A 24yearold Caucasian woman presented with diffuse left sided chest pain and shortness of breath. The pain was sharp, nonradiating, pleuritic, and began acutely while sitting at her computer. Patient reported a chronic nonproductive cough. The patient had a 5 pack year smoking history. Her past medical history was significant for asthma since the age of 13. Family history was unremarkable. The patient’s oxygen saturation was 96% on room air and EKG showed sinus tachycardia. Lab work including complete blood count and metabolic profile was within normal limits (WNL). Chest Xray (CXR) showed interstitial lung disease, bilateral pneumothorax, left greater than right. The patient was kept on 100% non rebreather. A left side pig tail catheter was placed with continuous suction and later due to expansion of the right sided pneumothorax a right sided pigtail catheter was placed. High resolution computed tomography (HRCT) of he chest was performed, which demonstrated diffuse bilateral spherical and lobulated thin wall cysts consistent with lymphangioleiomyomatosis (LAM) (Image). An autoimmune workup for lupus and a1antitrypsin deficiency was negative. Renal ultra sound and echocardiogram were WNL. Based on the presentation and HRCT, primary diagnosis of LAM was made. The patient was stabilized in the ICU and was later transferred to the city’s LAM center where the lung biopsy revealed a definitive diagnosis of Pulmonary Langherhans Cell Histiocytosis (PLCH).
PLCH and LAM are rare lung diseases with unknown incidence and prevalence; both diseases affect young adults and Caucasians most frequently. PLCH predominately affects patients aged 20 to 40 with equal gender distribution while LAM affects women of childbearing age. Both are diagnosed by CXR following a spontaneous pneumothorax or persistent respiratory symptoms. After an abnormal CXR, an HRCT is performed and both diseases show multiple cysts, while PLCH may also have nodules with middle to upper lobe predominance and interstitial thickening. Tissue confirmation by biopsy with immunostaining is definitive. Recurrent pneumothorax, pulmonary hypertension, diabetes insipidus, and malignancy are common complications of PLCH. While complicates of LAM include spontaneous pneumothorax, chylothorax, chylopericardium. The treatment for PLCH should focus on smoking cessation. Immunosuppressive therapy such as glucocorticoids and cytotoxic agents are of limited value. No therapy has been proven beneficial for the treatment of LAM. Lung transplant should be considered in advanced progressive disease.
LAM and PLCH although being rare should be considered in the differential diagnosis in patients with spontaneous bilateral pneumothorax.
Figure 1Bilateral Pneumothorax With Diffuse Bilateral Spherical and Lobulated Cysts.
To cite this abstract:Siddiqui A, Mansoor M, Pompa S, Kilkenny T. Lymphangioleiomyomatosis (Lam) Verses Pulmonary Langherhans Cell Histiocytosis (Plch): A Clinical Dilemma. Abstract published at Hospital Medicine 2012, April 1-4, San Diego, Calif. Abstract 97956. Journal of Hospital Medicine. 2012; 7 (suppl 2). https://www.shmabstracts.com/abstract/lymphangioleiomyomatosis-lam-verses-pulmonary-langherhans-cell-histiocytosis-plch-a-clinical-dilemma/. Accessed November 14, 2019.