Pneumothorax: Causes

Pathogenesis (development of disease)

Primary spontaneous pneumothorax is characterized by mostly apical (from Latin : apex “tip”: referring to the apex) subpleural bullae (blebs, blisters). The other lung tissue is healthy. Secondary spontaneous pneumothorax occurs mainly in pre-existing adhesions. In women, a special form of secondary pneumothorax is catamenial pneumothorax, which is often caused by subpleural endometriosis (focal occurrence of endometrium (uterine mucosa) outside its physiological location). Tension pneumothorax results from ever-increasing pressure in the pleural space (by a valvular mechanism). This results in restricted development of the reciprocal (healthy) lung with rapidly developing respiratory insufficiency/breathing weakness (severe dyspnea/breathlessness and cyanosis/cyanosis) and decreased blood return to the heart (hypotension and circulatory failure).

Etiology (Causes)

Biographic causes

  • Genetic burden
    • Genetic diseases
      • Birt-Hogg-Dubé syndrome (BHDS) – genetic disorder with autosomal dominant inheritance; germline mutations in the FLCN gene have been found in families with BHDS; clinical presentation: Skin lesions, renal tumors, and lung cysts, possibly associated with pneumothorax (lung collapse due to air in the pleural space (space between the ribs and lung pleura where there is physiological negative pressure)).
      • Cystic Fibrosis (ZF) – genetic disease with autosomal recessive inheritance, characterized by the production of secretions in various organs to be tamed.

Behavioral causes

  • Smoking – increases the risk in primary spontaneous pneumothorax.

Disease-related causes

Respiratory system (J00-J99)

Infectious and parasitic diseases (A00-B99).

  • Tuberculosis

Neoplasms – tumor diseases (C00-D48)

  • Histiocytosis/Langerhans-cell histiocytosis (abbreviation: LCH; formerly: histiocytosis X; Engl. histiocytosis X, langerhans-cell histiocytosis) – systemic disease with proliferation of Langerhans cells in various tissues (skeleton 80% of cases; skin 35%, pituitary gland (pituitary gland) 25%, lung and liver 15-20%); in rare cases neurodegenerative signs may also occur; in 5-50 % of cases, diabetes insipidus (hormone deficiency-related disturbance in hydrogen metabolism, leading to extremely high urine excretion) occurs when the pituitary gland is affected; the disease occurs disseminated (“distributed over the whole body or certain regions of the body”) frequently in children between 1-15 years of age, less frequently in adults, here predominantly with an isolated pulmonary affection (lung affection); prevalence (disease frequency) approx. 1-2 per 100,000 inhabitants
  • Lymphangioleiomyomatosis (LAM) – very rare disease of the lung, which is usually progressive (progressive), leads to chronic hypoxia (oxygen deficiency) and is ultimately life-threatening; affects almost exclusively women.
  • Neoplasms of the lung, unspecified.

Symptoms and abnormal clinical and laboratory findings not elsewhere classified (R00-R99)

  • Cough, severe or pressing → spontaneous pneumothorax.

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

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

  • Traumatic thoracic injuries (injuries to the chest) result in traumatic pneumothorax:
    • Rib fractures (most common).
    • Penetrating stab wounds
    • Blunt chest trauma

Other causes – the following medical actions can lead to iatrogenic pneumothorax:

  • Barotrauma (“pressure injury”) due to artificial ventilation.
  • Paravertebral nerve blocks – nerve blocks performed adjacent to the spine.
  • Puncture of the subclavian vein – puncture of the subclavian vein.
  • Transbronchial biopsy – sample collection through the bronchi.
  • Transthoracic fine needle aspiration – sample collection through the chest.