Presentation on theme: "RB-ILD DAD Alphabet Soup UIP NSIP DIP"— Presentation transcript:
1 RB-ILDDADAlphabet SoupUIPNSIPDIPA Primary Care Physician’s Guide to Idiopathic Interstitial PneumonitisMatt Mundy MS3IPFBOOPAIP
2 Idiopathic Interstitial Pneumonitis : The DilemmaIdentification of this class of diseases is notoriously difficult. Uncertain etiologies, discrepancies in the descriptions of the clinical, radiographic and pathologic characteristics of the disease processes and the necessity for multidisciplinary communication and cooperation in the clinical investigation all add to the challenge of making an accurate diagnosis. Combined with a seemingly endless string of acronyms used to refer to specific disease subtypes, these factors can make Idiopathic Interstitial Pneumonitis a seemingly insurmountable challenge to clinicians.
3 So………For a Primary Care Physician, what are the most important questions to ask about Idiopathic Interstitial Pneumonitis?What are the major subtypes of Idiopathic Interstitial Pneumonitis?What are the important Clinical, Pathologic, and Radiographic findings in each of these subtypes?What therapies are available when a diagnosis is ultimately made?
4 What are the most important questions to ask? By understanding the acronyms and answering just a few questions, a primary care doctor can have good insight into a patients’ disease and its prognosis. This understanding can then be used to guide future therapy and clinical decision making.
5 Welcome to IIP 101An quick and easy guide to the basics of Idiopathic Interstitial PneumonitisA, B, C, D, E, F, G………..
6 What do all the acronyms mean? Question #1What do all the acronyms mean?Clinical Diagnosis Pathologic DiagnosisIdiopathic Pulmonary Fibrosis (IPF)……………………..……..Usual Interstitial Pneumonia (UIP)Desquamative Interstitial Pneumonia (DIP)……………….……………………………….…….….same (DIP)Respiratory Bronchiolitis Interstial Lung Disease (RBILD)…….…………….same (RBILD)Acute Interstitial Pneumonia (AIP)…………………………..…Diffuse Alveolar Damage (DAD)Nonspecific Interstitial Pneumonia (NSIP)………………………………………………………same (NSIP)*adapted from: The American Thoracic Society / European Respiratory Society International Multidisciplinary Consensus Classification 2002
7 Important clarifications about the acronyms Each pathologic diagnoses has a distinct patternEach of the pathologic patterns has unique and consistent radiographic findingsThe pathologic and radiographic patterns can have multiple etiologiesA clinical diagnosis of a corresponding Idiopathic Interstitial Pneumonitis can only be made when no cause for the pathology can be identified
8 ILD Vs. DLD: What, more acronyms? Interstitial Lung Disease (ILD), a term used by pathologists refers to the same set of diseases that radiologists call Diffuse Lung disease(DLD).There are over 150 identified processes that fall under this descriptionAll of them can lead to lung scarring and respiratory failureExamples include: occupational, exposure related and drug induced diseases, eosinophilic pneumonias, sarcoidosis, collagen vascular related ILD, granulomatous disease and finally Idiopathic Interstitial Pneumonitis
12 UIP Everything Else What is the most important distinction to make? Question #2What is the most important distinction to make?Everything ElseUIPVs.
