Medicine:Idiopathic pulmonary haemosiderosis

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Idiopathic pulmonary haemosiderosis
Other namesIdiopathic pulmonary hemosiderosis
SpecialtyRespirology

Idiopathic pulmonary haemosiderosis (IPH) is a lung disease of unknown cause that is characterized by alveolar capillary bleeding and accumulation of haemosiderin in the lungs. It is rare, with an incidence between 0.24[1] and 1.23[2] cases per million people.

Pathophysiology

Being idiopathic, IPH by definition has an unknown cause. It is thought to be an immune-mediated disease.[3][4] The lung bleeding causes accumulation of iron, which in itself causes additional lung damage. Meanwhile, there is insufficient iron for inclusion into the haemoglobin molecules inside red blood cells which carry oxygen to body tissues for cellular respiration.[citation needed]

Idiopathic pulmonary haemosiderosis can occur either as a primary lung disorder or as the sequela to other pulmonary, cardiovascular or immune system disorder.

  • PH1 involves PH with circulating anti-GBM antibodies.
  • PH2 involves PH with immune complex disease such as systemic lupus erythematosus, SLE.
  • PH3 involves no demonstrable immune system involvement.

A distinct subset of patients with pulmonary hemosiderosis has hypersensitivity to cow's milk which result in formation of IgG antibodies against basement membrane. This is called Heiner syndrome. Mechanism of haemorrhage is similar to that observed in Goodpasture syndrome.

Related or similar conditions

There are many pulmonary problems that may seem to mimic haemosiderosis but do not necessarily include the deposits of iron into the lung. The deposition of iron in the lungs, occurring in the form of haemosiderin, is the defining characteristic of this illness. These other conditions may occur separately or together with haemosiderosis.[citation needed]

  • Pulmonary fibrosis
  • Adult respiratory distress syndrome (ARDS)
  • Immune complex disease
  • intra-alveolar bleeding

Diagnosis

Clinically, IPH manifests as a triad of haemoptysis, diffuse parenchymal infiltrates on chest radiographs, and iron deficiency anaemia. It is diagnosed at an average age of 4.5 plus or minus 3.5 years,[5] and it is twice as common in females.[5] The clinical course of IPH is exceedingly variable, and most of the patients continue to have episodes of pulmonary haemorrhage despite therapy. Death may occur suddenly from acute pulmonary haemorrhage or after progressive pulmonary insufficiency resulting in chronic respiratory failure.[citation needed]

Treatment

Corticosteroids are the mainstay of treatment of IPH, though they are controversial and lack clear evidence in their favour.[6] They are thought to decrease the frequency of haemorrhage,[7][8][9] while other studies suggest that they do not have any effect on the course or prognosis of this disease.[3][7][10] In either case, steroid therapy has significant side effects. Small trials have investigated the use of other medications, but none has emerged as a clear standard of care. This includes immune modulators such as hydroxychloroquine,[11][12] azathioprine,[13][14] and cyclophosphamide.[15] 6-mercaptopurine as a long-term therapy may prevent pulmonary haemorrhage.[16] A 2007 scientific letter reports preliminary success in preventing pulmonary haemorrhage with the anti-oxidant N-acetylcysteine.[17]

Prognosis

Death may occur rapidly with acute, massive pulmonary bleeding or over longer periods as the result of continued pulmonary failure and right heart failure. Historically, patients had an average survival of 2.5 years after diagnosis, but today 86% may survive beyond five years.[5]

History

The condition was first described as "brown lung induration" by Rudolf Virchow in 1864 in patients after their death. Wilhelm Ceelen later correlated his findings to the clinical symptoms of two children who died of IPH in 1931.[18] The first living patient was diagnosed by Jan Waldenström in 1944.[19] It has been given several names, including:

  • Haemosiderin accumulation
  • Pulmonary haemosiderosis
  • Brown induration of lung
  • Essential brown induration of lung
  • Ceelen-Gellerstedt syndrome (after physicians Wilhelm Ceelen and Nils Gellerstedt)[20]

