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Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis is caused by recurrent diffuse alveolar hemorrhage with permanent consequences. It is one of the types of primary pulmonary hemosiderosis and its diagnosis is based on the classical association of iron deficiency anemia, diffuse pulmonary infiltrates and hemoptysis. The onset of the disease can be at any age and often goes unrecognized in children.


Presentation

Idiopathic pulmonary hemosiderosis patients present with symptoms associated with pulmonary suffering and signs of anemia, usually before reaching the age of 10 years [1]. Respiratory complaints, often recurrent or chronic, include coughing (dry or productive), wheezing, dyspnea, tachypnea, and hemoptysis, sometimes being wrongly diagnosed with lower respiratory infections [2]. However, hemoptysis is sometimes absent in children due to the fact that they swallow their sputum [3], but this does not exclude the presence of the disease. Symptoms may occur in an insidious or acute manner and initial presentation may be dramatic, with cyanosis, hypoxemia, respiratory failure, tachycardia, and fever. Clinical diagnosis is difficult in these cases, especially since pulmonary auscultation reveals crackles (caused by fibrosis [4] [5]) and pneumonia is easily suspected. Chronic but limited lung bleeding leads to more non-specific findings, like pallor, asthenia, exercise intolerance, failure to thrive, unsatisfactory ponderal and statural curves and finger clubbing.

Idiopathic pulmonary hemosiderosis may be accompanied by other illnesses, such as celiac disease [6] or von Willebrand disease. These associations induce more severe anemia, as the first one is a bleeding disorder per se, and the second one leads to enteral blood loss. Celiac disease may also cause flatulence, steatorrhea, and diarrhea, but the presence of these findings is not compulsory. Other associations include chronic granulomatous disease [7], idiopathic [8] and rheumatoid arthritis [9].

Pallor
  • A 5-year-old male patient was admitted to our clinic with sudden-onset pallor during iron therapy given for anemia. While he was being investigated for clinical and laboratory signs mimicking hemolytic anemia, he developed cough and dyspnea.[ncbi.nlm.nih.gov]
  • Chronic but limited lung bleeding leads to more non-specific findings, like pallor, asthenia, exercise intolerance, failure to thrive, unsatisfactory ponderal and statural curves and finger clubbing.[symptoma.com]
Fatigue
  • A 14-year-old girl was admitted to the pediatric emergency department with complaints of cough, dyspnea, fatigue and bloody sputum for 6 months. She had been hospitalized 3 times during this period and received antibiotics and blood transfusion.[ncbi.nlm.nih.gov]
  • The most common symptoms in adults are exertional dyspnea and fatigue due to pulmonary hemorrhage and iron deficiency anemia.[merckmanuals.com]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019[patientslikeme.com]
  • Hemorrhage]]) Cough Dyspnea Hemoptysis: may be absent on initial presentation in some cases, even after significant bleed May be streaky or life-threatening Chest Pain Crackles Interstitial Lung Disease (see [[ILD-Etiology]]) Other Manifestations Fever Fatigue[mdnxs.com]
  • If the condition develops slowly, symptoms such as chronic fatigue, a persistent cough, runny nose, wheezing, and delayed growth may occur.[rarediseases.about.com]
Hypoxemia
  • We conclude that pulmonary hypertension is probably a result of chronic hypoxemia experienced by patients with pulmonary hemosiderosis.[ncbi.nlm.nih.gov]
  • Symptoms may occur in an insidious or acute manner and initial presentation may be dramatic, with cyanosis, hypoxemia, respiratory failure, tachycardia, and fever.[symptoma.com]
  • Definition / general Rare, usually children or teenagers with hemoptysis, hypoxemia, dypsnea and iron deficiency anemia similar to Goodpasture’s disease, but no antibodies in serum or tissue, no renal disease Clinical triad of hemoptysis, iron deficiency[pathologyoutlines.com]
Cough
  • The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. Her hemoglobin level was 60 g/L, total serum IgE level was 900 U/mL and eosinophilia was 9%.[ncbi.nlm.nih.gov]
Dyspnea
  • The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. Her hemoglobin level was 60 g/L, total serum IgE level was 900 U/mL and eosinophilia was 9%.[ncbi.nlm.nih.gov]
Respiratory Distress
  • He had a respiratory rate of 58/min with respiratory distress, cyanosis, and grade III clubbing. Respiratory system examination was normal. Several previous reports of hemoglobin were as low as 3.6 g/dl with hypochromic and microcytic anemia.[ncbi.nlm.nih.gov]
  • The patient experienced increased respiratory distress on the day of presentation and came to the hospital for further evaluation. She was found to be hypoxic, tachypneic, and tachycardic requiring intubation for acute respiratory failure.[lungindia.com]
  • Patients present with acute, severe respiratory distress or failure requiring mechanical ventilation and chest radiograph will usually demonstrate bilateral infiltrates.[cdc.gov]
  • 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.[en.wikipedia.org]
  • C-Xray was done showed bilateral white out (Figure-1) so methylprednisolone (2mg/kg/dose 6 hourly for 48 hours) was started considering the diagnosis of acute respiratory distress syndrome.[jpma.org.pk]
Tachypnea
  • Respiratory complaints, often recurrent or chronic, include coughing (dry or productive), wheezing, dyspnea, tachypnea, and hemoptysis, sometimes being wrongly diagnosed with lower respiratory infections.[symptoma.com]
  • Her tachypnea decreased and we were able to stop supplementary oxygen. The child was discharged. She was put on maintenance steroids and was symptom free for 15 months.[indianpediatrics.net]
  • […] explained by parents and was mistaken for haematemesis, therefore, it is important to distinguish between the two in a clinical setting. 8,9 Other studies, however, have included different respiratory symptoms, such as recurrent cough, wheezing, and tachypnea[jpma.org.pk]
  • Common findings are, tachypnea, tachycardia, cyanosis, clubbing, fine or coarse crackles, wheezing, and hypoxemia.[hawaii.edu]
Flatulence
  • Celiac disease may also cause flatulence, steatorrhea, and diarrhea, but the presence of these findings is not compulsory. Other associations include chronic granulomatous disease, idiopathic and rheumatoid arthritis.[symptoma.com]
Steatorrhea
  • Celiac disease may also cause flatulence, steatorrhea, and diarrhea, but the presence of these findings is not compulsory. Other associations include chronic granulomatous disease, idiopathic and rheumatoid arthritis.[symptoma.com]