13 Why?Complete recovery is possible with all types of Idiopathic Interstitial PneumonitisExcept UIP/IPF!!!
14 Because of its poor prognosis and refractoriness to treatment, it is essential to identify cases of usual interstitial pneumonia / idiopathic pulmonary fibrosisThings to remember:Immunosuppression or smoking cessation will improve survival all types of IIP EXCEPT UIP/IPFThe median survival after diagnosis for UIP/IPF is yearsNo therapy has been shown to reduce the severity of pathologic findings or improve the prognosis of UIP/IPF
15 The diagnosis of UIP/IPF does not require pathologic confirmation With a high clinical suspicion, the characteristic radiographic findings of UIP are specific enough to be sufficient for diagnosisStudies show that combining clinical history, including pulmonary function tests, and CT findings alleviates the need for biopsy in up to 89% of patientsWhen conflicting or inconclusive findings arise, a biopsy specimen can solidify the diagnosis
16 The diagnosis of UIP/IPF implies many things, including: Again…….The diagnosis of UIP/IPF implies many things, including:There is probably no need for a biopsyTreatment of any sort is likely to have little, if any effectMOST IMPORTANTLY: UIP/IPF implies a very poor prognosis for the patient no matter what their age, gender or extent of disease
17 What is “ground glass” anyway? Question #3What is “ground glass” anyway?*many of the radiographic patterns of IIP involve ground glass opacities, therefore, the ability to identify ground glass on a CXR and CT is essential
18 Ground Glass Opacity:“focal or diffuse areas of ill-defined, hazy, increased lung attenuation, which cause pulmonary vascular indistinctness, yet through which vessels can still be identified.”**Nowers et al
19 Ground Glass Opacity:The ground glass pattern can be identified on both CT and conventional radiographs
20 Ground Glass Opacity:Ground glass falls on a spectrum of increasing opacification that culminates in consolidationconsolidationGround glass
21 What are the major subtypes of IIP? andWhat are the most pertinent clinical, radiographic and pathologic findings?*remember that IIP is a multidisciplinary diagnosis
22 Important subtypes of IIP UIP/IPF (usual interstitial pneumonia/idiopathic pulmonary fibrosis)NSIP (nonspecific interstitial pneumonia)AIP/DAD (acute interstitial pneumonia/diffuse alveolar damage)RB-ILD (respiratory bronchiolitis interstitial lung disease)DIP (desquamative interstitial pneumonia)*RB-ILD and DIP are strongly associated with smoking and respond well to smoking cessation. Therefore, arguments have been made against classifying them as idiopathic.
24 (Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis) UIP / IPF(Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis)Clinical Presentation -Historyage: usually greater than 50male to female ratio: between 1:1 and 2:175% have a smoking historyinsidious exertional dyspnea which is disabling over timenonproductive cough - refractory to antitussive medicationfever, malaise and arthralgia reported in 50%
25 (Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis) UIP / IPF(Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis)Clinical Presentation - Physical Examtachypneabibasilar inspiratory crackles75% have digital clubbingPFTs show restrictive physiology ( VC and TLC, FEV1 to FVC ratio)reduced diffusion capacity (DLCO)symptoms usually start >6 months before clinical presentation
27 (Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis) UIP / IPF(Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis)Differential Diagnosis (etiologies that can cause a UIP pattern)asbestosiscollagen vascular disease induced interstitial lung diseasedrug toxicitychronic extrinsic allergic alveolitis
28 (Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis) UIP / IPF(Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis)Radiographic Findings – Chest RadiographPeripheral, lace-like areas of fine reticular opacityConcentrated posteriorly in the basesVisible fibrosis in costophrenic recesses on lateral CXRGround glass opacification of lower lobes
29 UIP/IPFPeripheral reticular opacity more predominant in the bases
35 (Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis) UIP / IPF(Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis)If the clinical and radiographic findings are consistent and suggest UIP/IPF a diagnosis can be made without a biopsy. Pathologic evidence is only necessary in cases where the diagnosis is uncertain*all other forms of IIP require pathologic confirmation for diagnosis
