Inflammatory Lung Disorders Flashcards
Granulomatosis with polyangiitis Pathophys Affects Sx How to differentiate between other pulm renal Rx remission & maintenance
Granulomatous inflammation of small vessels
URT & LRT
Th1 response to antigen: TNF & IFN-gamma
ANCA+ve in 90%
Sx: fever, lethargy, joint complaints, cough, hemotysis, recurrent pulmonary infiltrate from haemorrhage
Nasal & sinus disease differentiates from other pulmonary renal syndromes
Rx steroids & cyclophosphamide for remission +/- Rituximab (steroid & cyclophos sparing)
MTX or Azathioprine for maintenance
Sarcoidosis
- histo
- HRCT stages/ findings
- Rx
Noncaseating granulomas on histo
Bilateral hilar lymphadenopathy
Right paratracheal lymphadenopathy
S2: reticular opacities & lymphadenpathy
S3: reticular opacity in upper lobes; lymphade regress
S4: reticular opacities coalesce; traction bronchiectasis; calcium deposits
Spontaneous remission common; Rx steroids if S3 or symptoms, restricted pattern on LFT
Sarcoidosis
- DDx
- bloods
- BAL findings
CGD Granulomatous lymphocytic ILD associated w CVID Hypersensitivity pneumonitis Granulomatosis with polyangiitis TB HIV Pnemocystis jiroveci
Hypergammaglob, hypercalciuria, hypercal, ALP, serum ACE
BAL: marked predominance of CD4, lymphocyte percentage >16%, elevated BAL d-dimer
GLILD
- occurs in
- clinical
- HRCT
- Rx
CVID Clubbing, recurrent pneumonia Hypogammaglob Hilar/mediastinal lymphadenopathy Ground glass Parenchymal nodules Biopsy for Dx Elevated TNF TNF inhibitors, cyclosporine Combo therapy: Rituximab & AZA
Loffler syndrome
- causes
- symptoms
- dx
- tx
A lumbricoides most common cause. Also toxocara canis, toxocara cati, strongyloides stercoralis
Fertilised egg ingested, penetrates duodenum and enters circulation, enters alveoli, coughed up and swallowed
Cough, dyspnoea & migratory pulmonary infiltrate, blood eosinophilia
Stool microscopy for eggs
Antihelminthic therapy; may worse inflammation in lungs acutely; Rx steroids
Eosinophilic pneumonia
- cxr finding
- HRCT findings
Fluffy alveolar infiltrates in the peripheral lung field
Ground glass & areas of confluent consolidation. Upper lobes & sub pleural zones most affected
Eosinophilic GPA
- features
- bloods
- rx
Asthma Eosinophilia Mono or polyneuropathy Non fixed pulmonary infiltrates Biopsy findings or extra vascular eosinophilic infiltrates
Skin, heart, GI, renal
Rhinitis, fever, weight loss
Linked with leukotriene receptor antagonists
P-ANCA + in 40%
Eosinophilia serum parallels the vasculitis
Rx steroids
Cyclophosphamide, INFa, cyclosporine, IVIG, plasmapheresis, anti IL 5 (mepolizumab)
Eosinophilic pneumonia
BAL findings
Th2 cytokines
- IL 4,5,6,10, 13, 18
Toxic granule proteins: major basic protein, eosinophilic derived neurotoxin, eosinophil cationic protein
> 25% eosinophils
ABPA
Complex mixed immunological hypersensitivity reaction to aspergillus sp
Asthma & CF
Elevated total IgE, anti-aspergillus IgG, skin prick positive, proximal bronchiectasis
Dyspnoea, cough, rubber plugs, peripheral & pulmonary eosinophilia
Monitor IgE
ABPA
Rx
Steroids
Anti- fungal itraconazole
Anti IgE omalizumab
ABPA
Cxr
Hrct
Upper lobe infiltrates
Central bronchiectasis
Renal pulmonary syndromes
Ddx
Goodpastures (antiGBM)
GPA
Microscopic polyangiitis
Henoch schonlein purpura
Anti-GBM disease
Antibody action
IgG1 and IgG4 complement binding , activate complement
Complement fragments signal recruitment of neutrophils & macrophages -> damage & capillaritis
AntiGBM disease Labs CXR BAL Rx
Anti-GBM in 90% CXR : widely scattered patches of pulmonary infiltrate Anaemia Harmaturia & proteinuria pANCA in 20%: harder to treat Haemosiderin laden macrophages Steroids & cyclophosphamide
