Subacute Onset of Pulmonary Langerhans Cell Histiocytosis with Resolution after Smoking Cessation

Wolters, Paul J.; Elicker, Brett M.

doi:10.1164/rccm.201405-0974im

Cited by 9 publications

(4 citation statements)

References 2 publications

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“…At present, there is no definite therapeutic plan for PLCH. The first therapeutic approach recommended for patients affected by PLCH is smoking cessation[19,20]. Corticosteroids are the second method, applied after smoking cessation.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Langerhans cell histiocytosis in adults: A case report

Wang¹,

Liu²,

Zhu³

et al. 2019

WJCC

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BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult. CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea. CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thin-walled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.

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Section: Discussionmentioning

confidence: 99%

Pulmonary Langerhans cell histiocytosis in adults: A case report

Wang¹,

Liu²,

Zhu³

et al. 2019

WJCC

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“…Management of PLCH, for those with or without associated PH, should most importantly center around smoking cessation. Stabilization and even substantial disease regression has been observed in those successful at quitting (169)(170)(171)(172). While most case reports and series have focused on improvements in radiology or pulmonary function following smoking cessation, benefits on the pulmonary vascular component of disease should also be presumed.…”

Section: Pulmonary Hypertension Associated With Less Common Comorbidimentioning

confidence: 99%

Epidemiology, Pathogenesis, and Clinical Approach in Group 5 Pulmonary Hypertension

2021

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Pulmonary hypertension (PH) is recognized to be associated with a number of comorbid conditions. Based on these associations, PH is classified into 5 groups, considering common pathophysiologic drivers of disease, histopathologic features, clinical manifestations and course, and response to PH therapy. However, in some of these associated conditions, these characteristics are less well-understood. These include, among others, conditions commonly encountered in clinical practice such as sarcoidosis, sickle cell disease, myeloproliferative disorders, and chronic kidney disease/end stage renal disease. PH in these contexts presents a significant challenge to clinicians with respect to disease management. The most recent updated clinical classification schemata from the 6th World Symposium on PH classifies such entities in Group 5, highlighting the often unclear and/or multifactorial nature of PH. An in-depth review of the state of the science of Group 5 PH with respect to epidemiology, pathogenesis, and management is provided. Where applicable, future directions with respect to research needed to enhance understanding of the clinical course of these entities is also discussed.

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“…In PLCH, due to its link with smoking habits, smoking cessation may be the only intervention required in a number of patients. The effect of smoking cessation on extra-pulmonary manifestations of the disease is not clear [100,101] and there is no relationship between ECD and smoking. In patients affected by PLCH, in case of disease progression despite smoking cessation, corticosteroids or other chemotherapeutic drugs should be considered, although no trial demonstrates the effect of steroids on reducing the decline of lung function.…”

Section: Plch and Erdheim-chester Diseasementioning

confidence: 99%

Ultra-rare cystic disease

et al. 2020

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Diffuse cystic lung diseases include a group of heterogeneous disorders characterised by the presence of cysts within the lung parenchyma, sometimes showing a characteristic computed tomography scan pattern that allows diagnosis. The pathogenetic mechanisms underlying cyst formation in the lung are still not clear and a number of hypotheses have been postulated according to the different aetiologies: ball-valve effect, ischaemic dilatation of small airways and alveoli related to infiltration and obstruction of small vessels and capillaries that supply the terminal bronchioles and connective tissue degradation by matrix metalloproteases. A wide number of lung cyst diseases have been classified into six diagnostic groups according to the aetiology: neoplastic, congenital/genetic, lymphoproliferative, infective, associated with interstitial lung diseases, and other causes. This article focuses on lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and Erdheim–Chester disease, Birt–Hogg–Dubé, follicular bronchiolitis and lymphocytic interstitial pneumonia, light-chain deposition disease and amyloidosis, congenital lung disease associated with aberrant lung development and growth, and cystic lung disease associated with neoplastic lesion. These cystic diseases are epidemiologically considered as ultra-rare conditions as they affect fewer than one individual per 50 000 or fewer than 20 individuals per million. Despite the rarity of this group of disorders, the increasing use of high-resolution computed tomography has improved the diagnostic yield, even in asymptomatic patients allowing prompt and correct therapy and management without the need for a biopsy.

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Subacute Onset of Pulmonary Langerhans Cell Histiocytosis with Resolution after Smoking Cessation

Cited by 9 publications

References 2 publications

Pulmonary Langerhans cell histiocytosis in adults: A case report

Pulmonary Langerhans cell histiocytosis in adults: A case report

Epidemiology, Pathogenesis, and Clinical Approach in Group 5 Pulmonary Hypertension

Ultra-rare cystic disease

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