36 (Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis) UIP / IPF(Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis)Pathologic FindingsVideo Assisted Thoroscopic Biopsy (VATS) required because of the peripheral distribution of diseasePatchy, dense subpleural collagenous fibrosis +/- HoneycombingFibroblastic foci of acute injuryVery little inflammationAreas of interspersed normal lungThe hallmark is spatial and temporal heterogeneity of disease
37 UIP/IPF – gross specimen Small, firm, fibrosed lung seen in advanced disease
38 UIP/IPF – gross specimen White tissue represents interstitial fibrosis
39 UIP/IPF – histologic section Spatial heterogeneity: fibrosis with interspersed normal lung
40 UIP/IPF – histologic section Area of acute lung injury: fibroblastic foci
41 UIP/IPF – histologic section Honeycombing: the final stage of UIP/IPF
42 (Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis) UIP / IPF(Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis)Treatment and Prognosisno response to steroids or other immunosuppressive drugssmoking cessation does not change course of diseasecourse marked by periods of rapid clinical deterioration5 year survival rate: 10%-50%median survival after diagnosis: yearsprognosis worse in men
44 Nonspecific Interstitial Pneumonia NSIPNonspecific Interstitial PneumoniaClinical Presentation - History and Physicalless chronic than UIP with a duration of months to yearsage: yearsmale to female ratio: 2:168% with smoking historydyspnea, cough and occasional fevertachypnea, crackles, no digital clubbingPFT’s: restrictive
45 Nonspecific Interstitial Pneumonia NSIPNonspecific Interstitial PneumoniaDifferential Diagnosis (etiologies that can cause an NSIP pattern)collagen vascular disease induced interstitial lung diseaseextrinsic allergic alveolitisresolving acute lung injurydrug induced pneumonitisinfection (HIV)
46 Nonspecific Interstitial Pneumonia NSIPNonspecific Interstitial PneumoniaNSIP is further divided into subtypesCellular NSIP is the less severe form that occurs in younger peopleFibrotic NSIP shares many of the features of UIP and has a worse prognosis than the cellular subtypeThese distinctions become important for both the radiographic and pathologic investigation of the diseaseAlthough distinctions have been made, it is clear that the two subtypes lie on a continuum and overlap does exist
47 Nonspecific Interstitial Pneumonia NSIPNonspecific Interstitial PneumoniaRadiographic Findings – cellular subtypeRelative predominance of ground glass opacity with fewer areas of fibrosisCan be distributed diffusely or in a basilar predominant patternPeribronchovascular thickening and areas of consolidationOccasional traction bronchiectasis secondary to chronic inflammation
48 Nonspecific Interstitial Pneumonia NSIPNonspecific Interstitial PneumoniaRadiographic Findings – fibrotic subtypeRelative predominance of fibrosis with areas of ground glass opacityReticular opacities and traction bronchiectasis can be seenSevere form can be indistinguishable from UIPDecreased occurrence and severity of honeycombing in the face of severe fibrosis suggests NSIP
49 NSIP Reticular opacity Bilateral basal ground glass and reticular opacities with peripheral distributionGround glass
51 Nonspecific Interstitial Pneumonia NSIPNonspecific Interstitial PneumoniaPathologic FindingsDiffuse pattern of inflammationRelatively more fibrosis in fibrotic subtypeUnderlying parenchyma is intactFibrosis, when present, is temporally homogenousMature hypocellular collagenLittle or no honeycombingInflammation predominantly lymphocytes
52 NSIPWidened alveolar walls (arrows) with increased numbers of lymphocytes adjacent to an area of normal lung
53 Pattern of mixed inflammation and fibrosis NSIPPattern of mixed inflammation and fibrosisinflammationFibrosis*note the absence of honeycombing
54 Nonspecific Interstitial Pneumonia NSIPNonspecific Interstitial PneumoniaTreatment and Prognosisthe two subtypes are treated the same but their outcomes are very differentglucocorticoids are the mainstay of therapyprognosis highly dependent on amount of fibrosis presentcellular: 5 year survival - 100%, 10 year survival - 100%fibrotic: 5 year survival - 90%, 10 year survival - 35%
56 Acute interstitial Pneumonia/Diffuse Alveolar Damage AIP/DADAcute interstitial Pneumonia/Diffuse Alveolar DamageClinical Presentation - History and PhysicalAcute onset of respiratory distressMainly affects current smokersUsually presents in 4th or 5th decade – mean age is 49Male to female ratio: 2:1Cough and dyspnea lasting weeks to months50% have fever50% have digital clubbing
57 Acute interstitial Pneumonia/Diffuse Alveolar Damage AIP/DADAcute interstitial Pneumonia/Diffuse Alveolar DamageDifferential Diagnosis – (etiologies that can cause DAD pattern)ARDSShockSepsisRadiationChemotherapyToxic inhalantsViruses
