History of Infiltrative Lung Diseases:

Disease Category & Link Progress, Diagnosis, Treatment, & Notable People
Pulmonary Alveolar Proteinosis (PAP)

Wikipedia: Pulmonary Alveolar Proteinosis

 Progress: First described in 1958 by Rosen, Castleman, & Liebow, who found lungs filled with a lipid/protein material. The major breakthrough was discovering that 90% of cases are autoimmune: the body creates antibodies against GM-CSF, a protein needed to clear old surfactant.

Diagnosis & Imaging:

  • X-ray: Historically showed bilateral “bat-wing” infiltrates, mimicking pulmonary edema.
  • HRCT (High-Resolution CT): This revolutionized the diagnosis. It shows the highly specific “Crazy-Paving” pattern: a combination of ground-glass opacities (from the fluid-filled alveoli) with superimposed thickened septal lines (inflammation).
  • Diagnosis: Confirmed by bronchoalveolar lavage (BAL) showing “milky” fluid.

Treatment: The gold standard for decades has been Whole Lung Lavage (WLL), a procedure to physically wash out the patient’s lungs one at a time. Modern therapy also includes inhaling or injecting the missing GM-CSF.
Pulmonary Amyloidosis

Wikipedia: Amyloidosis

 Progress: A rare disorder where an abnormal, insoluble protein (amyloid) is deposited in the lungs. It can be localized to the lungs or part of a systemic (body-wide) disease (like Multiple Myeloma).

Diagnosis & Imaging:

  • Diagnosis: The only definitive diagnosis is a biopsy stained with Congo Red, which shows a classic “apple-green birefringence” under polarized light.
  • Imaging (CT): It is a great mimic. The “infiltration” can appear in three main ways:
    1. Tracheobronchial: Thickening of the airway walls, sometimes with calcification.
    2. Nodular: One or more slow-growing nodules, often mistaken for cancer.
    3. Diffuse Alveolar-Septal: Diffuse, net-like infiltrates, often with calcification, mimicking ILD.

Treatment: Very difficult. If localized, nodules can be resected. If systemic, treatment involves chemotherapy to stop the production of the abnormal protein.
Pulmonary Langerhans Cell Histiocytosis (PLCH)

Wikipedia: PLCH

 Progress: A rare infiltrative disease caused by the buildup of abnormal immune cells (Langerhans cells). It is almost exclusively a disease of smokers and was previously called “Histiocytosis X” or “Eosinophilic Granuloma.”

Diagnosis & Imaging:

  • Imaging (HRCT): The diagnosis is often made by HRCT alone due to its unique appearance. The disease evolves from small, star-shaped nodules (early) into bizarre, “cookie-cutter,” irregular, thin-walled cysts (late).
  • Key Feature: The disease is classically upper-lobe predominant and spares the lung bases (the opposite of IPF).

Treatment: The single most important treatment is absolute smoking cessation, which can halt or even reverse the disease in its early (nodular) stages.
Eosinophilic Pneumonia (Acute & Chronic)

Wikipedia: Eosinophilic Pneumonia

 Progress: An infiltrative disease where large numbers of eosinophils (a type of white blood cell) flood the alveoli. It can be acute (often linked to new smoking) or chronic (defined by Dr. P. Carrington in 1969).

Diagnosis & Imaging:

  • Diagnosis: Confirmed by a high eosinophil count in a bronchoalveolar lavage (BAL) fluid sample.
  • Imaging (X-ray/CT): The chronic form (CEP) has a classic appearance: peripheral, upper-lobe infiltrates. On X-ray, this is famously called the “photographic negative of pulmonary edema,” as edema is central (bat-wing) while CEP is “stuck” to the outer edge of the lung.

Treatment: Responds dramatically and rapidly to corticosteroids.

1. Notables Who Advanced Diagnosis & Management of Infiltrative Lung Disease

Name & Wikipedia Link Comment on Contribution
Rudolf Virchow (1821–1902) A “father of modern pathology.” In 1854, he was the first to coin the term “amyloid” (meaning “starch-like”) for the waxy, abnormal protein deposits he observed in organs.
Rosen, Castleman, & Liebow This team of pathologists at Massachusetts General Hospital (MGH) first described Pulmonary Alveolar Proteinosis (PAP) as a distinct disease entity in a landmark 1958 paper, noting the alveoli were filled with a protein-lipid material.
Jose Ramirez-Rivera (1926–2006) A pulmonary physician who pioneered Whole Lung Lavage (WLL) in the 1960s as the first effective treatment for PAP. This technique of “washing” the protein out of the lungs remains the gold standard of care today.
P.R. Carrington A physician-researcher who, in 1969, published the first major case series on Chronic Eosinophilic Pneumonia, defining it as a specific, idiopathic disease and noting its classic (and bizarre) “photographic negative of pulmonary edema” appearance on X-ray.
Louis Lichtenstein (1906–1977) A pathologist who, in 1953, first classified the “Histiocytosis X” group of diseases, which includes what we now call Pulmonary Langerhans Cell Histiocytosis (PLCH).

 

2. Notables Who Suffered From Infiltrative Lung Disease (Amyloidosis)

Name & Wikipedia Link Comment on Disease
Robert Jordan (1948–2007) The author of the best-selling Wheel of Time fantasy series. He was diagnosed with cardiac amyloidosis (a systemic form) and was very public about his illness, receiving treatment at the Mayo Clinic.
Colin Powell (1937–2021) The U.S. General and Secretary of State suffered from multiple myeloma, a blood cancer. This cancer is the primary cause of AL (light-chain) amyloidosis, which was a significant comorbidity at the time of his death.
Tom Menino (1942–2014) The longest-serving mayor of Boston. He was diagnosed with AA amyloidosis, a systemic form of the disease that was a complication of his long-standing Crohn’s disease.
King George V (1865–1936) The King of the United Kingdom. His death was caused by a chronic suppurative lung disease (bronchiectasis) and heart failure. However, a post-mortem review of his case strongly suggested he also suffered from systemic amyloidosis as a complication of his chronic infections.

 

Related Links

Google ImagesGoogleYouTubeWikipediaRadiopediaRadiographicsChatGPTOpenEvidence