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Diffuse Interstitial Pulmonary Fibrosis

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Diffuse Lung Disorders

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Abstract

Histiocytosis X is a systemic disease characterized by the proliferation and infiltration of Langerhans’ cells in various organs [1–4]. The disease can manifest as an isolated disorder (i.e., eosinophilic granuloma of lung or bone) or as a systemic disease with multiple involvement including the skeletal, lung, reticuloendothelial system, skin and genitourinary system. The disseminated forms are more likely to appear in children and adolescents. In adults, disease isolated to lung is most common. There are some adult cases of eosinophilic granuloma of the lung in which skeletal and posterior pituitary gland involvement are present. However, systemic dissemination of histiocytosis X is less common in the adult populations. Furthermore, although the disseminated forms are more apt to occur in younger individuals, isolated organ involvement can also occur. There is sufficient evidence to suggest that the systemic forms of histiocytosis X are clonal neoplastic disorders of the Langerhans’ cell, thereby differentiating the systemic from the localized forms [2]. Most adult forms of histiocytosis X fall into the category of eosinophilic granuloma of the lung also referred to as Langerhans’ cell granulomatosis of the lung or pulmonary histiocytosis X.

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Authors
  1. Marvin I. Schwartz
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  2. David Lynch
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  1. The Cardiothoracic Centre, University of Liverpool, Liverpool, UK

    Miriam Sperber M.D. (Consultant Radiologist) (Consultant Radiologist)

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Schwartz, M.I., Lynch, D. (1999). Diffuse Interstitial Pulmonary Fibrosis. In: Sperber, M. (eds) Diffuse Lung Disorders. Springer, London. https://doi.org/10.1007/978-1-4471-3440-4_20

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