Diffuse parenchymal diseaes 2 Flashcards
Treatment for ABPA
Steroids
Itraconazole (16weeks)
Asthma treatment
ICS
omalizumab ?
Loeffler Syndrome (secondary eosinophilic pneumonia)
Pulmonary eosinophilia
- Parasites
- Drugs
- idiopathic
DX:
- peripheral eosinophilia
- CXR with migratory opacities
- Fever, cough, wheezing, dyspnea
Tx:
- Self limited
Paragonimiasis
Eosinophilic PNA with eosinophilic pleural effusion
due to direct parenchymal invasion creating a nodular lesion
Sx:
- recurrent hemoptysis and chocolate colored sputum
CXR:
- Peripheral nodular lesion with GGO- mid and lower lobes
Labs:
- Peripheral eosinophilia (early)
- Eosinophilic pleural effusion
Dx:
- Eggs in sputum or BAL,
- ELISA
Tx:
- Praziquantel
- Triclabendazole
Medications that may cause Eosinophilic PNA
Daptomycin
ASA
Amiodarone
Cocaine
L trypophan
Nitrofurantoin - rash, pleural effusion
NSAIDS
SSRI
TX: stop drug +/- steroids
Idiopathic Acute Eosinophilic Pneumonia
Younger, male predominance
Recent onset of smoking/vaping/dust or enviromental stimuli)
Sx:
Fevers (5-7day)
cough, dyspnea
Imaging:
- Alveolar and interstitial opacities on imaging
- patchy opacities
- pleural effusions
BAL:
- >25% eos
—— increased IL-5, IL-18
- r/o other things- parasite, drugs, infection
Labs:
- Increased IgE
Tx:
- Steroids- rapid response (2-8WEEKS)
- Relapse is rare-
Chronic Eosinophilic Pneumonia
Middle aged woman
non- smoker
Asthma or atopy
Subacute/chronic presentation- cough, fevers, sweats, weight loss, wheezing
PNA that won’t resolve with ABX
Peripheral blood eos >30%
IgE elevated
BAL Eos >30-40%
PFTs: variable
Imaging:
- Peripheral opacities - GGO and dense consolidations
Tx:
- Steroids for 3-6m
- Relapse is common
Non-asthmatic Eosinophilic Bronchitis
Chronic cough
CXR clear
PFTs normal
Eos in sputum and BAL
Tx: CS/ICS
BHD (cystic lung disease)
Autosomonal dominant
FLCN gene (17p11.2)
Presents with PTX due to cystic lung disease
— cysts of varying size and shapes
Other - Fibrofolliculomas (diagnostic)
Kidney tumors
Pulmonary Light chain Deposition disease (cystic lung disease)
Monoclonal plasma cell proliferation localized more to the lung
non amyloid deposits in alveolar walls, small airways and vessels
HRCT: cysts and nodules. All over
Treatment: Chemotherapy
Can progress and result in respiratory failure
Follicular Bronchiolitis
Hyperplastic lymphoid follicles with reactive germinal centers along the bronchovascular bundles
Caused by:
- Idiopathic
- Infection
- CTD
- Hypersensitivity reactions
HRCT:
- Small nodules, GGOs, cysts
Management:
- Treat underlying cause
- bronchodilators
- Steroids
- Erythromycin
Pulmonary Alveolar Microlithiasis
Intra-alveolar deposition of CaP due to
- genetic mutation of SLC34A2 gene (affects phosphorous metabolism causes deposition)
PFTs:
- DLCO decreased
- FVC/TLC low
Treatment: transplant
pulmonary cement Embolism
After vertebroplasty
PE symptoms
Erdheim Chester Disease
Non-langerhans cell histiocytic disorder
- malignancy of myeloid progenitor cells
DOE for several months, with other organs also affected
— bones (lytic lesions- symmetric)
— pleuropulmonary disease
HRCT:
- Septal thickening
- GGO
- Mediastinal infiltration, pleural infiltration
BAL
- Foamy Histiocytes with MNG cells
- inflammatory cells
- CD1A negative
- BRAF mutation positive
Treatment:
- BRAF inhibitor (vemurafenib)
-
Diffuse pulmonary Lymphangiomatosis
Rare lymphatic disorder characterized by diffuse lymphatic proliferation in the lungs.
Dyspnea, cough
Chyloptysis
HRCT:
– septal thickening
– Mediastinal infiltration
– pleural effusion/thickening
– GGO
Dx: SLB
Tx: Sirolimus, lung transplant
Pulmonary Amyloidosis
Abnormal amyloid protein deposition in the lungs (all parts)
- AL most commonly
Airway disease:
- plaques
- Diffuse thickening of tracheobronchial tree
Parenchymal:
- nodular disease (may cavitate)
- Diffuse infiltrative disease- reticular pattern with septal thickening
** can calcify
Dx:
- biopsy- congo red
Tx:
- Nodules- observe
- Diffuse disease- treat underlying amyloid
- tracheo-bronchial tree: lasers
