Printer Friendly

Use of Corticosteroids in the management of Idiopathic Pulmonary Haemosiderosis: Do we have enough evidence.

Byline: Ammara Mushtaq , Subika Khatoon and Muhammad Asif Qureshi

ABSTRACT

Idiopathic Pulmonary Haemosiderosis (IPH) is a rare disease commonly affecting the paediatric population with approximately 500 globally reported cases in the literature. The disease usually presentswith a symptom triad consisting of ferropenic anaemia, cough with haemoptysis and diffuse bilateral alveolar infiltrates. Therapeutic options for this disorder are not only limited but also not fully effective. Moreover, corticosteroids remain the mainstay of IPH treatment. This communication reviews the available evidence in support of corticosteriod usage in the treatment of IPH. We conclude that the use of corticosteroid in IPH treatment is unfathomed and demands further investigation.

KEY WORDS: Idiopathic Pulmonary Haemosiderosis, Corticosteroids.

Idiopathic Pulmonary Haemosiderosis (IPH), first described in 1851 by Virchow as 'brown lung in duration'' used to bean incidental finding at autopsy. It is a rare disease with an incidence of about 0.24 and 1.23 cases per million according to a Swedish and a Japanese review, respectively1 and approximately 500 cases have been reported in the literature.1 The disease occurs mainly in the paediatric age group,2 although a number of adult cases have been reported. The aetiology and pathogenesis of the disease is largely unknown to date. IPH manifests itself as a triad comprising of ferropenic anaemia, haemoptysis associated cough and diffuse bilateral alveolar infiltrates. The course of the disease is usually variable, intermittent and progressive, and is characterized by a range of signs/symptoms including attacks of chronic cough, dyspnoea, fatigue, pallor or cyanosis, weight loss, tachycardia, vomiting, haematemesis, acute right-sided heart failure and engorged neck veins.

Iron d ficiency anaemia, due to the recurrently occurring haemorrhages in pulmonary blood capillaries can, however, be the sole presentation of the disease.3 The diagnosis is completed/ assured by the identification of haemosiderin- laden macrophages in the lung alveoli, sputum, bronchoalveolar lavage, lung biopsy specimens and gastric washings.4 Various therapeutic modalities have been tried for IPH including the use of inhaled and systemic corticosteroids, administration of immunosuppressant drugs and blood transfusions. Nevertheless, the efficacy of these therapeutic options is largely un-explored. Although a considerable proportion of patients with IPH are prescribe with corticosteroids, the role/efficacy of corticosteroids in IPH remains unclear. Several studies have reported the use of steroids in high doses (e.g., prednisone 2-5 mg/kg/d or equivalent) at initial phase of IPH management.5,6

In between attacks, corticosteroid maintenance therapy has been used and a number of results validated. A retrospective review of 23 children in whom IPH was diagnosed and steroids in low dose have been tried after the initial high dose on presentation, showed milder form of illness, prolonged survival and reduced exacerbations.7 This suggests that patients with IPH show an excellent response to systemic steroids, at least during the haemorrhagic episodes. However, the efficacy of this treatment in the long term steroid therapy has not been explored. In their review, Soergel and Sommers8 concluded that despite its usefulness during the acute bleeding episodes, steroid therapy was not of much importance in long term treatment of the disease. Steroids, when given in combination with other immunosuppressive drugs prove to be beneficial as part of the IPH maintenance therapy.1

Also, the administration of inhaled corticosteroid, beclomethazone dipropionate, have shown better responses than maintenance therapy on systemic steroids in the case of two children reported by Ban Halima N. and co-workers in their study.9 According to a case report, corticosteroid therapy has also been effective in preventing the episodes of haemoptysis in a patient who was initially maintained on a 20mg dose of prednisolone that was then tapered to 10mg dose of the drug one month later.10 In a case of IPH from Karachi, Pakistan11 coritcosteroids proved to be effective in an 8 year old child who presented with severe iron deficiency anaemia. Another success of corticosteroid treatment was reported in a case from India where the patient showed remarkable recovery within 10 days of therapy.12

In contrast to the aforementioned studies, a study documented that the administration of corticosteroids to a 19-year old male worsened the respiratory function and he experiences IPH remission within 3 years of lung transplantation.13

Similarly, there are studies that make a cogent case that prolonged survival of the patients was dependent on long term immunosuppressant drugs along with steroids, and corticosteroids alone were not sufficient for the long term treatment of the disease14 and their side-effects in long term therapy cannot be ignored.8 Also, old age (compared to the typical age of 1-7 years) at diagnosis has been associated with poor response to the therapy,4 making corticosteroid administration questionable in such cases. Other investigational treatment options include immunosuppressive agents like azathioprine, hydroxycholoroquine, cyclophosphamide and methotrexate, with variable success rates.1

Four patients in a study were prescribed azathioprine when they didn't respond to prednisolone.4

In summary, this manuscript highlights the facts that the use of corticosteroids in IPH management is a heavily debated topic that needs further in- vestigation to clarify the existing conflicts. In our opinion, this issue can be resolved either by using alternate treatment options including immuno sup- pressive agents like azathioprine, hydroxycholo- roquine, cyclophosphamide, methotrexate as well as azathioprine, which has been used in patients who were non responsive to prednisolon1 or run- ning clinical trials to prove or disprove the efficacy of corticosteroid treatment. However, it should be noted that due to rarity of the disease, it has been and will be difficult to conduct prospective clini- cal trials.1 Therefore, much of the understanding on this considerably disputed issue will rely on case reports explaining the course of the disease and the therapeutic response. Subsequent meta-amylases of these case reports will further augment the under- standing of this debated issue.

