Further Outpatient Care
- Periodic evaluation of growth, oxygen saturation, pulmonary and renal function, and hemography and chest radiography findings is appropriate in patients with pulmonary hemosiderosis.
- Long-term steroid use requires frequent monitoring for possible adverse effects.
Deterrence/Prevention
- Patients with Heiner syndrome should avoid milk and dairy products.
Complications
- Respiratory failure requiring mechanical ventilatory support may occur after an episode of acute pulmonary hemorrhage.
- Chronic cor pulmonale with pulmonary hypertension secondary to pulmonary fibrosis has been described in a minority of patients with idiopathic pulmonary hemosiderosis (IPH) who have lived for an exceptionally long time after the onset of their disease.
Prognosis
- The prognosis for the pulmonary hemosiderosis syndromes as a group is difficult to determine because of the infrequency of the diagnosis and the variability among cases and etiologies. Furthermore, no national database monitors children with PH.
- When focusing on idiopathic pulmonary hemosiderosis, the clinical course widely varies; however, the prognosis has always been regarded as poor, with a mean survival of 2.5-3 years after diagnosis. Death can occur acutely from massive hemorrhage or after progressive pulmonary insufficiency and right heart failure. The available therapeutic modalities are not associated with a better outcome.
- One study of 30 children with idiopathic pulmonary hemosiderosis listed the following prognostic criteria:
- The severity of the disease at its onset does not correlate with the survival.
- Females survive longer than males.
- Young age at the onset of disease seems to carry a less favorable prognosis.
- Common therapeutic modalities have not improved outcome.
- Another retrospective study of 15 children with idiopathic pulmonary hemosiderosis found that the presence of antineutrophil cytoplasmic antibodies (ANCA) or other autoantibodies signal poor prognosis. The same study, in which the mean duration of follow-up was 17.2 years (range, 10-36 y), reported a survival rate of 80%
- In pulmonary hemosiderosis associated with milk protein allergy, avoidance of dairy products is usually associated with complete remission.[5]
Gordon IO, Cipriani N, Arif Q, Mackinnon AC, Husain AN. Update in nonneoplastic lung diseases. Arch Pathol Lab Med. Jul 2009;133(7):1096-105. [Medline].
Sethi GR, Singhal KK, Puri AS, Mantan M. Benefit of gluten-free diet in idiopathic pulmonary hemosiderosis in association with celiac disease. Pediatr Pulmonol. Oct 21 2010;[Medline].
Kahraman H, Köksal N, Ozkan F. Eight Years Follow-up of a Case with Idiopathic Pulmonary Hemosiderosis After Corticosteroid Therapy. N Am J Med Sci. Jan 2012;4(1):49-51. [Medline]. [Full Text].
Bhatia S, Tullu MS, Vaideeswar P, Lahiri KR. Idiopathic pulmonary hemosiderosis: alveoli are an answer to anemia. J Postgrad Med. Jan-Mar 2011;57(1):57-60. [Medline].
[Guideline] American College of Allergy, Asthma, & Immunology. Food allergy: a practice parameter. Ann Allergy Asthma Immunol. Mar 2006;96(3 Suppl 2):S1-68. [Medline]. [Full Text].
Akyar S, Ozbek SS. Computed tomography findings in idiopathic pulmonary hemosiderosis. Respiration. 1993;60(1):63-4. [Medline].
CDC. Investigation of acute idiopathic pulmonary hemorrhage among infants - Massachusetts, December 2002-June 2003. MMWR Morb Mortal Wkly Rep. Sep 10 2004;53(35):817-20. [Medline].
Dearborn DG. Pulmonary hemorrhage in infants and children. Curr Opin Pediatr. Jun 1997;9(3):219-24. [Medline].
Hoca, Nevin Taci. Dayioglu, Didem. Ogretensoy, Mihriban. Pulmonary hemosiderosis in association with celia disease. Lung. Sep-Oct 2006;184(5):297-300. [Medline].
Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. Jul 2004;24(1):162-70. [Medline].
Katz SM, Foster E, Miller AS, Krevolin L, Schwartz AB. Goodpasture's syndrome mimicking idiopathic pulmonary hemosiderosis. Ann Clin Lab Sci. Jul-Aug 1989;19(4):280-6. [Medline].
Kayser K, Plodziszewska M, Waitr E, et al. Diffuse pulmonary hemosiderosis after exposure to pesticides. A case report. Respiration. 1998;65(3):214-8. [Medline].
Kiper N, Gocmen A, Ozcelik U, et al. Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979-1994): prolonged survival with low-dose corticosteroid therapy. Pediatr Pulmonol. Mar 1999;27(3):180-4. [Medline].
Le Clainche L, Le Bourgeois M, Fauroux B, et al. Long-term outcome of idiopathic pulmonary hemosiderosis in children. Medicine (Baltimore). Sep 2000;79(5):318-26. [Medline].
Malhotra P, Aggarwal R, Aggarwal AN, et al. Coeliac disease as a cause of unusually severe anaemia in a young man with idiopathic pulmonary haemosiderosis. Respir Med. Apr 2005;99(4):451-3. [Medline].
McCoy KS. Hemosiderosis. In: Taussig LM, Landau LI, eds. Pediatric Respiratory Medicine. St. Louis, Mo: Mosby; 1999:835-40.
Milman N, Pedersen FM. Idiopathic pulmonary haemosiderosis. Epidemiology, pathogenic aspects and diagnosis. Respir Med. Jul 1998;92(7):902-7. [Medline].
Pinto LA, Oliveira A, Collaziol S, et al. Postinfectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in childhood. J Bras Pneumol. Nov-Dec 2006;32(6):587-91. [Medline].
Rossi GA, Balzano E, Battistini E, et al. Long-term prednisone and azathioprine treatment of a patient with idiopathic pulmonary hemosiderosis. Pediatr Pulmonol. Jul 1992;13(3):176-80. [Medline].
Saeed MM, Woo MS, MacLaughlin EF, et al. Prognosis in pediatric idiopathic pulmonary hemosiderosis. Chest. Sep 1999;116(3):721-5. [Medline]. [Full Text].
Sun LC, Tseng YR, Huang SC, et al. Extracorporeal membrane oxygenation to rescue profound pulmonary hemorrhage due to idiopathic pulmonary hemosiderosis in a child. Pediatr Pulmonol. Sep 2006;41(9):900-3. [Medline].
Torres MJ, Giron MD, Corzo JL, et al. Release of inflammatory mediators after cow's milk intake in a newborn with idiopathic pulmonary hemosiderosis. J Allergy Clin Immunol. Dec 1996;98(6 Pt 1):1120-3. [Medline].
Zaki M, al Saleh Q, al Mutari G. Effectiveness of chloroquine therapy in idiopathic pulmonary hemosiderosis. Pediatr Pulmonol. Aug 1995;20(2):125-6. [Medline].

