Less Common Pulmonary Diseases Flashcards by Rodney Steff

Diameter of wall for classification of a cyst

<2 mm

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5 major types of diffuse cystic lung disease

LAM, PLCH, BHD, LIP, amyloidosis

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CT findings in LAM

diffuse cystic disease with normal intervening parenchyma

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Diagnostic criteria for LAM

Characteristic CT findings and 1 of the following (biopsy showing LAM, renal angiomyolipomas, chylothorax, tuberous sclerosis complex, high serum VEGF-D level > 800)

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Management of LAM

Sirolimus (rapamycin), supportive, lung transplant

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Cell proliferation causing LAM

HMB-45+ (LAM cells)

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Two inheritance patterns of LAM

Sporadic and TSC related gene mutations

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Characteristic individual with PLCH

young adult smokers

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Cells found in lesions with PLCH

CD1a+, langerin+ CD207

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Mutation associated with PLCH

BRAF-V600E

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2 extrapulmonary diseases associated with PLCH

Bone lesions and Diabetes Insipidus

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HRCT findings in PLCH

Irregular cysts with basilar sparing, nodules +/- cavidation, reticular opacities

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Diagnostic criteria for PLCH

Characteristic CT findings with BAL >5% CD1A cells or biopsy

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Management of PLCH

Stop smoking, 2-chlorodeoxyadenosine (2-CdA, cladribine), BRAF inhibitors (vemurafenib) if BRAF mutation, Steroids, Transplant

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Inheritance of BHD

autosomal dominant, phenotype variable

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Genes and chromosome associated with BHD

BHD (FLCN) gene on chromosome 17p11.2, tumor-suppressor protein gene, folliculin gene

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2 extrapulmonary findings in BHD

Fibrofolliculomas and renal tumors

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Diagnostic criteria for BHD

Cystic disease with any of the following (family hx of BHD, characteristic skin lesions, renal tumors, or gene testing)

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Treatment of BHD

Management of pneumothorax, life-long surveillance for kidney tumors, no specific therapy

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Disease characterized by monoclonal plasma cell proliferation localized to the lung

Pulmonary Light Chain Deposition Disease (LCDD)

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Substance secreted by macrophages in PLCDD that causes elastolysis and cyst formation

metalloproteinases

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Diagnostic criteria for PLCDD

Biopsy showing non-amyloid deposits in the alveoli and small airways (no beta pleated sheets and negative congo red staining)

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Treatment for PLCDD

Chemotherapy and HSCT

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HRCT findings in PLCDD

cysts with nodules

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Histopathologic findings in Follicular bronchiolitis

Hyperplastic lymphoid follicles with reactive germinal centers along bronchovascular bundles

HRCT findings in follicular bronchiolitis

Small nodules with patchy ground-glass opacities and occasional cysts

Diseases associated with follicular bronchiolitis

CTDs, immunodeficiency, infections, hypersensitivity reactions

2 rare inheritable causes of lung cysts

Neurofibromatosis type 1 (NF1) and Marfan syndrome

Disease characterized by accumulation of lipoproteinaceaous material in the alveoli

Pulmonary alveolar proteinosis (PAP)

Most common cause of PAP

autoimmune (90%) disruption of GM-CSF signaling leading to impaired surfactant metabolism by macrophages

Labs and antibodies elevated in PAP

Serum LDH, surfactant A and D, KL-6, anti-GM-CSF in serum and BAL

HRCT findings in PAP

GGO and/or consolidative opacities, patchy or diffuse with sharp demarcation from normal lung (Crazy Paving)

Treatment of PAP

Whole lung lavage (supportive care only if mild), GM-CSF replacement, rituximab, statin

Disease characterized by extensive intraalveolar deposition of concentrically lamellated calcium phosphate spheres

Pulmonary alveolar microlithiasis

Inheritance pattern and gene associated with pulmonary alveolar microlithiasis

Autosomal recessive, SLC34A2 gene

Mechanism behind pulmonary alveolar microlithiasis

SLC34A2 gene mutation leads to lack of type IIb sodium phosphate cotransporter in the lungs, leads to abnormal phosphorus metabolism by type II cells causing calcium phosphate microliths

