GPR: 2019 - Week 39

From MGH Learn Pathology

Clinical history

35 year-old man with history of chronic rhinosinusitis, and pancreatic insufficiency who was transferred from an outside hospital after embolization of bronchial artery for hemoptysis complicated by acute respiratory failure with hypoxemia and hypercarbia.


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GPR19-28 -s19-11922 R lung cut ncb.JPG

 
Cystic Fibrosis
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

Gross Pathology:

-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
 

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Idiopathic pulmonary fibrosis (IPF) / Usual interstitial pneumonia (UIP)

IPF – Idiopathic disease

Associated with smoking

Familial forms

Rarely younger than 50 years old

Chronic – progressive / poor prognosis mean survival 3.5-5 years


Gross Pathology:

-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.
 

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Miliary Tuberculosis

Lung infection caused by inhalation of Mycobacterium tuberculosis

Often associated with chronic, debilitating diseases or immunosuppressive states (AIDS, transplant, cancer, diabetes, alcoholism)


Different gross patterns of disease (different stages):

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.
 

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Lymphangioleiomyomatosis (LAM)

Rare, progressive and systemic disease that typically results in cystic lung destruction.

-Sporadic

-Associated with TSC complex


Women of reproductive age.

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.


Gross Pathology:

There is formation of numerous uniform thin-walled cysts and bullae throughout the lungs creating a honeycomb appearance.
 
Megan Fitzpatrick, MD

Nicole Babich

Erik Williams, MD

Elisabeth Tabb, MD

Dharam M. Ramnani, M.D.

This week's Gross Pathology Roundup was presented by Julian Villalba, MD on Mon, Sep 23, 2019.