease may explain the poor prognosis observed in this patient group, according to a new study. Scleroderma News, 03/19/2019.

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.

Case Report: Systemic lupus erythematosus (SLE) presenting as severe alveolar hypoventilation and the shrinking lung syndrome (SLS). The interesting features of this case report consist of the presentation of the SLS as an alveolar hypoventilation with a fatal outcome and the presentation of systemic lupus as SLS. PubMed, Rev Mal Respir. (Also see Symptoms and Complications of Lupus)

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