idiopathic pulmonary hemosiderosis

特発性肺ヘモジデリン沈着症 IPH

WordNet

1. (of diseases) arising from an unknown cause; "idiopathic epilepsy"
2. abnormal deposit of hemosiderin; often a symptom of thalassemia or hemochromatosis (同)haemosiderosis

PrepTutorEJDIC

1. 特発性疾患の(病気の原因があいまいな,また不明な場合)
2. 肺の;肺を冒す

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/26 11:57:50」(JST)

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Idiopathic pulmonary hemosiderosis
Classification and external resources
ICD-9-CM	516.1
OMIM	178550
DiseasesDB	29717

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 0.24 and 1.23 cases per million people.

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. The first living patient was diagnosed by Jan Waldenström in 1944. 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)

Pathophysiology

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

• 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, and it is twice as common in females. 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.

Treatment

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

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.

References

External links

• Hemosiderosis at eMedicine
• About.com article
• Discovery.com article

UpToDate Contents

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• 1. 特発性肺ヘモジデローシス idiopathic pulmonary hemosiderosis
• 2. びまん性肺胞出血症候群 the diffuse alveolar hemorrhage syndromes
• 3. 成人におけるセリアック病の病因、疫学、および臨床症状 pathogenesis epidemiology and clinical manifestations of celiac disease in adults
• 4. 大量喀血：原因 massive hemoptysis causes
• 5. 肺移植後の非感染性合併症 noninfectious complications following lung transplantation

English Journal

• Unusual presentation: pulmonary hemosiderosis with celiac disease and retinitis pigmentosa in a child.

• Keskin O, Keskin M, Guler E, Tutar E, Saygili O, Kucukosmanoglu E, Kor Y, Celik H, Coskun E.SourcePediatric Allergy and Immunology Unit, Medical Faculty, Gaziantep University, Gaziantep, Turkey.
• Pediatric pulmonology.Pediatr Pulmonol.2011 Aug;46(8):820-3. doi: 10.1002/ppul.21420. Epub 2011 Feb 18.
• Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by anemia, hemoptysis and recurrent alveolar hemorrhage. The combination of IPH and celiac disease (CD) is extremely rare. We report a 9-year-old boy with Lane-Hamilton syndrome, co-occurrence of pulmonary hemosiderosis with CD
• PMID 21337729

• Lung vasculitis and alveolar hemorrhage: pathology.

• Fishbein GA, Fishbein MC.SourceDavid Geffen School of Medicine at UCLA, Los Angeles, California.
• Seminars in respiratory and critical care medicine.Semin Respir Crit Care Med.2011 Jun;32(3):254-63. doi: 10.1055/s-0031-1279823. Epub 2011 Jun 14.
• Pulmonary vasculitides are a diverse group of limited and systemic disorders associated with inflammation of pulmonary vessels and parenchyma. These diseases often have distinctive clinical, serological, and histopathological features-extrapulmonary sites of involvement, circulating autoantibodies,
• PMID 21674412

Japanese Journal

• 研究・症例 30年間少量の喀血を繰り返したのちに診断された成人発症の特発性肺ヘモジデローシスの1例

• 日本胸部臨床 75(7), 786-793, 2016-07
• NAID 40020896233

• 研究・症例 急速な経過を辿った特発性肺ヘモジデローシスの1例

• 日本胸部臨床 73(9), 1112-1117, 2014-09
• NAID 40020196143

• 進行性に肺胞構造の破壊をきたした特発性肺ヘモジデローシスの1成人例

• 日本呼吸器学会誌 = Annals of the Japanese Respiratory Society 2(4), 354-358, 2013-07-10
• NAID 10031183747

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★リンクテーブル★

リンク元	「特発性肺ヘモジデリン沈着症」「IPH」
関連記事	「idiopathic」「pulmonary」「hemosiderosis」「pulmonary hemosiderosis」

「特発性肺ヘモジデリン沈着症」

　　[★]

英

idiopathic pulmonary hemosiderosis IPH

同

特発性肺血鉄症、ツェーレン-ゲレルステト症候群 Ceelen-Gellerstedt syndrome

「IPH」

　　[★]

• 特発性門脈圧亢進症 idiopathic portal hypertension
• 特発性肺ヘモジデリン沈着症 idiopathic pulmonary hemosiderosis

「idiopathic」

　　[★]

• adj.

• 特発の、特発性の。原因不明の。ある個人に独特の

関

agnogenic、cryptogenic、idiopathy

　 　

「pulmonary」

　　[★]

• adj.

• 肺の、肺性の

関

lung、pneumo

　

「hemosiderosis」

　　[★] ヘモジデリン沈着症 　

「pulmonary hemosiderosis」

　　[★] 肺ヘモジデリン沈着症