Hemosiderosis Clinical Presentation

Updated: Jan 14, 2015
  • Author: Galia D Napchan, MD; Chief Editor: Michael R Bye, MD  more...
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Presentation

History

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  • A triad of hemoptysis, iron deficiency anemia, and diffuse pulmonary infiltrates characterizes pulmonary hemosiderosis (PH).
  • Clinical presentation can range from an insidious onset to a fulminant course.
  • Hemoptysis may or may not be present in children. The absence of this symptom does not rule out the diagnosis of pulmonary hemosiderosis.
  • Sudden decrease in hematocrit levels associated with the onset of active respiratory disease is strongly suggestive of pulmonary hemosiderosis.
  • Nonspecific recurrent or chronic pulmonary symptoms, such as cough (dry or productive), dyspnea, tachypnea, and wheezing, are observed in most patients.
  • With severe pulmonary hemorrhage, hypoxemia and frank respiratory failure may result.
  • Symptoms of chronic fatigue, severe exercise limitation, and growth failure may accompany long-standing pulmonary hemosiderosis.
  • In Heiner syndrome, common clinical symptoms include chronic rhinitis, recurrent otitis media, recurrent cough, and poor weight gain. In these patients, symptoms improve when cow's milk is removed from the diet.
  • Pulmonary hemosiderosis, while uncommon, can occur in association with a wide variety of clinical disorders, many of which have overlapping features of glomerulonephritis and immune complex diseases.
  • von Willebrand disease, the most common inherited bleeding disorder, has multiple variants. Patients with this condition tend to have mucocutaneous bleeding and can present with pulmonary hemorrhage.
  • The combination of idiopathic pulmonary hemosiderosis (IPH) and celiac disease is extremely rare. In this case, pulmonary bleeding can occur in association with severe anemia and GI symptoms (eg, diarrhea, steatorrhea, flatulence) or even in the absence of the latter.
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Physical

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  • After an acute episode of pulmonary hemorrhage, the physical examination may reveal the following:
    • Tachypnea
    • Dyspnea (use of accessory muscles, retractions, flaring)
    • Pallor
    • Tachycardia
    • Cyanosis
    • Crackles
    • Wheezing
    • Fever
  • Long-standing severe pulmonary hemosiderosis is associated with the following:
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Causes

See the list below:

  • Primary pulmonary hemosiderosis
    • Idiopathic pulmonary hemosiderosis - The most common cause of pulmonary hemosiderosis in childhood
    • Heiner syndrome - Hypersensitivity to proteins from cow's milk
    • Goodpasture syndrome - Anti-glomerular basement membrane (GBM) antibody–mediated hemosiderosis
  • Secondary pulmonary hemosiderosis
    • Congenital or acquired cardiopulmonary abnormalities -Bronchogenic cyst, pulmonary sequestration, congenital arteriovenous fistula, tetralogy of Fallot, Eisenmenger complex, mitral valve stenosis, pulmonic valve stenosis, congenital pulmonary vein stenosis, pulmonary arterial stenosis, pulmonary embolism, left ventricular failure
    • Infections and their complications - Bacterial pneumonia, sepsis (disseminated intravascular coagulation [DIC]), pulmonary abscess, bronchiectasis, bronchiolitis obliterans
    • Immunologically mediated diseases - Systemic lupus erythematosus (SLE), periarteritis nodosa, Wegener granulomatosis, Henoch-Schönlein purpura, immune complex–mediated glomerulonephritis, allergic bronchopulmonary aspergillosis
    • Neoplasms - Primary bronchial tumors (adenoma, carcinoid, sarcoma, hemangioma, angioma) or metastatic lesions (sarcoma, Wilms tumor, osteogenic sarcoma)
    • Drugs - Penicillamine, cocaine
    • Toxins - Pesticide substances (synthetic peritroids)
    • Environmental molds -S atra, Memnoniella echinata
    • Miscellaneous causes - Retained foreign body, pulmonary trauma, pulmonary alveolar proteinosis, congenital hyperammonemia
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