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  • 1. Bassel Ericsoussi, MD Pulmonary and Critical Care Fellow University of Illinois at Chicago
  • 2.
    • Pulmonary Langerhans Cell Histiocytosis (PLCH)
    • Eosinophilic Granuloma of the Lung
    • Pulmonary Langerhans Cell Granulomatosis
    • Pulmonary Histiocytosis X
      • Uncommon ILD
      • Young adults
      • Caucasians
      • Equal gender distribution
      • Smoke is an etiologic factor
      • The duration of illness is usually less than one year prior to diagnosis
  • 3.
    • Langerhans cells:
      • PLCH (cluster of Langerhans cells )
      • Smokers
      • IPF
      • Bronchioloalveolar carcinoma
    • Early inflammatory lesions surround the bronchioles, pulmonary arterioles and venules
    • Intraluminal fibrosis with progression to interstitial fibrosis (occur with advanced disease)
    • Predominance of disease in the mid to upper zones of the lung (IPF: lower zone predominance )
  • 4. Early lesions appear as cellular infiltrates Vascular intimal fibrosis with luminal obliteration (arrow) in a region involved by interstitial fibrosis and chronic inflammation
  • 5.
    • Strong association with cigarette smoking
    • No association of smoking with extrapulmonary Langerhans cell histiocytosis
    • Generalized activation or abnormalities in immune function
      • Increase in IgG in BAL
      • Immune complexes in the circulation
      • Abnormalities in T-cell function
  • 6.
    • Present to medical attention
      • Incidental findings on CXR
      • Spontaneous pneumothorax
      • Respiratory or constitutional symptoms:
        • Nonproductive cough (56 to 70 %)
        • Dyspnea (40 to 87 %)
        • Pleuritic chest pain (10 to 21 %)
        • Fatigue (30 %)
        • Weight loss (20 to 30 %)
        • Fever (15 %)
  • 7.
    • Usually unremarkable
    • Fine crackles and digital clubbing
    • LABORATORY STUDIES
    • Usually unrevealing
    • Eosinophil count is normal
  • 8.
    • Recurrent spontaneous pneumothorax (15 to 25%)that may cause pleural thickening or effusion
      • Pulmonary arteriopathy and veno-occlusive disease independent from parenchymal and airway disease
      • Impair diffusing capacity and exercise capacity (out of proportion to imaging findings)
      • Pulmonary hypertension
        • Severe
        • Common (88 % by echocardiogram)
        • a/w increased mortality
    • Hemoptysis (13 %)
    • Tumors (strong a/w smoking) - precede, follow, or occur concomitantly
      • Bronchogenic carcinoma (5 %)
      • Hodgkin's and non-Hodgkin's lymphoma [
      • Pulmonary carcinoid tumor
      • Mediastinal ganglioneuroma.
  • 9.
    • Cystic bone lesions (4 to 20 %)
      • localized pain or a pathologic bone fracture
    • Diabetes insipidus (hypothalamic involvement)
      • 15 %
      • Worse prognosis.
  • 10.
    • Symptoms are nonspecific
    • h/o recurrent pneumothorax, diabetes insipidus, or bone pain can be helpful
    • Current or past smoking history
  • 11.
    • CXR
    • Ill-defined nodules
    • Reticulonodular infiltrates (middle, upper lung zones )
    • Preservation of lung volume (hyperinflation and reduced volume can occur )
    • Costophrenic angle sparing
  • 12.
    • HRCT: progression from nodules to cavitating nodules to cystic lesions
    • Multiple cysts and nodules (mid to upper zones)
    • Interstitial thickening
    • Honeycombing
  • 13.
    • Restrictive pattern
    • Out of proportion reduction in DLCO
      • Pulmonary arteriopathy and veno-occlusive disease
    • Minority of patients (more advanced, cystic disease) may have hyperinflation with reactive airways
      • sometimes may benefit from bronchodilator
  • 14.
    • Limitation in activity and exercise intolerance out of proportion to pulmonary function abnormalities and imaging studies
      • Pathologic involvement of the pulmonary vasculature
      • Pulmonary arteriopathy and veno-occlusive disease independent from parenchymal and airway disease
    • Alveolar-arterial oxygen gradient
      • Normal at rest
      • Increased in exercise
  • 15.
