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Updated: Jul 3, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Interstitial lung disease: trials and tribulations.
1Division of Pulmonary and Critical Care Medicine, Keck School of Medicine, University of Southern California, Los Angeles, California, USA. kafshar@usc.edu
Current Opinion in Pulmonary Medicine
|July 31, 2008
Summary
Treatments for diffuse parenchymal lung diseases like idiopathic pulmonary fibrosis, scleroderma, and sarcoidosis show varied efficacy. Pulmonary hypertension significantly worsens prognosis, and current therapies offer limited benefit for these complex conditions.
Area of Science:
- Pulmonology
- Rheumatology
- Cardiology
Background:
- Diffuse parenchymal lung diseases (DPDs) encompass idiopathic pulmonary fibrosis (IPF), scleroderma-associated lung disease, and sarcoidosis.
- Pulmonary hypertension (PH) frequently complicates these DPDs, significantly impacting patient outcomes.
- Current therapeutic strategies for DPDs and associated PH have limitations.
Purpose of the Study:
- To review recent advancements in the clinical characteristics, treatment, and prognosis of DPDs and PH.
- To evaluate the effectiveness of current therapies in IPF, scleroderma-associated lung fibrosis, and pulmonary sarcoidosis.
- To assess the impact of PH on survival and the efficacy of PH-specific agents in these patient groups.
Main Methods:
- Literature review of recent clinical trials and studies.
- Analysis of treatment outcomes for specific pharmaceutical agents.
- Evaluation of prognostic factors, including the presence of pulmonary hypertension.
Main Results:
- Bosentan may delay disease progression in IPF.
- Cyclophosphamide offers short-term benefit for scleroderma-associated lung fibrosis.
- Infliximab has not shown efficacy in randomized trials for pulmonary sarcoidosis.
- Pulmonary hypertension is an independent risk factor for mortality.
- Not all PH-specific agents are effective in secondary PH associated with DPDs.
Conclusions:
- Lung fibrosis is a debilitating condition with variable treatment responses.
- Current medical options are not universally effective, necessitating further research into novel therapies.
- Pulmonary hypertension significantly impairs quality of life and survival in DPD patients.
- The efficacy of PH-specific agents is limited in these specific patient populations.
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