pulmonary langerhans PLCH symptoms, PFT, CT finding
- related: pulmonary langerhans histiocytosis PLCH
- tags: #literature #pulmonary
- disease: 1.PLCH
- weird sx/complications: 2.spontaneous pneumothorax, DI
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Pulmonary Langerhans cell histiocytosis (PLCH) most often presents in a young patient with dyspnea and non-productive cough.1
Spontaneous pneumothorax or diabetes insipidus are two complications seen in patients with pulmonary Langerhans cell histiocytosis (PLCH).1
The majority of patients with PLCH have low DLCO, which is the most common abnormality observed on physiologic testing. Reduction in DLCO may occur in isolation or accompany restrictive, obstructive, or mixed abnormality and is primarily a reflection of pulmonary vascular dysfunction. A restrictive pattern is more frequently observed in earlier stages of disease, while an obstructive pattern is more common as disease advances, and is the predominant pattern as disease progresses. The CT imaging features of PLCH are multiple ill-defined micronodules with a centrilobular distribution. As the disease progresses, these nodules tend to cavitate, and a combination of bizarre-shaped cysts and nodules is characteristic.2
The finding of multiple very irregular cysts and nodules in the middle to upper lung zones (vs. lower lung zone predominance in idiopathic pulmonary fibrosis) on CT in a young smoker is considered diagnostic for the disease.
Stellate lesions extending into the lung parenchyma is the characteristic histopathologic lesion characteristic of pulmonary Langerhans cell histiocytosis.