History of Lung Diseases: Inflammation & Immune

Disease Category & Link Progress, Diagnosis, Treatment, & Notable People
Acute Respiratory Distress Syndrome (ARDS)

Wikipedia: ARDS

Progress: This is an acute, severe inflammatory reaction of the entire lung, not a specific disease. It was first described in 1967 by Dr. David Ashbaugh and Dr. Thomas Petty. They identified 12 patients with severe, unexplained breathing failure (hypoxemia), stiff lungs, and fluid-filled lungs.

Diagnosis & Imaging: Imaging is central to the diagnosis.

X-ray: The 1967 paper relied on chest X-rays, which showed diffuse, bilateral, “fluffy” infiltrates that looked like pulmonary edema (fluid) but without the enlarged heart seen in heart failure. This “whiteout” lung became the classic sign.

CT Scan: The major leap in understanding came with CT in the 1980s. Dr. Luciano Gattinoni showed that the ARDS lung is not uniformly sick. He described the “baby lung” concept—a small, relatively healthy (aerated) portion of the lung, with the majority of the lung being collapsed and fluid-filled (dependent). This discovery, made possible by CT, revolutionized mechanical ventilation, leading to the low tidal volume strategy (ARDSNet) that protects the “baby lung” and saves lives.

Treatment: Supportive care with mechanical ventilation, treating the underlying cause, and prone-positioning (lying the patient on their stomach).

Pneumoconiosis (Silicosis, CWP)

Wikipedia: Pneumoconiosis

Progress: An occupational ILD caused by inhaling inorganic dust (silica, coal). Pliny the Elder (1st cent. AD) and Bernardino Ramazzini (1700) described diseases of miners. The link became undeniable during the Industrial Revolution.

Diagnosis & Imaging: Diagnosis is based on exposure history and imaging.

  • X-ray: This was the cornerstone of 20th-century occupational medicine. The ILO Classification (International Labour Organization) was created to standardize X-ray readings.
    • Silicosis: Classically shows small, rounded nodules, especially in the upper lung zones. “Eggshell calcification” of the hilar lymph nodes is a very specific sign.
    • Progressive Massive Fibrosis (PMF): In advanced cases, X-ray shows the small nodules merging into large, dense masses (fibrotic scars), typically in the upper lobes.
  • CT Scan: More sensitive for detecting early nodules and the large masses of PMF.

Treatment: None exists. Management is purely preventative (dust control).

Asbestosis (and Pleural Disease)

Wikipedia: Asbestosis

Progress: An ILD caused by inhaling asbestos fibers. The link was established in the 1920s (Nellie Kershaw case). Dr. Irving Selikoff‘s work in the 1960s on insulation workers proved the definitive link between asbestos, asbestosis, lung cancer, and mesothelioma.

Diagnosis & Imaging: Imaging is key to distinguishing it from other lung diseases.

  • X-ray: Classically shows linear, net-like (reticular) patterns, primarily in the lower lung zones (the opposite of silicosis). The most specific sign of exposure (not asbestosis itself) is pleural plaques—thick, often calcified, patches on the diaphragm or chest wall.
  • High-Resolution CT (HRCT): This is the gold standard. HRCT can detect the earliest signs of lung fibrosis (reticulation, traction bronchiectasis, and honeycombing) and is far more sensitive for seeing the pleural plaques. The pattern of fibrosis (known as UIP) is often indistinguishable from idiopathic pulmonary fibrosis.

Treatment: None. Supportive care and oxygen.

Idiopathic Pulmonary Fibrosis (IPF) / ILD

PubMed Review: History of IPF

Progress: A devastating chronic, progressive lung-scarring disease of unknown cause. First described as a specific entity (“Hamman-Rich Syndrome”) in 1935 by Louis Hamman and Arnold Rich. The term ILD (Interstitial Lung Disease) now covers over 200 related conditions, including those caused by autoimmune diseases (like Rheumatoid Arthritis, Scleroderma).

Diagnosis & Imaging: This is a field defined by imaging.

  • X-ray: Shows non-specific reticular patterns, mainly at the lung bases.
  • High-Resolution CT (HRCT): This technology revolutionized ILD diagnosis in the 1990s. Radiologists can now identify specific patterns on CT that predict the disease’s behavior. The classic “Definite UIP Pattern” (Usual Interstitial Pneumonia) is so specific for IPF that a surgical lung biopsy is often not needed. This pattern is defined by:
    1. Subpleural, basal-predominant disease.
    2. Reticulation (net-like scarring).
    3. Traction Bronchiectasis (airways pulled open by scars).
    4. Honeycombing (stacked, cystic airspaces)—the hallmark of end-stage fibrosis.

