Pulmonary Langerhans’ cell histiocytosis
Journal Title: Postępy Nauk Medycznych - Year 2011, Vol 24, Issue 4
Abstract
Pulmonary Langerhans’ cell histiocytosis (PLCH) is a smoking-related interstitial lung disease. PLCH is characterized histologically by bronchiolocentric stellate nodules composed of Langerhans’ cells admixed with other inflammatory cells, often organized as loosely formed granulomas. The nodular lesions frequently cavitate and form thick and thin-walled cysts in pulmonary parenchyma. Affected patients are typically young adults who often present with cough and dyspnoea.The characteristic radiographic features of PLCH are bilateral nodular and reticulonodular areas of opacity that predominantly involve the upper and middle lung zones with relative sparing of the costophrenic angles. High-resolution computed tomography (HRCT) has proved to be useful in the diagnosis of PLCH, showing in typical cases nodules and cysts in the same distribution. The definitive diagnosis of PLCH generally requires surgical lung biopsy. In many cases a presumptive diagnosis can often be made on the basis of a characteristic clinical presentation and radiological findings. The course of the disease is often difficult to predict, and may range from spontaneous remission to progressive respiratory failure, cor pulmonale and death. Presently, smoking cessation, supportive therapy and, in advanced cases, lung transplantation, are the only therapies, that can be offered to patients with PLCH.
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