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Adult LCH

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    The course of pulmonary LCH in adults is variable and unpredictable.[6] Fifty-nine percent of patients do well with either spontaneous remission with cessation of smoking, or with some form of therapy. Adults with pulmonary LCH who have minimal symptoms have a good prognosis, although some have steady deterioration over many years.[11] Age older than 26 years and lower FEV1/FVC ratio and higher RV/TLC ratio are adverse prognostic variables.[12] About 10% to 20% have early severe progression to respiratory failure, severe pulmonary hypertension, and cor pulmonale. Adults who have progression with diffuse bullae formation, multiple pneumothoraces, and fibrosis have a poor prognosis.[13,14] The remainder have a variable course with stable disease in some patients and relapses and progression of respiratory dysfunction in others, some after many years.[15] One study reported that smoking cessation did not increase the longevity of pulmonary LCH patients, apparently because the tempo of disease is so variable.[12] Patients receiving lung transplantation for treatment of pulmonary LCH have a 77% survival rate at 1 year and 54% survival rate at 10 years, with a 20% chance of LCH recurrence.[16]

    The most frequent pulmonary function abnormality finding in patients with pulmonary LCH is a reduced carbon monoxide diffusing capacity in 70% to 90% of cases.[12,17] A high-resolution computed tomography (CT) scan, which reveals a reticulonodular pattern classically with cysts and nodules, usually in the upper lobes and sparing the costophrenic angle, is characteristic of LCH. Despite the typical CT findings, most pulmonologists agree that a lung biopsy is needed to confirm the diagnosis.[18] The presence of cystic abnormalities on high-resolution CT scans appears to be a poor predictor of which patients will have progressive disease.[19] A study correlating lung CT findings and lung biopsy results in 27 pulmonary LCH patients has shed some light on pulmonary LCH.[20] Thin-walled and bizarre cysts had active LCs and eosinophils. Fifty-two percent of patients improved, most with smoking cessation, and some with steroid treatment within 14 months of diagnosis. Four patients (15%) were stable, and nine (33%) progressed over 22 months.

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