Pulmonary Langerhans Cell Histiocytosis in a young Non-Smoking Female --Too many Rituals spoil the Lung: PLCH in a never smoker female

Pulmonary Langerhans Cell Histiocytosis in a young Non-Smoking Female --Too many Rituals spoil the Lung

PLCH in a never smoker female

Authors

  • Benhur Joel Shadrach Pulmonary, Critical care & Sleep Medicine, Department of Pulmonary Medicine, All India Institute of Medical Sciences (AIIMS), Jodhpur
  • Diksha Agnihotri Department of Neurosciences, Academia Sinica, Taipei
  • Rishabh Goel Department of Pulmonary Medicine, Shahi Global Hospital, Gorakhpur
  • Hari haran Department of Pulmonary Medicine Sarojini Naidu Medical College, Agra

Keywords:

PLCH, female, non-cigarette smoke.

Abstract

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare idiopathic cystic interstitial granulomatous lung disease seen almost exclusively in cigarette smokers. It typically occurs between 20-40 years of age and equally distributed among both sexes. Management includes smoking cessation, avoidance of second-hand smoke, and close follow-up. Corticosteroids may be required in those with symptomatic disease with worsening lung function despite smoking cessation and progressive nodular stage of the disease. Here, we report an interesting case of biopsy-proven PLCH in a young never smoker female. Detailed questioning revealed significant exposure to incense smoke, highlighting a rare presentation of non-cigarette smoke-related PLCH. Avoidance of incense smoke combined with oral prednisolone (0.5mg/kg) tapered over 6 months led to complete resolution of symptoms, disappearance of nodules in high resolution computed tomography (HRCT) of the thorax and improvement in lung function.

References

Lorillon G, Tazi A. How I manage pulmonary Langerhans cell histiocytosis. Eur Respir Rev. 2017;26:170070. doi:10.1183/16000617.0070-2017

Hidalgo A, Franquet T, Giménez A, et al. Smoking-related interstitial lung diseases: radiologic-pathologic correlation. Eur Radiol. 2006;16:2463–0. doi:10.1007/s00330-006-0340-0

Suri HS, Yi ES, Nowakowski GS, Vassallo R. Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis. 2012;7:16. doi:10.1186/1750-1172-7-16

Thomeer M, Demedts M, Vandeurzen K; VRGT Working Group on Interstitial Lung Diseases. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin Belg. 2001;56:163–2. doi:10.1179/acb.2001.026

Vassallo R, Ryu JH, Schroeder DR, et al. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med. 2002;346:484–0. doi:10.1056/NEJMoa012087

Li Y, Zhen W, Costable U, et al. A confusing case report of pulmonary langerhans cell histiocytosis and literature review. Open Med (Wars). 2016;11:178–2. doi:10.1515/med-2016-0034

Tadokoro A, Ishii T, Bandoh S, Yokomise H, Haba R, Ishida T. Pulmonary Langerhans cell histiocytosis in a non-smoking Japanese woman. Nihon Kokyuki Gakkai Zasshi. 2011 Mar;49(3):203-7.

Fernandes L, Vadala R, Mesquita AM, Vaideeswar P. Rare interstitial lung disease: Pulmonary Langerhans Cell Histiocytosis in a young non smoking Indian female. Indian J Tuberc. 2015;62(1):46‐49. doi:10.1016/j.ijtb.2015.02.008

Deokar K, Niwas R, Chauhan N, et al. Recurrent pneumothorax, skin lesions and frequent urination. Breathe (Sheff). 2020;16(1):190318. doi:10.1183/20734735.0318-2019

Baqir M, Vassallo R, Maldonado F, et al. Utility of bronchoscopy in pulmonary Langerhans cell histiocytosis. J Bronchology Interv Pulmonol. 2013;20:309–2. doi:10.1097/LBR.0000000000000021

Roden AC, Yi ES. Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective. Arch Pathol Lab Med. 2016;140:230–40. doi:10.5858/arpa.2015-0246-RA

Canuet M, Kessler R, Jeung MY, Métivier AC, Chaouat A, Weitzenblum E. Correlation between high-resolution computed tomography findings and lung function in pulmonary Langerhans cell histiocytosis. Respiration. 2007;74(6):640‐646. doi:10.1159/000106843

Kinoshita Y, Watanabe K, Sakamoto A, Hidaka K. Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation. Intern Med. 2016;55:491–5. doi:10.2169/internalmedicine.55.5152

Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans' cell histiocytosis. Thorax. 2000;55:405–16. doi:10.1136/thorax.55.5.405

Vassallo R, Ryu JH, Colby TV, et al. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med. 2000;342:1969–78. doi:10.1056/NEJM200006293422607

Lorillon G, Bergeron A, Detourmignies L, et al. Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med. 2012;186:930–2. doi:10.1164/ajrccm.186.9.930

Downloads

Published

30-04-2021

How to Cite

1.
Shadrach BJ, Agnihotri D, Goel R, haran H. Pulmonary Langerhans Cell Histiocytosis in a young Non-Smoking Female --Too many Rituals spoil the Lung: PLCH in a never smoker female. Acta Biomed [Internet]. 2021 Apr. 30 [cited 2024 Jul. 23];92(S1):e2021138. Available from: https://www.mattioli1885journals.com/index.php/actabiomedica/article/view/10133