Page 1261 - TNFlipTest

Toronto Notes 2019 Interstitial Lung Disease
Signs and Symptoms
• asymptomatic,cough,dyspnea,fever,arthralgia,malaise,erythemanodosum,chestpain • chestexamoftennormal
• commonextrapulmonarymanifestations
■ cardiac (arrhythmias, sudden death)
■ eye involvement (anterior or posterior uveitis)
■ skin involvement (skin papules, erythema nodosum, lupus pernio) ■ peripheral lymphadenopathy
■ arthralgia
■ hepatomegaly ± splenomegaly
• lesscommonextra-pulmonarymanifestationsinvolvebone,CNS,kidney • twoacutesarcoidsyndromes
■ Lofgren’s syndrome: fever, erythema nodosum, bilateral hilar lymphadenopathy, arthralgias
■ Heerfordt-Waldenstrom syndrome: fever, parotid enlargement, anterior uveitis, facial nerve palsy
Investigations
• CBC(cytopeniasfromspleenormarrowinvolvement)
• serumelectrolytes,creatinine,liverenzymes,calcium(hypercalcemia/hypercalciuriaduetovitaminD
activation by granulomas)
• hypergammaglobulinemia,occasionallyRFpositive
• elevatedserumACE(non-specificandnon-sensitive)
• CXR:predominantlynodularopacitiesespeciallyinupperlungzones±hilaradenopathy
• PFTs:normal,obstructivepattern,restrictivepatternwithnormalflowratesanddecreasedDLCO,or
mixed obstructive/restrictive pattern
• ECG:toruleoutconductionabnormalities
• slit-lampeyeexam:toruleoutuveitis
Diagnosis
• biopsy
■ transbronchial lung biopsy, transbronchial lymph node aspiration, endobronchial ultrasound
guided surgical (EBUS) biopsy, or mediastinoscopic lymph node biopsy for granulomas
■ in~75%ofcases,transbronchialbiopsyshowsgranulomasintheparenchymaeveniftheCXRisnormal
Staging
• radiographic,basedonCXR
■ Stage 0: normal radiograph
■ Stage I: bilateral hilar lymphadenopathy ± paratracheal lymphadenopathy
■ Stage II: bilateral hilar lymphadenopathy with pulmonary infiltration
■ Stage III: pulmonary infiltration alone (reticulonodular pattern or nodular pattern) ■ Stage IV: pulmonary fibrosis (honeycombing)
Treatment
• 85%ofstageIresolvespontaneouslywithin2yr
• 50%ofstageIIresolvespontaneouslywithin5yr
• steroidsforsymptoms,declininglungfunction,hypercalcemia,orinvolvementofeye,CNS,kidney,or
heart (not for abnormal CXR alone)
• methotrexateorotherimmunosuppressivesoccasionallyusedassteroidsparingagentswhenlongterm
prednisone required
Prognosis
• approximately10%mortalitysecondarytoprogressivefibrosisoflungparenchyma
Known Etiologic Agents
HYPERSENSITIVITY PNEUMONITIS
• alsoknownasextrinsicallergicalveolitis
• non-IgEmediatedinflammationoflungparenchyma(acute,subacute,andchronicforms)-Type4
hypersensitivity reaction
• causedbysensitizationtoinhaledagents,usuallyorganicdust
• pathology:airway-centred,poorlyformedgranulomas,andlymphocyticinflammation
• exposureusuallyrelatedtooccupationorhobby
■ Farmer’s Lung (Thermophilic actinomycetes)
■ Bird Breeder’s/Bird Fancier’s Lung (immune response to bird IgA) ■ Humidifier Lung (Aureobasidium pullulans)
■ Sauna Taker’s Lung (Aureobasidium spp.)
Signs and Symptoms
• acutepresentation:(4-6hafterexposure)
■ dyspnea, cough, fever, chills, malaise (lasting 18-24 h) ■ CXR: diffuse infiltrates
■ type III (immune complex) reaction
Respirology R15
Most common presentation of sarcoidosis: asymptomatic CXR finding
Hilar adenopathy refers to enlargement of mediastinal lymph nodes which is most often seen by standard CXR as spherical/ellipsoidal and/or calcified nodes. If unilateral - think neoplasia, TB, or sarcoid. If bilateral - think sarcoid or lymphoma
Corticosteroids for Pulmonary Sarcoidosis
Cochrane DB Syst Rev 2005;CD001114
Study: Meta-analysis of 13 RCTs involving 1,066 participants examining the use of steroids (oral or inhaled) in sarcoidosis.
Results: Oral steroids demonstrated an improvement
in CXR (RR 1.46, 95% CI 1.01-2.09). For inhaled corticosteroids, two studies showed no improvement in lung function and one study showed an improvement in diffusing capacity. No data on side-effects.
Conclusions: Oral steroids improve CXR findings and global scores of CXR, symptoms, and spirometry over 3-24 mo, but do not improve lung function or modify disease course. Oral steroids may be of benefit for patients with Stage 2 and 3 disease.
Calcified diaphragmatic plaques are highly suggestive of asbestosis, especially if bilateral
CXR Fibrotic Patterns
• Asbestosis: lower > upper lobes • Silicosis: upper > lower lobes
• Coal: upper > lower lobes