Amer Saleem, Zafar Hussain Iqbal.
Pulmonary Lymphangioleiomyomatosis – very rare D.P.L.D. in asians.
J Allama Iqbal Med Coll Jan ;7(4):59-63.
Pulmonary lymphangioleiomyomatosis (LAM) consists of <1% of ILDs. This rare subtype of ILD is very rarely seen in Asians. Its cause is unknown and traditionally affects young women of reproductive age group. It is characterized by a proliferation of atypical smooth muscle cells, preferentially along the bronchovascular structures, causing progressive respiratory failure. Because of its rareness and nonspecific presenting symptoms, patients often receive a missed or delayed diagnosis. Here we present a case of young woman who attended our out-patient department with progressively worsening breathlessness. She was being treated for bronchial asthma at a periphery hospital and latter we diagnosed her as a case of pulmonary lymphangioleiomyomatosis. LAM may be more common than previously recognized, and it is imperative for primary care providers to be able to recognize this disease so they can make referrals to the specialty centers.
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