References

  1. ↑ "Idiopathic pulmonary haemosiderosis in Swedish children". Acta Paediatr Scand 73 (5): 584–8. September 1984. doi:10.1111/j.1651-2227.1984.tb09978.x. PMID 6485774. 
  2. ↑ Ohga, S; Takahashi, K; Miyazaki, S; Kato, H; Ueda, K (1995). "Idiopathic pulmonary haemosiderosis in Japan: 39 possible cases from a survey questionnaire". European Journal of Pediatrics 154 (12): 994–995. doi:10.1007/BF01958645. PMID 8801109. 
  3. ↑ 3.0 3.1 "Idiopathic pulmonary hemosiderosis and related syndromes". Am J Med 32 (4): 499–511. 1962. doi:10.1016/0002-9343(62)90051-7. 
  4. ↑ "Idiopathic pulmonary hemosiderosis: evidence of capillary basement membrane abnormality". Am Rev Respir Dis 114 (4): 689–698. 1976. doi:10.1164/arrd.1976.114.4.689. PMID 970745. 
  5. ↑ 5.0 5.1 5.2 Saeed, Muhammad M; Woo, Marlyn S; MacLaughlin, Eithne F; Margetis, Monique F; Keens, Thomas G (September 1999). "Prognosis in Pediatric Idiopathic Pulmonary Hemosiderosis". Chest 116 (3): 721–725. doi:10.1378/chest.116.3.721. PMID 10492278. http://chestjournal.chestpubs.org/content/116/3/721.full. 
  6. ↑ Sethi, GR; Singhal, KK (Oct 2008). "Pulmonary diseases and corticosteroids". Indian Journal of Pediatrics 75 (10): 1045–56. doi:10.1007/s12098-008-0209-0. PMID 19023529. 
  7. ↑ 7.0 7.1 Matsaniotis N; Karpouzas J; Apostolopoulou E et al. (1968). "Idiopathic pulmonary hemosiderosis in children". Arch Dis Child 43 (229): 307–309. doi:10.1136/adc.43.229.307. PMID 5652705. 
  8. ↑ "Familial idiopathic pulmonary hemosiderosis". Am. J. Dis. Child. 133 (6): 609–11. June 1979. doi:10.1001/archpedi.1979.02130060049010. PMID 375718. 
  9. ↑ "Severe idiopathic pulmonary hemosiderosis in the absence of clinical or radiologic evidence of pulmonary disease". J Pediatr 75 (1): 118–121. 1969. doi:10.1016/S0022-3476(69)80110-1. PMID 5790393. 
  10. ↑ Boat, TF (1998). "Idiopathic pulmonary hemosiderosis". Kendig's disorders of the respiratory tract in children. Philadelphia PA: WB Saunders. pp. 628–9. 
  11. ↑ "Chloroquine in idiopathic pulmonary hemosiderosis". Arch Dis Child 67 (5): 625–627. 1992. doi:10.1136/adc.67.5.625. PMID 1599302. 
  12. ↑ "Effectiveness of chloroquine therapy in idiopathic pulmonary hemosiderosis". Pediatric Pulmonology 20 (2): 125–126. 1995. doi:10.1002/ppul.1950200213. PMID 8570303. 
  13. ↑ "Immunosuppressive treatment of idiopathic pulmonary hemosiderosis". JAMA 226 (4): 458–9. 1973. doi:10.1001/jama.226.4.458. PMID 4800237. 
  14. ↑ "Long-term prednisone and azathioprine treatment of a patient with idiopathic pulmonary hemosiderosis". Pediatr Pulmonol 13 (3): 176–180. 1992. doi:10.1002/ppul.1950130310. PMID 1437333. 
  15. ↑ "Treatment of life-threatening primary pulmonary hemosiderosis with cyclophosphamide". Chest 102 (3): 959–960. 1992. doi:10.1378/chest.102.3.959. PMID 1516434. 
  16. ↑ "Maintenance therapy with dose-adjusted 6-mercaptopurine in idiopathic pulmonary hemosiderosis". Pediatric Pulmonology 43 (11): 1067–71. Nov 2008. doi:10.1002/ppul.20894. PMID 18972408. https://zenodo.org/record/3447437. Retrieved 2019-12-03. 
  17. ↑ "Idiopathic Pulmonary Haemosiderosis - World 1st Medical Treatment By Researchers At Queen Mary University London And University Of Leicester". Medical New Today. 2 June 2007. http://www.medicalnewstoday.com/articles/72840.php. 
  18. ↑ Ceelen, W. (1931). "Die Kreislaufstörungen der Lungen" (in de). Handbuch der speziellen pathologischen Anatomie und Histologie. 3. Berlin: Springer. p. 10. 
  19. ↑ Heiner, DC (1990). "Pulmonary hemosiderosis". Disorders of the respiratory tract in children. Philadelphia PA: WB Saunders. pp. 498–509. 
  20. ↑ Ceelen-Gellerstedt syndrome at Who Named It?

External links

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