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Idiopathic pulmonary haemosiderosis

idiopathic pulmonary hemosiderosis, idiopathic pulmonary haemosiderosis
Idiopathic pulmonary haemosiderosis or idiopathic pulmonary hemosiderosis; 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 0241 and 1232 cases per million people


  • 1 History
  • 2 Pathophysiology
    • 21 Related or similar conditions
  • 3 Diagnosis
  • 4 Treatment
  • 5 Prognosis
  • 6 References
  • 7 External links


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 19313 The first living patient was diagnosed by Jan Waldenström in 19444 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 Gellerstedt5


Being idiopathic, IPH by definition has an unknown cause It is thought to be an immune-mediated disease67 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

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 as in Goodpasture syndrome

Related or similar conditionsedit

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

  • Pulmonary Fibrosis
  • Adult Respiratory Distress Syndrome ARDS
  • Immune Complex Disease
  • intra-alveolar bleeding


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 45 plus or minus 35 years,8 and it is twice as common in females8 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


Corticosteroids are the mainstay of treatment of IPH, though they are controversial and lack clear evidence in their favour9 They are thought to decrease the frequency of haemorrhage,101112 while other studies suggest that they do not have any effect on the course or prognosis of this disease61013 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,1415 azathioprine,1617 and cyclophosphamide18 6-mercaptopurine as a long-term therapy may prevent pulmonary haemorrhage19 A 2007 scientific letter reports preliminary success in preventing pulmonary haemorrhage with the anti-oxidant N-acetylcysteine20


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 25 years after diagnosis, but today 86% may survive beyond five years8


  1. ^ Kjellman B, Elinder G, Garwicz S, Svan H September 1984 "Idiopathic pulmonary haemosiderosis in Swedish children" Acta Paediatr Scand 73 5: 584–8 PMID 6485774 doi:101111/j1651-22271984tb09978x 
  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 letter|format= requires |url= help 154 12: 994–995 PMID 8801109 doi:101007/BF01958645 
  3. ^ Ceelen, W 1931 "Die Kreislaufstörungen der Lungen" In Henke, F; Lubarsch, O Handbuch der speziellen pathologischen Anatomie und Histologie in German 3 Berlin: Springer p 10 
  4. ^ Heiner, DC 1990 "Pulmonary hemosiderosis" In Chernick V, Kendig EL Jr Disorders of the respiratory tract in children Philadelphia PA: WB Saunders pp 498–509 
  5. ^ Ceelen-Gellerstedt syndrome at Who Named It
  6. ^ a b Soergel KH, Sommers SC 1962 "Idiopathic pulmonary hemosiderosis and related syndromes" Am J Med 32: 499–511 doi:101016/0002-93436290051-7 
  7. ^ Gonzalez-Crussi F, Hull MT, Grosefeld JL 1976 "Idiopathic pulmonary hemosiderosis: evidence of capillary basement membrane abnormality" Am Rev Respir Dis 114 4: 689–698 PMID 970745 
  8. ^ a b c 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 PMID 10492278 doi:101378/chest1163721 
  9. ^ Sethi, GR; Singhal, KK Oct 2008 "Pulmonary diseases and corticosteroids" Indian Journal of Pediatrics 75 10: 1045–56 PMID 19023529 doi:101007/s12098-008-0209-0 
  10. ^ a b Matsaniotis N, Karpouzas J, Apostolopoulou E, et al 1968 "Idiopathic pulmonary hemosiderosis in children" Arch Dis Child 43 229: 307–309 PMC 2019947  PMID 5652705 doi:101136/adc43229307 
  11. ^ Beckerman RC, Taussig LM, Pinnas JL June 1979 "Familial idiopathic pulmonary hemosiderosis" Am J Dis Child 133 6: 609–11 PMID 375718 doi:101001/archpedi197902130060049010 
  12. ^ Gilman PA, Zinkham WH 1969 "Severe idiopathic pulmonary hemosiderosis in the absence of clinical or radiologic evidence of pulmonary disease" J Pediatr 75 1: 118–121 PMID 5790393 doi:101016/S0022-34766980110-1 
  13. ^ Boat, TF 1998 "Idiopathic pulmonary hemosiderosis" In Chernick V, Boat T Kendig’s disorders of the respiratory tract in children Philadelphia PA: WB Saunders pp 628–9 
  14. ^ Bush A, Sheppard MN, Warner JO 1992 "Chloroquine in idiopathic pulmonary hemosiderosis" Arch Dis Child 67 5: 625–627 PMC 1793713  PMID 1599302 doi:101136/adc675625 
  15. ^ Zaki M, Al Saleh Q, Al Mutari G 1995 "Effectiveness of chloroquine therapy in idiopathic pulmonary hemosiderosis" Pediatric Pulmonology 20: 1206 doi:101002/ppul1950200213 
  16. ^ Byrd RB, Gracey DR 1973 "Immunosuppressive treatment of idiopathic pulmonary hemosiderosis" JAMA 226 4: 458–9 PMID 4800237 doi:101001/jama2264458 
  17. ^ Rossi GA, Balzano E, Battistini E 1992 "Long-term prednisone and azathioprine treatment of a patient with idiopathic pulmonary hemosiderosis" Pediatr Pulmonol 13 3: 176–180 PMID 1437333 doi:101002/ppul1950130310 
  18. ^ Colombo JR, Stolz SM 1992 "Treatment of life-threatening primary pulmonary hemosiderosis with cyclophosphamide" Chest 102 3: 959–960 PMID 1516434 doi:101378/chest1023959 
  19. ^ Luo XQ, Ke ZY, Huang LB, Guan XQ, Zhang XL, Zhu J, Zhang YC Nov 2008 "Maintenance therapy with dose-adjusted 6-mercaptopurine in idiopathic pulmonary hemosiderosis" Pediatric Pulmonology 43 11: 1067–71 PMID 18972408 doi:101002/ppul20894 
  20. ^ "Idiopathic Pulmonary Haemosiderosis - World 1st Medical Treatment By Researchers At Queen Mary University London And University Of Leicester" Medical New Today 2 June 2007 

External linksedit

  • Hemosiderosis at eMedicine
  • Aboutcom article
  • Discoverycom article

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