Pulmonary Hemosiderosis

Published on 25/03/2015 by admin

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Chapter 400 Pulmonary Hemosiderosis

The diagnosis of pulmonary hemosiderosis refers to the chronic and diffuse alveolar process diffuse alveolar hemorrhage (DAH), rather than focal or self-limited pulmonary hemorrhage. Pulmonary hemosiderosis has classically been characterized by the triad consisting of iron-deficiency anemia, hemoptysis, and multiple alveolar infiltrates on chest radiographs. A high level of clinical suspicion may be required for the diagnosis, because any or all of these features of the disease can be absent at any point in the course of the disease. Pulmonary hemosiderosis can exist in isolation, but more commonly, it occurs in association with an underlying condition. A precise etiology for hemorrhage is not always found. A diagnosis of idiopathic pulmonary hemosiderosis (IPH) is made when alveolar hemorrhage occurs in isolation and an exhaustive evaluation for underlying disease is found to be negative.


Most cases of DAH are associated with an underlying immunologic, rheumatologic, or vasculitic disorder but other diagnoses may manifest as recurrent or chronic pulmonary bleeding (Table 400-1).


Disorders with pulmonary capillaritis

Disorders without pulmonary capillaritis:   Noncardiovascular causes Cardiovascular causes

From Susarla SC, Fan LL: Diffuse alveolar hemorrhage syndromes in children, Curr Opin Pediatr 19:314–320, 2007.

Pulmonary hemosiderosis has historically been classified as primary or secondary. Primary pulmonary hemosiderosis (PPH) is described as encompassing the diagnoses of IPH, Goodpasture syndrome (Chapter 511), and Heiner syndrome (cow’s milk hyperreactivity); Goodpasture syndrome (or anti–basement membrane antibody disease) appears to be the most common among these entities as a cause of pulmonary hemorrhage.

Secondary pulmonary hemosiderosis refers to the remaining, diverse group of potential etiologies. Among these are cardiac causes of pulmonary hemosiderosis, such as congestive heart failure, pulmonary hypertension, and mitral valve stenosis. Vasculitic and collagen vascular diseases such as systemic lupus erythematosus (SLE; Chapter 152), rheumatoid arthritis (Chapter 148), Wegener granulomatosis (Chapter 161.4), and Henoch-Schönlein purpura (HSP; Chapter 161.1) are another important group to consider in the differential diagnosis. Coagulopathies are encountered and may be either inherited or acquired. Prematurity is also a recognized risk factor for hemorrhage. Pulmonary hemosiderosis has been well described in association with celiac disease. Postinfectious processes such as hemolytic-uremic syndrome (Chapter 478.4) and immunodeficiency syndromes, including chronic granulomatous disease (CGD; Chapter 124) have also been implicated. Numerous medications, environmental exposures, chemicals, and food allergens have been reported as potential causes.

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