Moreover, we em- phasiz upon active research in order to investigate the cause of this disorder so that selective, and di- rected, therapeutic approaches can be undertaken.

Financial support: None.

Competing interest: None declared.

REFERENCES

1. Loachimescu OC, Sieber S, Kotch A. Idiopahtic pulmonary hemosiderosis revisited. Eur Respir J. 2004;24(1):162-170. do i:10.1183/09031936.04.00116302

2. Pinto M, Correia J, Leal I, et al. Idipathic pulmonary hemosiderosis. Acta Med port. 1996;9:41-44.

3. Chen KC, Hsiao CC, Huang SC, Ko SF, Niu CK. Anemia as the sole presenting symptom of idiopathic pulmonary hemosiderosis: report of two cases. Chang Gung Med J. 2004;27:824-829.

4. Kabra SK, Bhargava S, Lodha R, Satyavani A, Walia M. Idiopathic Pulmonary Hemosiderosis: clinical profile and follow-up of 26 children. Indain Pediatr. 2007;44:333-338

5. Kjellman B, Elinder G, Garwicz S, Svan H. Idiopathic pulmonary hemosiderosis in Swedish children. Acta Pediatr Scand 1984;73:584-588. doi: 10.1111/j.1651-2227.1984. tb09978.x

6. Chryssanthopoulos C, Cassimos C, Panagiotidou C. Prognostic criteria in idiopathic pulmonary hemosiderosis in children. Eur J Pediatr. 1983;140:123-125. doi:10.1007/ BF00441658

7. Kiper N, Gocmen A, Ozcelik U, Dilber E, Anadol D. Long- term clinical course of patients with Idiopathic Pulmonary Hemosiderosis (1979-1994): prolonged survival with low dose corticosteroid therapy. Pediatr Pulmonol. 1999;27:180- 184. doi: 10.1002/(SICI)1099-0496(199903)27:3less than 180::AID- PPUL5greater than 3.0.CO;2-8

8. Soergel KH, Sommers SC. Idiopathic pulmonary hemosiderosis and related syndromes. Am J Med. 1962;32:499-511. doi:10.1016/0002-9343(62)90051-7

9. Halima BN, Karray A, Krichen A, Feki H, Ben Salah M, Chabchoub I, et al. Inhaled corticosteroids in idiopathic pulmonary hemosiderosis: 2 cases. Tunis Med. 2003;81(4):283-287.

10. Chen CH, Yang HB, Chiang SR, Wang PC. Idiopathic pulmonary hemosiderosis: Favourable response to corticosteroids. J Chinese Med Assoc. 2008;71(8):421-424. doi:10.1016/S1726-4901(08)70094-7

11. Afzal N, Mushtaq A, Rahman A, Qureshi S. Idiopathic Pulmonary Haemosiderosis presenting as severe iron deficiency anaemia- A case from Pakistan. J Pak Med Assoc. 2012;62(8):845-846.

12. Bhatia S, Tullu MS, Vaideeswar P, Lahiri KR. Idiopathic pulmonary hemosiderosis: alveoli are an answer to anemia. J Postgrad Med. 2011;57(1):57-60. doi: 10.4103/0022- 3859.74290

13. Fiorella C, Cinzia G, Federico R, Monica L, Francesco S, Guiseppe DV, et al. Recurrence of Idiopathic pulmonary hemosiderosis in a young adult patient after bilateral single lung transplantation. Transplantation. 2002;74:1643-1645.

14. Saeed MM, Woo MS, Maclaughlin EF, Margetis MF, Keens TG. Prognosis in paediatric idiopathic pulmonary hemosiderosis. Chest. 1999;116(3):721-725. doi:10.1378/ chest.116.3.721

Authors' Contributions:

AM: Conception of idea and manuscript drafting.

SK and MAQ: Manuscript drafting.
COPYRIGHT 2015 Asianet-Pakistan
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 2015 Gale, Cengage Learning. All rights reserved.

 
Article Details
Printer friendly Cite/link Email Feedback
Publication:Pakistan Journal of Medical Sciences
Date:Apr 30, 2015
Words:1425
Previous Article:Prevalence and associated factors of Carpal Tunnel Syndrome (CTS) among medical laboratory staff at King Saud University Hospitals, KSA.
Next Article:Ebola Virus: The need to take some preventive measures.
Topics:

Terms of use | Privacy policy | Copyright © 2018 Farlex, Inc. | Feedback | For webmasters