Treatment: For decades, there was no treatment. The 2010s saw the first-ever effective drugs (Pirfenidone, Nintedanib), which are anti-fibrotics that slow the scarring process.

Sarcoidosis

Wikipedia: Sarcoidosis

Progress: An immune-mediated disease where the body forms tiny clusters of inflammatory cells (granulomas) in various organs, most commonly the lungs and lymph nodes. Caesar Boeck (1899) described the skin lesions. Its nature as a systemic lung disease was understood by the mid-20th century.

Diagnosis & Imaging: The diagnosis is almost entirely driven by imaging.

  • X-ray: The Siltzbach Staging System (1967) is based entirely on the chest X-ray:
    • Stage 1: Bilateral hilar lymphadenopathy (enlarged “potato nodes”) only.
    • Stage 2: Hilar nodes + lung infiltrates (nodules).
    • Stage 3: Lung infiltrates only.
    • Stage 4: Fibrosis (permanent scarring).
  • CT Scan: More sensitive. It shows the characteristic perilymphatic nodules (tiny nodules tracking along the lung’s airways, vessels, and pleura) and the enlarged nodes.

Treatment: Often resolves on its own. For symptomatic disease, the mainstay has been corticosteroids (like Prednisone) for decades.

1. Notables Who Advanced Diagnosis & Management of Inflammation & Immune Diseases of the Lung

Name & Wikipedia Link Comment on Contribution
David Ashbaugh & Thomas Petty In 1967, these two physicians co-authored the first description of what we now call ARDS (Acute Respiratory Distress Syndrome). They identified 12 patients with severe, unexplained respiratory failure, establishing its key diagnostic criteria.
Luciano Gattinoni An Italian intensivist whose CT scan research in the 1980s revolutionized ARDS management. He developed the “baby lung” concept, showing that only a small part of the lung is functional, which led directly to the life-saving low tidal volume ventilation strategy.
Irving J. Selikoff (1914–1992) A pioneer in occupational medicine. His research on insulation workers in the 1960s provided the definitive, undeniable link between asbestos exposure and the development of asbestosis, lung cancer, and mesothelioma.
Bernardino Ramazzini (1633–1714) Considered the “father of occupational medicine.” In his 1700 book De Morbis Artificum Diatriba (Diseases of Workers), he described the lung diseases of miners, linking their “scanty breathing” to the inhalation of dust (pneumoconiosis).
Louis Hamman & Arnold Rich (1877–1946) & (1893–1968) In 1935, these two Johns Hopkins physicians described “Hamman-Rich Syndrome,” a rapidly fatal, acute interstitial pneumonia. This was the first description of what we now classify as AIP and helped define the category of IPF/ILD.

2. Notables Who Suffered From Pulmonary Inflammation & Immune Disease

Name & Wikipedia Link Comment on Disease
Steve McQueen (1930–1980) The iconic “King of Cool” actor died from malignant mesothelioma. He believed his cancer was caused by exposure to asbestos in the U.S. Marines (removing lagging from pipes) and from his racing suit insulation.
Bernie Mac (1957–2008) The comedian and actor suffered from sarcoidosis, an inflammatory granulomatous disease that primarily affected his lungs. His death was ultimately caused by complications from pneumonia, but his long-standing, immunosuppressed sarcoidosis was a critical factor.
Evel Knievel (1938–2007) The legendary stunt performer suffered from idiopathic pulmonary fibrosis (IPF) for many years, a disease he believed was caused by his numerous high-impact crashes. He required continuous oxygen therapy and received a lung transplant in 1999.
Marlon Brando (1924–2004) The Academy Award-winning actor died from respiratory failure due to pulmonary fibrosis. He also suffered from congestive heart failure, and the lung disease was a major contributor to his death.
Peter Benchley (1940–2006) The author of the novel Jaws died from pulmonary fibrosis. He became a dedicated ocean conservationist later in life, ironically working to protect the sharks he had once demonized.
Bernie Banton (1946–2007) An Australian social justice advocate who became the public face of the legal battle against asbestos manufacturer James Hardie. He suffered from and died of mesothelioma, having previously been diagnosed with asbestosis.