GPR: 2019 - Week 39
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Multisystem disorder due to autosomal recessive mutations in CFTR gene (epithelial chloride channel)
Recurrent infections cause chronic lung disease, the most severe manifestation of the disease.
Other manifestations: Pancreatic insufficiency, Malnutrition, Cirrhosis
-Age: disease usually manifests in young children
-Airway dilatation extending into peripheral lung
-Airway-centered areas of fibrosis
-Thick, purulent mucus in dilated airways
-Parenchymal abscess from infection
-Patchy areas of consolidation - bronchopneumonia.-Areas of diffuse hemorrhage
Idiopathic pulmonary fibrosis (IPF) / Usual interstitial pneumonia (UIP)
IPF – Idiopathic disease
Associated with smoking
Rarely younger than 50 years old
Chronic – progressive / poor prognosis mean survival 3.5-5 years
-Peripheral subpleural areas of fibrosis of lung parenchyma, predominantly in lower lobes
-Peripheral honeycomb cystic changes in more advanced lesions. Mainly in lower lobes.
-In more advanced stages, there is traction bronchiectasis.
-Areas of beefy red, congested, and edematous parenchyma can can be seen in the setting of acute exacerbation of UIP - with superimposed diffuse alveolar damage.-Advanced stages with extensive fibrosis and honeycombing may be impossible to diagnose in absence of history.
Lung infection caused by inhalation of Mycobacterium tuberculosis
Often associated with chronic, debilitating diseases or immunosuppressive states (AIDS, transplant, cancer, diabetes, alcoholism)
1-Primary infection usually asymptomatic and leads to formation of Ghon complex (apical nodule with calcification) and hilar lymphadenopathy.
2-Progressive primary TB: primary foci do not involute and progress to disseminated, cavitary lesions, that may often lead to erosion into bronchus with intrabronchial spread.
3-Consolidation resembling bacterial pneumonia with areas of caseous (cheese-like) necrosis.4-Miliary TB: Numerous small (1-2 mm) nodules diffusely distributed throughout lungs.
Rare, progressive and systemic disease that typically results in cystic lung destruction.
-Associated with TSC complex
Lung destruction: consequence of diffuse infiltration by neoplastic smooth muscle-like cells that invade all lung structures
About 15% of patients with LAM have tuberous sclerosis.
There is formation of numerous uniform thin-walled cysts and bullae throughout the lungs creating a honeycomb appearance.
Erik Williams, MD
Elisabeth Tabb, MDDharam M. Ramnani, M.D.
This week's Gross Pathology Roundup was presented by Julian Villalba MD on 2019-09-23.