| A 26 year old female with no known allergies, a 1 pack a day smoker and no prior exposure to toxic inhalations and no exposure to birds. She takes no regular medications. She complains of characteristic pleuritic pain over the right shoulder of 24hrs duration, not accompanied by any other respiratory symptoms. Physical examination: normal. Absence of crepitations and other respiratory sounds. No clubbing. Mantoux negative.
Respiratory Function Tests normal: pulmonary volumes and flow/volume curves normal. BP negative. Alveolar resistance in normal range. DLCO normal. Basal GSA normal.
Chest Xray (figure 1): diffuse bilateral interstitial pattern predominantly in the superior lobes, with the formation of small cysts and bullae, which may be shown in the CT cut corresponding to the superior lobes (figure 2).
Pulmonary biopsy was carried out which showed diffuse infiltration by cells of monocytic-macrophagic lineage with some eosinophilic accompaniment (20x HE). The cells were positive for the AS100 protein characteristic of Histiocytosis X or histiocytosis of Langerhan’s cells.
Histiocytosis X is closely related etiologically to tobacco use. It can present asymptomatically and be a coincidental find, or present with dyspnoea or chest pain secondary to a pneumothorax. Its evolution may produce pulmonary fibrosis.
Autor:
Juan Ignacio
Pérez Calvo
Servicio de Medicina Interna
Hospital Clínico Universitario
Zaragoza
Translation: Kelly
Watt
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