Treatment for pulmonary alveolar microlithiasis

none, lung transplant

HRCT findings in pulmonary alveolar microlithiasis

"Sandstorm" appearence of parenchyma with black pleural line

Rare disease characterized by foamy CD1a negative histiocytes, fibrosis, and osteosclerotic lesions of long bones

Erdheim-Chester Disease

Management of Erdheim-Chester Disease

BRAF inhibitors (vemurafenib), interferon-alpha, chemo

Rare disorder characterized by diffuse lymphatic proliferation in the lungs

Diffuse pulmonary lymphangiomatosis

HRCT findings in diffuse pulmonary lymphangiomatosis

Diffuse interlobular septal thickening, patchy GGO, mediastinal infiltration, and pleural thickening/effusion

Treatment of Diffuse pulmonary lymphangiomatosis

sirolimus, bevacizumab

Most common form of amyloidosis

Two different patterns of parenchymal amyloidosis

Nodules (amyloidomas) or diffuse infiltrates with septal thickening

Diagnosis of pulmonary amyloidosis

biopsy material showing apple-green birefringence with polarized light on congo red staining

Treatment of trachobronchial amyloidosis

Bronchoscopic laser, external beam therapy

Treatment of diffuse parenchymal amyloidosis

treat underlying plasma cell dyscrasia

Disease characterized by lymphoplasmacytic infiltrate of IgG4 plasma cells and fibrosis

IgG4 related disease

Diagnosis requirements of IgG4RD

2 out of 3 (dense lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis), or High IgG4 level with clinical symptoms

Treatment of IgG4RD

30-60 mg/day of prednisone then taper over weeks to months, rituximab

Timing post radiation for radiation pneumonitis

4-12 weeks

Timing post radiation for radiation fibrosis

6-12 months

Treatment of radiation pneumonitis

40-60 mg/day prednisone x 2 weeks, then taper over 4-12 weeks

Disease characterized by congenital pulmonary manifestation with anomalous systemic arterial supply from the aorta

Bronchopulmonary sequestration

Raoof article differential for true cysts that are subpleural

Bullae, paraseptal emphysema, honeycombing

Raoof article differential for true cysts that are intraparenchymal and associated with GGO

PCP pneumonia, DIP

Raoof article differential for true cysts that are intraparenchymal with nodules on CT

LIP, PLCH, Light chain deposition disease, AMyloidosis

Raoof article differential for true cysts that are intraparenchymal, no other CT findings, and are solitary or incidental

Incidental cyst, pneumatocele, bronchogenic cyst

Raoof article differential for true cysts that are intraparenchymal, no other CT findings, and diffuse/multiple found

BHD, LIP, Malignancy, Infection, LAM, PLCH, tracheobronchial papillomatosis

Cystic lung disease associated with * Lower lobe predominant * Elongated * Subpleural and paramediastinal * Associated with a 24% pneumothorax risk

Birt Hogg Dube

Fibrofolliculomas in birt hogg dube

Cystic lung disease associated with * Round cysts * Diffuse involvement including _juxtaphrenic recesses_ * Intervening normal lung * Chylous effusions * Effects women of childbearing age * Renal angiomyolipomas

LAM

Cystic lung disease associated with * Cysts with nodular walls * Pathcy opacities and nodules * Areas of fibrosis * Multiple resections of papillomas and tracheostomies

Trachobronchial papillomatosis

Cystic lung disease associated with * Few cysts or centrolobular nodules * Bilateral reticular opacities, lower lobe predominant * Septal thickening * Middle aged women * Hx of CVD, HIV, or CVID

LIP

Cystic lung disease associated with * Nodules predominantly * Mediastinal adenopathy * Plasma cell dyscrasias * Ig deposition in kidneys, heart, and liver

Light chain deposition disease

Cystic lung disease associated with * Nodules primarily * Sjogren's syndrome * GGO * Nodules are lower lobe predominant

Amyloidosis

Cystic lung disease associated with * Upper and middle lobe nodules * Variable size nodules that are bizarre shapes * Spares costophrenic angles

PLCH

Less Common Pulmonary Diseases Flashcards

(68 cards)