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]

• 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]

• Heart Block

  A young woman with idiopathic pulmonary hemosiderosis (IPH) and advanced heart block below the atrioventricular node requiring pacemaker therapy is discussed. [ncbi.nlm.nih.gov]

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.

• Pulmonary Consolidation

  These events can produce all types of lung disease, pulmonary consolidations, and lymphadenopathy. Obstructive disease can be seen as the airways narrow with an increase in edema, mucus production and shedding of epithelial cells into the airway. [hawaii.edu]

• 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]

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.