Pulmonary Involvement In Systemic Inflammatory Disease Flashcards
Life-threatening complication of JIA
Macrophage activation syndrome
Pulmonary involvement in JIA
Pleuritis, usually with pericarditis
Pulmonary hemosiderosis
Lymphoid folllicular bronchiolitis
Lymphocytic interstitial fibrosis
Alveolar proteinosis
Pulmonary manifestations of MAS
Pulmonary hemorrhage
Pulmonary edema
Pneumonitis
PFT in JIA
Restrictive > obstructive
Varying reductions in DLCO
Treatment of JIA
- NSAIDs; CS if unresponsive
- Methotrexate
- TNFa inhibitors (etanercept, adalimumab, infliximab)
Others: IL-1 antagonists, IL-6 antagonist (tocilizumab), cyclosporine
Prognosis of JIA
Low mortality
Acute pleuritis responds well without sequelae
High mortality in PAH, ILD, AP
Pulmonary involvement in SLE
Pleuritis with pleural effusion
Pulmonary infections
Inflammatory pulmonary lesions
Pleural effusion in SLE
Exudative
PFT in SLE
Slow, progressively restrictive pattern which may improve or stabilize
Histopathology in SLE
Alveolar wall thickening, interstitial fibrosis and infiltrates, deposits of immunoglobulin and complement
Rare complications of SLE
Pulmonary hemorrhage
Pulmonary hypertension
Shrinking lung syndrome
Common infections in pulmonary hemorrhage in SLE
Aspergillosis
Pseudomonas
CMV
Risk factor for pulmonary hypertension in SLE
Lupus anticoagulant
Treatment of SLE
Pleural effusion - corticosteroid
Pulmonary hemorrhage - MPPT, CS, cyclophosphamide, plasmapharesis
Others: hydroxychloroquine, rituximab
Most common cause of death in SLE
Infection
Associated with ILD in JDM in adults
Anti-Jo-1
Antisynthetase antibodies
Pulmonary involvement in JDM
ILD - can be rapidly progressive
Pneumomediastinum
Aspiration pneumonia
Hypoventilation
Most common presenting symptoms of ILD in JDM
Cough, dyspnea
PFTs in JDM
Restrictive - decreased lung volumes, normal or elevated FEV1/FVC
Reduced DLCO
HRCT in JDM
Irregular linear opacities with areas of consolidation and GGO (active inflammation)
Honeycombing is uncommon
Treatment of JDM
Corticosteroid, MPPT
Methotrexate
Cyclosporine + CS
IVIg
Rituximab
Pulmonary involvement in SSc
ILD
PAH
Cardiomyopathy
Mediator of vascular changes in SSc
Endothelin-1
Radiographic findings in SSc
GGO
Reticular pattern
Traction bronchiectasis
Honeycombing
Subpleural micronodules
DLCO in SSc
Reduced due to ILD or PAH
PFTs in SSc
Restrictive due to limited chest wall expansion, lung volume loss, pulmonary fibrosis
Associated with ILD in SSc
KL-6
Treatment for SSc
Cyclophosphamide
Lung transplantation
Stem cell transplantation
Others: MMF, azathioprine, rituximab
MCTD clinical features are a combination of:
SSc, SLE, JDM
Antibodies associated with MCTD
Anti-U1 RNP
Differentiates MCTD from SLE
Absence of neurologic or renal disease in MCTD
Pulmonary involvement in MCTD
Pulmonary fibrosis
Pulmonary effusion
PAH
Less common:
Thromboembolic disease
Pulmonary hemorrhage
Diaphragmatic dysfunction
Aspiration pneumonitis
Treatment of MCTD
Glucocorticoids
NSAIDs
Hydroxychloroquine
Others:
Nifedipine for Reynaud phenomenon
Methotrexate
Cytotoxics
Treatment of PAH in MCTD
Prostacycline analogues
Endothelin receptor antagonists
PDE5 inhibitors
Prognosis of MCTD
Incurable
Deaths are often due to rapid-onset PAH and infections
Pathology of sarcoidosis
Noncaseating epithelioid cell granulomas with predilection for thoracic lymph nodes and lung tissue
Long-term morbidities of sarcoidosis
Pulmonary fibrosis
Uveitis
Pathogenesis of sarcoidosis
Exaggerated TH-1 immune response to unidentified antigen in genetically susceptible individuals leading to granuloma formation
Hallmark of sarcoidosis
Granulomatous lesions, noncaseating, located in perilymphatic areas, subpleural soace, and perilobular spaces
Fate of granulomatous lesions
Heal with preservation of lung parenchyma or produce fibrotic scar tissue
Acute arthritis, BHL, erythema nodosum
Löfgren Syndrome
Pulmonary involvement in sarcoidosis