    • Strongly suggests the diagnosis
      • > 5 % Langerhans cells on BAL
    • Langerhans cells can be seen
      • Smokers
      • IPF
      • Bronchioloalveolar carcinoma
  • 16.
    • Transbronchial biopsy
      • Usually is sufficient to make the diagnosis
      • Contraindicated in severe PAH (common in PLCH)
    • Video thoracoscopic lung biopsy
    • Langerhans cells
      • Characteristic staining for S-100 protein
      • Immunostaining with CD1a
    • Extensive fibrosis and less Langerhans cells in progressive disease
  • 17.
    • Cessation of smoking
    • Immunosuppressive therapies (steroids, cytotoxics) are of limited value
    • Palliative radiotherapy for symptomatic bone lesions
    • Lung transplantation
      • In advanced, progressive disease (severe pulmonary hypertension in end-stage PLCH)
      • Recurrence in the transplanted lung 20 %
      • Good postransplant survival rate
  • 18.
    • The natural history
      • Progression to end-stage fibrotic lung disease (with continued cigarette smoking)
      • Spontaneous remission of symptoms (with the cessation of smoking )
    • Good prognostic factors
      • Smoking Cessation
      • Sparing of the costophrenic angle
    • Poor prognostic factors
      • Pulmonary hypertension (increased mortality)
      • Diabetes insipidus (hypothalamic involvement)
      • Cigarettes smoking
      • Involvement of the costophrenic angle
  • 19.  
  • 20. 5 yrs survival 74%
  • 21.
    • Video thoracoscopic lung biopsy to make the diagnosis of PLCH (transbronchial biopsy is contraindicated in severe PH)
    • Echocardiogram should be done to all PLCH dyspneic pts to diagnose PH
    • Diminished exercise capacity in PLCH is related to pulmonary vascular dysfunction (not to ventilatory limitation)
    • PH in PLCH is related to pulmonary circulation involvement independent of small airway and lung parenchyma injury
      • Pulmonary arteriopathy and veno-occlusive disease
      • Recurrence in the transplanted lung 20 %
  • 22.
    • Histiocytosis Association of America: www.histio.org/us/assn
    • Pulmonary Langerhans cell histiocytosis: www.uptodate.com
    • Dauriat G, Mal H, Thabut G, Mornex JF, Bertocchi M, Tronc F, Leroy-Ladurie F, Dartevelle P, Reynaud-Gaubert M, Thomas P, Pison C, Blin D, Stern M, Bonnette P, Dromer C, Velly JF, Brugière O, Lesèche G, Fournier M. Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis. Transplantation. 2006 Mar 15;81(5):746-50.
    • Chaowalit N, Pellikka PA, Decker PA, Aubry MC, Krowka MJ, Ryu JH, Vassallo R. Echocardiographic and clinical characteristics of pulmonary hypertension complicating pulmonary Langerhans cell histiocytosis. Mayo Clin Proc. 2004 Oct;79(10):1269-75.
    • Harari S, Comel A. Pulmonary Langerhans cell Histiocytosis. Sarcoidosis Vasc Diffuse Lung Dis. 2001 Oct;18(3):253-62.
    • Hamada K, Teramoto S, Narita N, Yamada E, Teramoto K, Kobzik L. Pulmonary veno-occlusive disease in pulmonary Langerhans' cell granulomatosis. Eur Respir J. 2000 Feb;15(2):421-3.
    • Fartoukh M, Humbert M, Capron F, Maître S, Parent F, Le Gall C, Sitbon O, Hervé P, Duroux P, Simonneau G. Severe pulmonary hypertension in histiocytosis X. Am J Respir Crit Care Med. 2000 Jan;161(1):216-23
    • Crausman RS, King TE Jr. Pulmonary vascular involvement in pulmonary histiocytosis X. Chest. 1997 Dec;112(6):1714
    • Harari S, Brenot F, Barberis M, Simmoneau G. Advanced pulmonary histiocytosis X is associated with severe pulmonary hypertension. Chest. 1997 Apr;111(4):1142-4