Correlation between Pulmonary Fibrosis and other Diseases or Complications

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Lung and Esophageal
Pulmonary Fibrosis and:
—Connective Tissue Disease
—Pulmonary Hypertension
—Thyroid Disease
Patient Stories


Pulmonary fibrosis complications iconPulmonary fibrosis can be correlated or complicated with the following conditions, such as esophageal issues, cancer, connective tissue diseases, heart problems, infections, pulmonary hypertension and thyroid diseases.

Lung Involvement and Esophageal

Worsening of esophageal dilatation is associated with increase in a high-resolution computed tomography (HRCT) score in early systemic sclerosis-associated interstitial lung disease (Ssc-ILD). Our study cohort found that a worsening esophageal diameter was a predictor of progression of lung fibrosis determined by HRCT score in early SSc-ILD. PubMed, Clin Rheumatol, 08/15/2020. (Also see Gastrointestinal Involvement)

The role of high-resolution manometry in the assessment of upper gastrointestinal involvement in systemic sclerosis: a systematic review. Esophageal body dysmotility on HRM positively correlates with the presence of interstitial lung disease. PubMed, Clin Rheumatol, 11/11/2019. (Also see Dysmotility Syndrome)

Gastroesophageal Reflux Disease (GERD) in Idiopathic Pulmonary Fibrosis (IPF): Uncertainties and Controversies. The present review aims to discuss the latest data on the controversial association of IPF and GERD. PubMed, Respiration, 2018;96(6):571-587. (Also see Aspiration Pneumonia)

Consider GERD in patients with pulmonary diseases. Gastroesophageal reflux disease (GERD) has been linked to many forms of pulmonary disease. However, because not all presentations of lung diseases include GERD’s more commonly recognized symptoms, it may be missed as a contributing factor. American College of Physicians. (Also see Esophageal Involvement)

Pulmonary Fibrosis and Cancer

Interstitial Lung Disease (ILD) Associated with Lung Cancer (LC): A Case-Control Study. ILD was a major prognosis factor for LC and should be taken into consideration for LC management. PubMed, J Clin Med, 2020 Mar 5;9(3):700. (Also see Cancer)

Pulmonary Fibrosis and Connective Tissue Disease

Radiological images of interstitial pneumonia in mixed connective tissue disease (MCTD) compared with scleroderma (SSc) and polymyositis/dermatomyositis(PM/DM). HRCT images in MCTD patients can be classified as SSc pattern or PM/DM pattern. PubMed, Eur J Radiol, 2018 Oct;107:26-32. (Also see Mixed Connective Tissue Disease and Dermatomyositis and Polymyositis)

CT Features of the Usual Interstitial Pneumonia (UIP) Pattern: Differentiating Connective Tissue Disease–Associated Interstitial Lung Disease (CTD–ILD) From Idiopathic Pulmonary Fibrosis (IPF). Although UIP is usually associated with IPF, the index of suspicion for CTD-ILD should be raised in the care of patients with any of the three CT signs. PubMed, AJR Am J Roentgenol, 2018 Feb;210(2):307-313.

Lung involvement in connective tissue diseases (CTD): A comprehensive review and a focus on rheumatoid arthritis. Rheumatoid Arthritis (RA), unlike other rheumatic disorders, resembles Idiopathic Pulmonary Fibrosis (IPF) in numerous aspects. PubMed, Autoimmun Rev. (Also see:Rheumatoid Arthritis Symptoms)

Pulmonary Fibrosis and COV-19

Infection or autoimmunity? The clinical challenge of interstitial lung disease in systemic sclerosis during COVID 19 pandemic. The striking CT similarities between the 2 diseases make difficult to distinguish a worsening of SSc-ILD from COVID-19 superinfection. PubMed, J Rheumatol, 2020 Dec 1;jrheum.200832.

The Systemic Sclerosis Patient in the COVID-19 Era: The Challenging Crossroad Between Immunosuppression, Differential Diagnosis and Long-Term Psychological Distress. The rheumatologist in daily clinical practice should carefully differentiate the possible COVID-19 infection in order to optimize the patient management. PubMed, Clin Rheumatol, 2020 Jun 8;1-5.

Pulmonary Fibrosis and Heart Complications

Association of serum homocysteine level and lung involvement in systemic sclerosis (SSc). It seems that the serum level of homocystein has a minor role in the lung involvement of SSc patients or its effects are modified by other factors. PubMed, Curr Rheumatol Rev, 06/28/2018.

Free light chains (FLC) of immunoglobulins in patients with systemic sclerosis (SSc): correlations with lung involvement and inflammatory milieu. FLC levels are elevated in SSc and high levels are associated with lung involvement and with a higher degree of inflammation, supporting a possible role of B cell activation in the pathophysiology of the disease. PubMed, J Clin Pathol, 01/31/2018. (Also see Research about Scleroderma Cardiac (Heart) Involvement)

Pulmonary Fibrosis and Infection

Respiratory tract infections. Respiratory tract infections (RTIs) are any infection of the sinuses, throat, airways or lungs. They’re usually caused by viruses, but can be caused by bacteria. NHS Choices.

Case Report: Interstitial Lung Disease in Systemic Scleroderma, Complicated with Bilateral Pulmonary Aspergilloma: An Unusual Association. Management of aspergilloma in a background of extensive interstitial lung disease remains poorly defined and complicated and overall prognosis is unfavourable. PubMed, J Clin Diagn Res.

Pulmonary Fibrosis and Pulmonary Hypertension

Relationship of main pulmonary artery diameter to pulmonary arterial pressure (PH) in scleroderma patients with and without interstitial fibrosis. In scleroderma patients, an enlarged main pulmonary artery predicts PH even in the presence of mild to moderate fibrosis. PubMed, J Comput Assist Tomogr. (Also see Pulmonary Hypertension)

Pulmonary Fibrosis and Thyroid Disease

Severe hypothyroidism can cause apparent pulmonary fibrosis, which is reversible with correction of hypothyroidism. (Also see Hashimoto's Thyroiditis)

Patient Stories

Brenda M: Primary Biliary Cirrhosis (PBC), Fibromyalgia, Pulmonary Fibrosis, and Sjogren's Syndrome In 1965, when I was twenty-six, I went to work for a firm of electro-platers, which was mainly aircraft work…

Debbie G: CREST, Lupus, Interstitial Lung Disease and Pulmonary Hypertension It has been a very long and bumpy road at times but somehow through all the hospital visits and other illnesses I have managed to stay ahead of the game by a step or two…

Don Alfera: CREST, Pulmonary Fibrosis and Sjögren's I entitled this "My Time So Far" since this has been a journey unlike any that I could have ever tried to imagine in my life…

Tony B: Systemic Scleroderma with Pulmonary Fibrosis, Pulmonary Hypertension and Raynaud's It has taken me some time to come to terms with this disease…

Judy Tarro: Diffuse Scleroderma, Sjogren's, Pulmonary Fibrosis and Pulmonary Hypertension The best we can figure, I was diagnosed with diffuse scleroderma in 1956. I am now webmaster of SD World…

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