Workup

Idiopathic pulmonary hemosiderosis is a diagnosis of exclusion. Therefore cow milk protein allergy and autoimmune diseases, as well as other frequently associated illnesses like systemic lupus erythematosus, celiac disease, Goodpasture syndrome or Wegener granulomatosis must be ruled out. Towards this purpose, the physician may find it useful to order serum precipitins to casein and lactalbumin titers, anti-glomerular basal membrane, antinuclear antibodies, anti-DNA, anti-gliadin, anti-reticulin and anti-endomysial antibodies. The complete cell blood count will reveal the presence of anemia and possible eosinophilia. The anemia has iron deficiency characteristics (hypochromic and microcytic) [10]. Enteral blood loss is detected using guaiac tests. Keeping in mind that sputum is often swallowed by children, a gastric lavage analysis and bronchoalveolar lavage may prove useful, as they demonstrate the presence of presence of hemosiderin-bearing macrophages [4]. Despite the fact that pulmonary biopsy remains the gold standard for diagnosis [11], it can also be achieved using bronchoalveolar lavage [12] [13]. If sputum can be obtained, a cytological and bacteriologic analysis is necessary. Urinalysis may reveal proteinuria or hematuria. Coagulation disorders should also be investigated.

Chest radiography reveals bilateral alveolar infiltrates which are seen as opacities located in the basal and perihilar areas, that may be accompanied by edema [8]. After blood absorption, during remission phase, a reticular pattern remains. [4] Hilar adenopathy may be present in the acute phase. A more accurate diagnosis may be obtained with computed tomography, that also characterizes bronchial and vascular status, but pulmonary embolism is best excluded by ventilation/perfusion nuclear scanning.

Spirometry testing reveals a restrictive pattern between acute episodes and a mixed pattern during pulmonary hemorrhagic events.

Microcytic Anemia
  • IPH should be listed in the differential diagnosis of a child presenting with unexplained hypochromic, microcytic anemia and respiratory symptoms.[ncbi.nlm.nih.gov]
Ventricular Hypertrophy
  • Cardiac involvement in the form of myocarditis and right ventricular hypertrophy have been reported to occur in association with IPH, although findings on echocardiography have not been described.[ncbi.nlm.nih.gov]

Treatment

  • Results: All the patients achieved complete response after treatment with corticosteroid, leflunomide and MSCs. The median follow-up was 23 months (range: 4-34 months).[ncbi.nlm.nih.gov]
  • All the patients exhibited response to the initial treatment.[doi.org]

Prognosis

  • CONCLUSION: Adult IPH patients showed good prognosis. However, the present patients are clinically slightly different from the previously characterized IPH.[ncbi.nlm.nih.gov]
  • Keens , Prognosis in Pediatric Idiopathic Pulmonary Hemosiderosis , Chest , 116 , 3 , (721) , (1999) . N. Milman and F.Møller Pedersen , Idiopathic pulmonary haemosiderosis.[doi.org]
  • : in 75% of cases, mean survival is 3-5 years Lung Transplantation: controversial, as disease recurs after bilateral lung transplant Prognosis Adults have better prognosis than children 25% of adults remit after first episode 25% have persistent dyspnea[mdnxs.com]

