Abstract
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related, interstitial lung disease, and pulmonary hypertension (PH) is associated with mortality. We herein report a case of PLCH complicated by severe PH and respiratory impairment. After developing PH, the patient displayed a cystic pattern on chest high-resolution computed tomography (HRCT). This, in turn, corresponded with the scarring stage of PLCH. However, the patient's PH and respiratory impairment improve dramatically following smoking cessation. PLCH patients with a cystic pattern on chest HRCT may still be able to improve their PH and respiratory impairment when they are able to quit smoking.
MeSH terms
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Diuretics / therapeutic use
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Female
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Histiocytosis, Langerhans-Cell / complications
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Histiocytosis, Langerhans-Cell / diagnostic imaging
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Histiocytosis, Langerhans-Cell / physiopathology*
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Histiocytosis, Langerhans-Cell / therapy
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Humans
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Hypertension, Pulmonary / diagnostic imaging
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Hypertension, Pulmonary / etiology
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Hypertension, Pulmonary / physiopathology*
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Hypertension, Pulmonary / therapy
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Middle Aged
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Respiratory Insufficiency / diagnostic imaging
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Respiratory Insufficiency / etiology
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Respiratory Insufficiency / physiopathology*
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Respiratory Insufficiency / therapy
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Smoking / adverse effects*
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Smoking Cessation*
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Tomography, X-Ray Computed
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Treatment Outcome
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Vasodilator Agents / therapeutic use
Substances
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Diuretics
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Vasodilator Agents