58 Acute interstitial Pneumonia/Diffuse Alveolar Damage AIP/DADAcute interstitial Pneumonia/Diffuse Alveolar DamageRadiographic FindingsInitial patchy ground glass opacityEvolves into more confluent ground glassScattered areas of consolidationTraction bronchiectasis and architectural distortion may be seen on CT
59 AIP/DADDiffuse symmetric ground glass opacities with slightly peripheral predominance
65 Acute interstitial Pneumonia/Diffuse Alveolar Damage AIP/DADAcute interstitial Pneumonia/Diffuse Alveolar DamageTreatment and PrognosisSupportive care is mainstay of treatment – mechanical ventilation is often requiredSteroids and antibiotics have shown little benefit>60% mortalityMost deaths occur within the first 6 monthsPeople who survive usually do not have recurrence of disease
67 Respiratory Bronchiolitis Associated Interstitial Lung Disease RB-ILDRespiratory Bronchiolitis Associated Interstitial Lung DiseaseClinical Presentation – history and physicalUsually asymptomaticMay have cough or mild dyspneaAge: usually 4th or 5th decadeMale to female ratio: 1:1History of heavy smokingBibasilar end inspiratory crepitationsCoarse rales
68 Respiratory Bronchiolitis Associated Interstitial Lung Disease RB-ILDRespiratory Bronchiolitis Associated Interstitial Lung DiseaseRadiographic findingsIll-defined ground glass centrilobular nodulesConfluent patchy areas of ground glass may also be seenmid to upper lung zone predominantFibrosis not seen on CXR or CTProgressive decrease in lung volumesCXR is often normal
69 RB-ILDDiffuse centrilobular opacities predominantly in lower lung zones
71 Respiratory Bronchiolitis Associated Interstitial Lung Disease RB-ILDRespiratory Bronchiolitis Associated Interstitial Lung DiseasePathologic FindingsVATS required for diagnosis because disease is centered around terminal bronchiolesMild interstitial mononuclear infiltrates of terminal bronchioles that extend into adjacent alveolar septaMild interstitial thickeningLittle or no fibrosis
72 RB-ILDAlveolar macrophages in lumen of respiratory bronchiolemildly thickened bronchiolar wall
73 Respiratory Bronchiolitis Associated Interstitial Lung Disease RB-ILDRespiratory Bronchiolitis Associated Interstitial Lung DiseaseTreatment and PrognosisSmoking cessation is mainstay of therapyFavorable but variable response to corticosteroids100% 10 year survival
75 Desquamative Interstitial Pneumonia DIPDesquamative Interstitial PneumoniaClinical Presentation – history and physical>90% are smokersAge: 4th or 5th decadeSubacute cough and dyspnea lasting weeks to monthsMale to female ratio: 2:1Restrictive PFT’s, Decreased DLCO, hypoxemia50% with digital clubbing
76 Desquamative Interstitial Pneumonia DIPDesquamative Interstitial PneumoniaDifferential Diagnosis (etiologies that result in DIP pattern)Subacute extrinsic allergic alveolitisPCPSarcoidDrug toxicityAsbestosisEosinophilic granulomatosisUIP, NSIP
77 Desquamative Interstitial Pneumonia DIPDesquamative Interstitial PneumoniaRadiographic findingsDiffuse ground glass opacitiesSimilar to RB-ILD, but confluent patchy areas of ground glass predominateMore likely affects basilar and sub-pleural areasFibrosis rarely seen on CXR or CTProgressive decrease in lung volumes
83 A quick recap……..IIPs are a spectrum of interstitial lung diseases for which no clear etiology can be determinedThe primary responsibility, when confronted with a patient with IIP is to differentiate whether they have a case of UIP/IPF or a case of something elseAlthough different types of IIP can present similarly in the clinic, radiographic and pathologic findings can clarify the diagnosis - A multidisciplinary approach is essentialWhether it be smoking cessation, or corticosteroid therapy, all types of IIP can be effectively treated and even cured, except UIP/IPF
84 Most Importantly…………Remember that behind the radiographs, chest CTs and histological sections, somewhere lost in the sea of acronyms is a person with a disease. Therefore, making an accurate diagnosis is essential, as it will dramatically influence the way your patient approaches the future.
85 ReferencesMyers J. Pathologic Classification of Idiopathic Interstitial Pneumonias. Up To Date, 2004.Nowers K. Rasband JD. Berges G. Gosselin M. Approach to Ground Glass Opacification of the Lung. Seminars in Ultrasound, CT and MRI. 23(4): , August 2002.Pandit-Bhalla M. Diethelm L. Ovella T. Sloop GD. Valentine VG. Idiopathic interstitial pneumonias: an update. Journal of Thoracic Imaging. 18(1):1-13, Jan uary 2003.Reynolds JH. Hansell DM. The interstitial pneumonias: understanding the acronyms. Clinical Radiology. 55(4):249-60, April 2000.Sholand MB. Clinical Approach to Interstitial Lung Disease. Seminars in Ultrasound, CT and MRI. 23(4): , August 2002.Szakacs JG. Pathologist’s Approach to Diffuse Lung Disease. Seminars in Ultrasound, CT and MRI. 23(4): , August 2002.