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Toronto Notes 2019
Diseases of the Mediastinum and Pleura
Respirology R21
Pulmonary Vasculitis
Table 23. Pulmonary Vasculitis
Disease
Granulomatosis with Polyangiitis (GPA, previously Wegener’s Granulomatosis)
(see Nephrology, NP22) Eosinophilic
Granulomatosis with Polyangiitis (EGPA, Churg-Strauss)
Anti-GBM Disease (Goodpasture’s)
(see Nephrology, NP24)
Systemic Lupus Erythematosus, Rheumatoid Arthritis, Scleroderma
Definition
Systemic vasculitis of medium and small arteries
Multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral eosinophilia
A disorder characterized by diffuse alveolar hemorrhage and glomerulonephritis caused by anti-GBM antibodies, which cross-react with basement membranes of the kidney
and lung
See Rheumatology, RH17
Pulmonary Features
Necrotizing granulomatous lesions of the upper and lower respiratory tract
Asthma Infiltrates
Hemoptysis
May follow influenza infection
Extra-pulmonary Features
Focal necrotizing lesions of arteries and veins; crescentic glomerulonephritis
Life-threatening systemic vasculitis involving the lungs, pericardium and heart, kidneys, skin, and PNS (mononeuritis multiplex)
Anemia
Investigations
CXR: nodules, cavities, and alveolar opacities
c-ANCA
Tissue confirmation
Peripheral eosinophilia is the most common finding p-ANCA may be positive Biopsy involved tissue
CXR: may see alveolar infiltrates if hemorrhage is profuse
ELISA test with anti-GBM antibodies
Renal biopsy/indirect immunofluorescence shows linear staining
Treatment
Corticosteroids and cyclophosphamide or rituximab
Corticosteroids
Acutely: corticosteroids, plasmapheresis, immunosuppressive therapy
Severe cases: bilateral nephrectomy
Pulmonary Edema
• seeCardiologyandCardiacSurgery,C35
Diseases of the Mediastinum and Pleura
Mediastinal Masses
Definition
• mediastinum:boundbythethoracicinlet,diaphragm,sternum,vertebralbodies,andthepleura • canbebrokendowninto3compartments:anterior,middle,andposterior
Etiology and Pathophysiology
• diagnosisisaidedbylocationandpatient’sage
• anteriorcompartment:morelikelytobemalignant
■ “Four Ts” (see sidebar), lymphoma, lipoma, pericardial cyst • middlecompartment
■ pericardial cyst, bronchogenic cyst/tumour, lymphoma, lymph node enlargement, aortic aneurysm • posteriorcompartment
■ neurogenic tumours, meningocele, enteric cysts, lymphoma, diaphragmatic hernias, esophageal tumour, aortic aneurysm
Signs and Symptoms
• 50%asymptomatic(mainlybenign);whensymptomatic,50%aremalignant
• chestpain,cough,dyspnea,recurrentrespiratoryinfections
• hoarseness,dysphagia,Horner’ssyndrome(seesidebar),facial/upperextremityedema(SVCcompression) • paraneoplasticsyndromes(e.g.myastheniagravis[thymomas])
Investigations
• CXR(comparetoprevious)
• CTwithcontrast(anatomiclocation,density,relationtomediastinalvascularstructures) • MRI:specificallyindicatedintheevaluationofneurogenictumours
• U/S(bestforassessmentofstructuresincloseproximitytotheheartandpericardium)
• radionuclidescanning:131I(forthyroid),gallium(forlymphoma)
• biochemicalstudies:thyroidfunction,serumcalcium,phosphate,PTH,AFP,β-hCG
• biopsy(mediastinoscopy,percutaneousneedleaspiration)
Management
• excisionifsymptomaticenlargingbenignmassesorconcernsofmalignancy
• resectbronchogeniccystsandlocalizedneurogenictumoursviaminimallyinvasivevideoassisted
procedures
• explorationviasternotomyorthoracotomy
• diagnosticbiopsyratherthanmajoroperationifmassislikelytobealymphoma,germcelltumour,or
unresectable invasive malignancy
• ±post-operativeradiotherapy/chemotherapyifmalignant
Scleroderma is the most common collagen vascular disease affecting the lung
Horner’s Syndrome
Ptosis, Miosis, Anhydrosis
Differential of an Anterior Compartment Mass
4Ts
Thymoma
Thyroid enlargement (goitre) Teratoma
Tumours (lymphoma, parathyroid, esophageal, angiomatous)
Mediastinal Components
Anterior: sternum to pericardium and great vessels. Includes: thymus, extrapericardial aorta and branches, great veins, lymphatic tissues
Middle: pericardium (anteriorly), posterior pericardial reflection, diaphragm, thoracic inlet. Includes: heart, intrapericaridal great vessels, pericardium, trachea
Posterior: posterior pericardial reflection, posterior border of vertebral bodies, first rib to the diaphragm. Includes: esophagus, vagus nerve, thoracic duct, sympathetic chain, azygous venous system