uncommon ILD - UNCOMMON INTERSTITIAL LUNG DISEASES Dr. Zia...

Info iconThis preview shows pages 1–15. Sign up to view the full content.

View Full Document Right Arrow Icon
UNCOMMON INTERSTITIAL LUNG DISEASES Dr. Zia Hashim PGIMER Chandigarh
Background image of page 1

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full DocumentRight Arrow Icon
Pulmonary Langerhans Cell Histiocytosis
Background image of page 2
Definition • Forms part of a spectrum of diseases characterized by monoclonal proliferation and infiltration of organs by Langerhans ' cell s • Lung involvement may occur either in isolation or as part of a multiorgan disease: bone, skin, pituitary gland, liver, lymph nodes, and thyroid. • PLCH refers to disease in adults that affects the lung, in isolation or in addition to other organ systems
Background image of page 3

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full DocumentRight Arrow Icon
Epidemiology • Afflicts predominantly whites, uncommon in Asian • 20-40 yrs • Initial reports: males: recent studies females • Incidence: ± 5% surgical biopsy series (N=502) ± 6.6% Italian registry of ILD (N=1382) • Rare cases of LCH have been reported following Hodgkin disease treated with chemoRx and radioRx
Background image of page 4
Relationship to smoking • Epidemiological: >90% smokers smoking precipitates PLCH in pts with LCH • Experimental observation: dendritic cells Nonclonal proliferation of Langerhans cells • Clinicopathologic observation: ¾ Langerhans cells in asymptomatic smokers ¾ Bronchiolocentric distribution of lesions ¾ Recurrence of disease in post-transplant ¾ Regression of after smoking cessation
Background image of page 5

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full DocumentRight Arrow Icon
Langerhans’cells • Type of dendritic cells • Site: beneath the tracheobronchial tree, Functions: serve as a primary line of defense surveying the inhaled antigens ¾ Inflammatory response ¾ tolerance
Background image of page 6
Pathogenesis Cigerette smoke Tobacco glycoprotein decreases IL-2 bombesin like peptides (from neuroendocrine cells) nodules Langerhans’ cells Reduced chemotaxis Don’t migrate Fibrob alst GF TGF- β , TNF- ά TNF- ά GM-CSF alveolar M φ
Background image of page 7

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full DocumentRight Arrow Icon
Pathogenesis Uptake of cigarette-smoke antigens by alveolar M φ or Langerhans’ cells promote local expansion of T cells and further inflammation stimulate B-lymphocyte Ab and Ag-Ab complexes
Background image of page 8
Role of GM-CSF & TNF- ά CD34+ hematopoietic stem cells GM-CSF TNF- ά Langerhans cell Fibrosis
Background image of page 9

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full DocumentRight Arrow Icon
Role of IL-2 • IL-2 inhibits histiocyte proliferation • relative lack of IL-2 favors local proliferation of Langerhans' cells in the lung Clinical remission of Langerhans'-cell histiocytosis in a child treated with intravenous interleukin-2.
Background image of page 10
Mechanism of cyst formation Nodule granulomatous destruction Cavitated nodule granulomatous destruction + bronchiolar dilatation Thick-walled cyst Fibrosis Thin-walled cyst
Background image of page 11

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full DocumentRight Arrow Icon
Increase in cyst size ¾ traction bronchiolectasis ¾ ball-valve effect in partially obstructed bronchioles • Confluence of cysts: honeycombing
Background image of page 12
Pathology • Histologic diagnosis: identification of typical lung lesions + reliable demonstration of no. of Langerhans' cells • Symmetric stellate lesions: Centripetal replacement of granulomatous nodular infiltrates by fibroblasts • Respiratory bronchiolitis • Emphysema
Background image of page 13

Info iconThis preview has intentionally blurred sections. Sign up to view the full version.

View Full DocumentRight Arrow Icon
Identification of Langerhans’ cells. Birbeck granules
Background image of page 14
Image of page 15
This is the end of the preview. Sign up to access the rest of the document.

Page1 / 89

uncommon ILD - UNCOMMON INTERSTITIAL LUNG DISEASES Dr. Zia...

This preview shows document pages 1 - 15. Sign up to view the full document.

View Full Document Right Arrow Icon
Ask a homework question - tutors are online