Pulmonary Fibrosis: Or something else? Differential Diagnosis

Congenital malformations, deformities, and chromosomal abnormalities (Q00-Q99).

  • Hermansky-Pudlak syndrome – genetic disorder with autosomal recessive inheritance associated with albinism, photophobia, and increased bleeding tendency; usually also pulmonary fibrosis and increased bleeding tendency.
  • Neurofibromatosis – genetic disease with autosomal dominant inheritance; belongs to the phakomatoses (diseases of the skin and nervous system); three genetically distinct forms are distinguished:
    • Neurofibromatosis type 1 (von Recklinghausen’s disease) – patients form multiple neurofibromas (nerve tumors) during puberty, often occurring in the skin but also occurring in the nervous system, orbita (eye socket), gastrointestinal tract (gastrointestinal tract), and retroperitoneum (space located behind the peritoneum on the back toward the spine)
    • [Neurofibromatosis type 2 – characterized by bilateral (bilateral) acoustic neuroma (vestibular schwannoma) and multiple meningiomas (meningeal tumors).
    • Schwannomatosis – hereditary tumor syndrome]
  • Tuberous sclerosis (Bourneville-Pringle disease) – genetic disorder with autosomal dominant inheritance associated with malformations and tumors of the brain, skin lesions, and mostly benign (benign) tumors in other organ systems.

Respiratory system (J00-J99)

  • Acute respiratory distress syndrome (ARDS)
  • Aspiration pneumonia – pneumonia caused by inhalation of foreign material (often stomach contents)
  • Byssinosis (cotton fever) – lung disease caused by inhalation of cotton dust.
  • Eosinophilic pneumonia – pneumonia associated with an eosinophilic pulmonary infiltrate.
  • Exogenous allergic alveolitis (EAA) – inflammation of the alveoli (air sacs) triggered by an allergic reaction.
  • Fibrosing alveolitis – chronic inflammatory lung diseases that predominantly affect the interstitium and have varying degrees of alveolar cell proliferation; occasionally, this is accompanied by damage in the area of the bronchioles
  • Hamman-Rich syndrome (acute interstitial pneumonia, AIP) – is a usually lethal pneumonia (pneumonia).
  • Idiopathic interstitial pneumonia (IIP) – pneumonia, the cause is unclear.
  • Coal dust pneumonia – pneumonia caused by inhalation of coal dust.
  • Pneumoconioses (dust lung diseases) – asbestosis, silicosis.
  • Pulmonary hemorrhagic syndromes such as anti-GBM (glomerular basement membrane) disease (synonym: Goodpasture’s syndrome), idiopathic pulmonary siderosis.
  • Radiation pneumonitis (synonym: radiation pneumonia) – pneumonia secondary to radiation; interstitial lung disease.
  • Talc pneumonia – pneumonia caused by inhalation of talc.

Blood, blood-forming organs – immune system (D50-D90).

  • Langerhans cell granulomatosis – systemic disease associated with proliferation of dendritic cells.
  • Sarcoidosis – inflammatory systemic disease affecting mainly the skin, lungs and lymph nodes.

Endocrine, nutritional and metabolic diseases (E00-E90).

  • Amyloidosis – extracellular (“outside the cell”) deposits of amyloids (degradation-resistant proteins) that can lead to cardiomyopathy (heart muscle disease), neuropathy (peripheral nervous system disease), and hepatomegaly (liver enlargement), among other conditions.
  • Niemann-Pick disease (synonyms: Niemann-Pick disease, Niemann-Pick syndrome or sphingomyelin lipidosis) – genetic disease with autosomal recessive inheritance; belongs to the group of sphingolipidoses, which in turn are classified as lysosomal storage diseases; main symptoms of Niemann-Pick disease type A are hepatosplenomegaly (liver and spleen enlargement) and psychomotor decline; in type B, no cerebral symptoms are observed.
  • Storage diseases, unspecified

Cardiovascular system (I00-I99)

  • Left ventricular failure (left-sided heart failure) with chronic congestive lung disease

Infectious and parasitic diseases (A00-B99).

  • Infections, unspecified (e.g., infectious interstitial pneumonia (e.g., cytomegalovirus pneumonia, HIV-associated alveolitis, pneumocitis jirovecii pneumonia, and others).

Liver, gallbladder, and biliary tract-pancreas (pancreas) (K70-K77; K80-K87).

  • Chronic active hepatitis (liver inflammation).
  • Primary biliary cholangitis (PBC, synonyms: non-purulent destructive cholangitis; formerly primary biliary cirrhosis) – relatively rare autoimmune disease of the liver (affects women in about 90% of cases); begins primarily biliary, i.e., at the intra- and extrahepatic (“inside and outside the liver”) bile ducts, which are destroyed by inflammation (= chronic non-purulent destructive cholangitis). In the longer course, the inflammation spreads to the entire liver tissue and eventually leads to scarring and even cirrhosis; detection of antimitochondrial antibodies (AMA); PBC is frequently associated with autoimmune diseases (autoimmune thyroiditis, polymyositis, systemic lupus erythematosus (SLE), progressive systemic sclerosis, rheumatoid arthritis); associated with ulcerative colitis (inflammatory bowel disease) in 80% of cases; long-term risk of cholangiocellular carcinoma (bile duct carcinoma, bile duct cancer) is 7-15%.

Mouth, esophagus (food pipe), stomach, and intestine (K00-K67; K90-K93).

Musculoskeletal system and connective tissue (M00-M99).

  • Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome (CSS) – granulomatous (roughly: “granule-forming”) inflammation of small to medium-sized blood vessels in which the affected tissue is infiltrated (“walked through”) by eosinophilic granulocytes (inflammatory cells).
  • Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis – necrotizing (tissue dying) vasculitis (vascular inflammation) of the small to medium-sized vessels (small-vessel vasculitides), which is accompanied by granuloma formation (nodule formation) in the upper respiratory tract (nose, sinuses, middle ear, oropharynx) as well as the lower respiratory tract (lungs)
  • Collagenoses (group of connective tissue diseases caused by autoimmune processes) – systemic lupus erythematosus (SLE), polymyositis (PM) or dermatomyositis (DM), Sjögren’s syndrome (Sj), scleroderma (SSc) and Sharp syndrome (“mixed connective tissue disease”, MCTD).
  • Mixed connective tissue disease (Sharp syndrome) – chronic inflammatory connective tissue disease that includes symptoms of multiple collagenoses.
  • Rheumatoid arthritis
  • Vasculitis (inflammation of blood vessels)

Neoplasms – tumor diseases (C00-D48)

  • Lymphangioleiomyomatosis (LAM) – very rare disease of the lung that is usually progressive, leads to chronic hypoxia (oxygen deprivation), and is ultimately life-threatening; affects almost exclusively women

Genitourinary system (kidneys, urinary tract – sex organs) (N00-N99).

Injuries, poisoning, and other sequelae of external causes (S00-T98).

Drugs

  • See “Causes” under medications

Environmental pollution – intoxications (poisoning).

  • Herbicides (weed killers) such as paraquat.
  • Inhalation of noxious agents such as tobacco smoke, gases, vapors, aerosols, hairspray, wood dusts, metal dusts (workers in metal smelters), stone dusts (siliceous silica/workers in quarries as well as sandblasters; fibrous silicate minerals: asbestos), and plant and animal particles.