12/29/2025
This video, presented by Dr. Alyson Wong, provides a comprehensive overview of Interstitial Lung Disease (ILD) in Systemic Sclerosis (SSc), also known as scleroderma.
Here's a summary of the key topics covered:
Introduction to Interstitial Lung Disease (ILD) (0:55): Dr. Wong explains that ILD is an umbrella term for conditions affecting the interstitium, the "saran wrap layer" that provides structural integrity to the lungs. Inflammation or scarring of this layer can impede gas exchange. ILD can be exposure-related, autoimmune-related (where SSc falls), or idiopathic. The term "Systemic Autoimmune Disease" (SARD) is now used to encompass conditions like SSc, inflammatory muscle diseases, and vasculitis (2:32).
Epidemiology and Impact of ILD in Systemic Sclerosis (3:49): SSc affects many organs, with skin involvement being almost universal. Lung involvement occurs in 65% of people with scleroderma (4:30), and ILD is the leading cause of death in SSc, significantly impacting quality of life and survival (5:05).
Diagnosis of ILD in Scleroderma (6:22):
Symptoms: The most common symptoms are fatigue, shortness of breath, and cough, but many patients are asymptomatic, emphasizing the need for screening (6:28).
Screening Guidelines: New guidelines from 2023 recommend screening for ILD in SSc patients, especially those with high-risk features like anti-CL70 positivity, diffuse skin involvement, male s*x, early disease (within 7 years), and elevated inflammatory markers (6:46).
Diagnostic Tests:
Pulmonary Function Tests (PFTs): These tests measure lung volumes (forced vital capacity, total lung capacity) and gas exchange (diffusing capacity), which are typically reduced in ILD (9:50).
High-Resolution CT (HRCT) Scan: HRCT is crucial for diagnosis, as it provides a much clearer view of lung abnormalities compared to a standard chest X-ray (10:30). Examples of ILD findings on HRCT include hazy appearances and dilated airways (12:03).
Six-Minute Walk Test: This test assesses functional status and helps determine the need for home oxygen (1:17:54).
Treatment of ILD in Scleroderma (14:24):
Immunosuppression:
Cyclophosphamide: Formerly first-line, it showed improvement in lung function but had severe side effects (15:38).
Mycophenolate: This drug has largely replaced cyclophosphamide as the first-line therapy due to similar positive effects with fewer side effects (17:36).
Rituximab: This IV medication also showed improvements in lung function and quality of life with fewer adverse events than cyclophosphamide, and it reduced the need for steroids (18:39).
Anti-fibrotic Therapies:
Nintedanib: Approved in 2019, Nintedanib slows the rate of lung function decline in SSc-ILD (19:34). While it slows decline, it often causes gut-related side effects like diarrhea (22:10). The video discusses exploratory data suggesting a potential benefit of combination therapy with mycophenolate and nintedanib (20:54).
Naredemalast: A newer anti-fibrotic that also slows disease progression in progressive pulmonary fibrosis (PPF), including autoimmune-related ILDs (26:09). It showed less diarrhea compared to Nintedanib (28:04).
General Approach to Treatment: The guideline from 2023 recommends immunosuppression (mycophenolate or rituximab) as first-line therapy, with the addition of anti-fibrotics like Nintedanib. Strong recommendation against steroids due to increased risk of renal crisis (28:43).
Co-morbidities Affecting the Lungs (29:37):
Gastrointestinal Issues (Reflux): Reflux is common in SSc and can aggravate ILD, so managing digestive tract issues is crucial (29:49). Supportive measures and anti-reflux therapies are discussed (30:41).
Obstructive Sleep Apnea (OSA): Reported in up to 70% of ILD patients, untreated OSA can worsen reflux symptoms (31:11). Screening for sleep apnea is recommended if symptoms are present (31:22).
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