Etiology

  • Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage with unknown etiology.[ncbi.nlm.nih.gov]
  • TERMINOLOGY Abbreviations Idiopathic pulmonary hemosiderosis (IPH) Synonyms Idiopathic pulmonary hemorrhage Definitions Intrapulmonary hemorrhage of unknown etiology that is unassociated with other systemic manifestations ETIOLOGY/PATHOGENESIS Etiology[basicmedicalkey.com]

Epidemiology

  • An epidemiologic history and genetic investigation should be included in the evaluation of patients with any disease when the cause is obscure.[ncbi.nlm.nih.gov]
  • […] of unknown etiology that is unassociated with other systemic manifestations ETIOLOGY/PATHOGENESIS Etiology Unknown No immunologic mechanism has been identified Familial clustering has been reported, supporting genetic susceptibility CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Epidemiology, pathogenic aspects and diagnosis , Respiratory Medicine , 92 , 7 , (902) , (1998) .[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • BACKGROUND: Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar hemorrhage in children and its pathophysiology remains obscure.[ncbi.nlm.nih.gov]
  • Clinical, pathophysiologic, radiologic and therapeutic findings in idiopathic pulmonary hemosiderosis Pascalis L., Saba L., Sessini P.[minervamedica.it]
  • Following this classification, the pathophysiology of pulmonary hemosiderosis [1] can be divided into 3 groups. Group 1 pulmonary hemosiderosis is defined by pulmonary hemorrhage associated with circulating anti-GBM antibodies.[emedicine.medscape.com]
  • Various hypotheses have been proposed to explain the pathophysiology of IPH: allergic, environmental, genetic and/or auto-immune. The allergic hypothesis is based on the frequent association between IPH and cow’s milk allergy [ 15 ].[ojrd.biomedcentral.com]

Prevention

  • Early diagnosis and treatment with corticosteroids prevents further episodes of recurrent alveolar hemorrhage and improves the clinical outcome.[ncbi.nlm.nih.gov]
  • A consistent chapter format enables you to rapidly and effortlessly locate the most current protocols on manifestations, etiologies, triggers, approaches to treatment, complications, and preventative strategies.[books.google.com]

References

Article

  1. Bakalli I, Kota L, Sala D, et al. Idiopathic pulmonary hemosiderosis—a diagnostic challenge. Italian Journal of Pediatrics. 2014;40:35.
  2. Yao T, Hung I, Wong K, et al. Pitfalls in the diagnosis of idiopathic pulmonary haemosiderosis. Arch Dis Child. 2002;86:436–438.
  3. Chen K, Hsiao C, Huang S, et al. Anemia as the sole presenting symptom of idiopathic pulmonary hemosiderosis: report of two cases. Chang Gung Med J. 2004;27:824–829.
  4. Ioachimescu O, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. European Respiratory Journal. 2004;24(1):162–170.
  5. Bulucea C, Sorin D. Idiopathic pulmonary hemosiderosis in children: a Romanian experience. Pediatrics. 2008;121:158–159.
  6. Ertekin V, Selimoglu M, Gursan N, Ozkan B. Idiopathic pulmonary hemosiderosis in children with celiac disease. Respiratory Medicine. 2006;100(3):568–569.
  7. Hartl D, Belohradsky B, Griese M, et al. Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous disease. Pediatric Pulmonology. 2004;38(4):344–348.
  8. Chu S, Shyur S, Peng Y, et al. Juvenile idiopathic arthritis with pulmonary hemosiderosis: a case report. Journal of Microbiology, Immunology and Infection. 2002;35(2):133–135.
  9. Topaloğlu R, Kiper N, Göçmen A, et al. Pulmonary hemosiderosis with juvenile rheumatoid arthritis: a case report. Turkish Journal of Pediatrics. 2000;42(2):148–150.
  10. Sankararaman S, Shah K, Maddox K, et al. Clinical case of the month. Idiopathic pulmonary hemosiderosis presenting as a rare cause of iron deficiency anemia in a toddler-a diagnostic challenge. J La State Med Soc. 2012;164(5):293–296.
  11. Afzal N, Mushtaq A, Rahman A, et al. Idiopathic pulmonary haemosiderosis presenting as severe iron deficiency anaemia–a case from Pakistan. J Pak Med Assoc. 2012;62(8):845–847.
  12. Khonglah Y, Raphael V, Bhuyan A, et al. Idiopathic pulmonary haemosiderosis: diagnosis by gastric lavage. Afr J Resp Med. 2013;8(2):26–27.
  13. Keller C, Langston C. Childhood idiopathic pulmonary hemosiderosis. N Engl J Med. 2000;343:781.

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Last updated: 2019-07-11 20:02