Commonly affects intrathoracic LN and parenchyma
Dyspnea, wheezing, cough, chest pain
Staging of BHL in sarcoidosis
0: normal
1: BHL
2: BHL + parenchymal infiltrates
3. Parenchymal infiltrates without BHL
4: pulmonary fibrosis
HRCT of sarcoidosis
Widespread micronodules and nodules in perilymphatic/peribronchovascular distribution
Nodular pleural thickening
Thickening of interlobular septae
Hilar, mediastinal LAD
Pulmonary fibrosis with honeycombing if long-standing
PFT and DLCO of sarcoidosis
Restrictive > obstructive
Normal PFT in St. 0 or 1
Reduced DLCO
Airway involvement in sarcoidosis
Waxy yellow mucosal nodules
Bronchial stenosis
Airway hyperreactivity
Complications of sarcoidosis
Pulmonary fibrosis
End-stage lung disease
Bronchiectasis
Chronic infection
Aspergilloma
Diagnosis of sarcoidosis
Tissue biopsy with noncaseating granulomas + clinical criteria
Biopsy NOT NEEDED for Löfgren
BAL profile in sarcoidosis
Lymphocytic
Treatment of sarcoidosis
Corticosteroids for 12-18 months
Methotrexate
Hydroxychloroquine
Prognosis of sarcoidosis
Spontaneous recovery in 2/3
Progressive pulmonary fibrosis and end-stage CLD if St. 3 on presentation
Excellent prognosis for Löfgren
Large- or medium-sized vasculitis causes:
Arterial insufficiency (infarction, necrosis, end organ dysfunction)
Small-sized vasculitis causes:
Leakage of blood into tissue (DAH)
Small-vessel vasculitides with frequent pulmonary involvement (ANCA-associated vasculitis)
GPA
MPA
EGPA
IPC
Features of EGPA
Severe atopic asthma and AR
Most common vasculitis syndrome presenting to pulmonologist
GPA
Etiology of GPA
Autoimmune (universal presence of ANCA, response to immunosuppressive therapy)
Pathogenesis of GPA
ANCA are directed against PR-3-ANCA, causing neutrophil activation and interaction with endothelium. Neutrophils migrate through the endothelium and degranulate, releasing toxic products and causing tissue damage. T-helper and B cells assist in autoantibody reaction.
Histopathology of ANCA-associated vasculitis
Necrotizing vasculitis of small blood vessels WITHOUT immune complex deposition
Unique about GPA among AAV
Anti-PR-3-ANCA
Granuloma
Triad of GPA
Upper airway
Lower respiratory tract
Renal
Presenting pulmonary symptoms of GPA
Dyspnea, chronic cough
Hemoptysis (necrotizing mucosal airway involvement)
Stridor and hoarseness (SG stenosis)
Upper airway findings in GPA
Saddle-nose deformity
Most common CXR finding
Nodules with fixed infiltrates
Others:
Cavitation
Mediastinal LAD
Pleural effusion
Pneumothorax
Chest CT findings in GPA
Nodules (multiple, >5mm, cavitating in 17%)
GGO
Air space consolidations (hemorrhage)
PFTs in GPA
Depends on tissues involved
Bronchoscopy findings in GPA
Mucosal erythema
Edema
Ulceration
Hemorrhage
Cobblestoning
Nodules
Polyps
Submucosal tunnelling
Synechial bands
Stenosis
Diagnosis of GPA
Clinical findings (e.g. pulmorenal syndrome)
Serology (anti-PR-3-ANCA)
Histopathology (pauci-immune granulomatous inflammation of small/medium-sized vessels)
Treatment of GPA
Glucocorticoids, cyclophosphamide
Rituximab if severe
Maintenance:
Methotrexate
Azathioprine
Treatment of GPA obstructive airway lesions
Intralesional steroid
Endoscopic dilatation
Stenting
Tracheostomy
Prognosis of GPA
Most will respond but will relapse
Pulmonary involvement of Sjogren’s sclerosis
Sicca cough
Small airway obstruction
Airway hyperreactivity
Follicular bronchiolitis
ILD/LIP
Treatment of SS
Steroid
Cyclophosphamide
Infliximab
DLCO in SS
Reduction that worsens during active disease
Single most common disorder in SS in adults
Bronchiectasis
Most common presentation for ILD
Slowly progressive dyspnea with or without dry cough
HRCT correlations
GGO: parenchymal lung disease
Peribronchovascular changes and air trapping: airways disease
Pleural thickening with effusion: pleural inflammation
DLCO in chest wall restriction
Reserved until with severe loss of volume
PFT in bronchiectasis
Obstructive
DLCO in DAH
Increased if hemorrhage is recent
