1 / 34

Biopsy not required for Idiopathic Pulmonary Fibrosis Dr. Altay Şahin Hacettepe University

Biopsy not required for Idiopathic Pulmonary Fibrosis Dr. Altay Şahin Hacettepe University. IPF is an idiopathic interstitial pneumonitis , which has the histological presentation of UIP and leads to chronic fibrosis

anthea
Download Presentation

Biopsy not required for Idiopathic Pulmonary Fibrosis Dr. Altay Şahin Hacettepe University

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Biopsy not required for Idiopathic Pulmonary FibrosisDr. Altay Şahin Hacettepe University

  2. IPF is an idiopathic interstitial pneumonitis, which has the histological presentation of UIP and leads to chronic fibrosis UIP pattern is not specific for IPF, can be also found in Collagen Vascular Disease (CVD) and even sarcoidosis Major criteira for the diagnosis: A) the other causes for ILD should be excluded, B) PFT defect, inadequate gas exchange, C) consistent HRCT findings, D) BALF analysis not supporting an alternative diagnosis, a) Age >50 years, b) acute emergence of dyspnea, c) duration of disease >3 months, d) Bibasillar inspiratuar crackles

  3. Gregory B. Diette; John C. Scatarige; Edward F. Haponik; Barry Merriman; Elli.. Do High-Resolution CT Findings of Usual Interstitial Pneumonitis Obviate Lung....Respiration; Mar/Apr 2005; 72, 2;134-141 • Experienced with the help of HRCT and clinical presentation radiologist can diagnose IPF, LAM, Eosinophilic granuloma, sarcoidosis and PCP with a high rate of correct result. • Biopsy from different lobes may show different histopathology upto 26% of cases • Most of the clinicians in US accept HRCT in the diagnosis of IPF, Asbestosis, Silicosis, ve bronchiectasis. Only 3% does not accept HRCT instead of biopsy. • Only 60% of the clinicans have read ATS/ERS guide, 10% were aware, while the others were unaware

  4. Fishbein MC. Chest 2005;128(5):520S-525SPeckham RM, et al. Respiration 2004;71:165-169 Board certifiedspecialistsevaluatedtheclinicaland HRCT findings in 26 patientsundergoingsurgeryand put thediagnosis of UIP in 14, NSIP in 5, sarcoidosis in 2, malignancy in 2, COP in 2, respiratorybronchiolitisassociated ILD andendstagefibrosis Forthediagnosis of IPF: Sensitivitywas 71%, positivepredictivevaluewas 77%, spesifitywas 75%, negativepredictivevaluewas 69%, therewas 4 falsenegativeand 3 falsepositivecases

  5. Kappa - Agreement • >0.8: almost perfect agreement • 0.6-0.8: substantial agreement • 0.4-0.6: moderate agreement • 0.2-0.4: fair agreement • 0.0-0.2: slight agreement • <0.0: poor agreement

  6. Nicholson AG, et al. Interobserver variation between pathologist in diffuse parenchymal lung disease. Thorax 2004;59:500-505 • Mean kappa for the first choice diagnosis was 0.38, and improved to 0.43 when the biopsies were received from multiple lobes • Weighted kappa, which shows the diagnostic probability had a mean of 0.58 with a range of 0.40-0.75 • Confidence in diagnosis was low in 18% of the biopsies • Difference between the pathologists in the diagnosis of NSIP especially the differential diagnosis from IPF exceeded 50%

  7. Irish Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Group, a subgroup of the British Thoracic Society Standards of Care Committee, A U Wells, N Hirani and on behalf of the BTS Interstitial Lung Disease Guideline Interstitial lung disease guideline Thorax 2008;63;v1-v58 • In the UK histopathological panel, primary diagnosis proportion was too low to be for the clinical use. For the majority of the cases histopathological features were indistinct, and consistent with two or more of the diagnoses. • Another important problem was that the biopsy was not representative of the predominant disease. This is particulat for the distinction between UIP and NSIP. • With consistent clinical presentation and HRCT findings IPF can be diagnosed in 70% of the cases. For these cases surgical biopsy does not have an additional benefit. Surgical biopsy by itself is not significant without the clinical and radiological findings. • Final diagnosis changed by 50% for the cases which lack the typical clinical and HRCT findings.

  8. Idiopathic Interstitial Pneumonia: What Is the Effect of a Multidisciplinary Approach to Diagnosis? Kevin R Flaherty; Talmadge E King Jr; Ganesh Raghu; Joseph P Lynch III; et al American Journal of Respiratory and Critical Care Medicine; Oct 15, 2004; 170:904-910 58 IIP cases diagnosed with surgical lung biopsy were investigated ina a stepwise manner Step 1: 3 clinicians and 2 radiologists independently reviewed HRCT findings and recorded their individual diagnoses Step 2: 3 + standard clinical information and recorded their individual diagnoses Step 3: + discussed HRCT findings with standard clinical information and recorded their individual diagnoses Patologhists (n: 2) unaware of these information recorded their individual diagnosis Step 4: + 2 pathologists independently reviewed and discussed HRCT findings with standard clinical information and pathological findings and recorded their individual diagnoses Step 4: + a consensus was sought, in case of disrepancy each recorded their individual diagnoses Agreement of the evaluations in these steps have been investigated

  9. Idiopathic Interstitial Pneumonia: What Is the Effect of a Multidisciplinary Approach to Diagnosis? Kevin R Flaherty; Talmadge E King Jr; Ganesh Raghu; Joseph P Lynch III; et al American Journal of Respiratory and Critical Care Medicine; Oct 15, 2004; 170:904-910 Correct diagnosis % as compared to pathologists: Clinicians 17-27% less with only HRCT review, 12-18% less with clinical infromation and radiologists’ discussion Radiologists %38-44 less with with only HRCT review, 34-41% less with clinical infromation and clinicians ’ discussion Histopatolojik bilgiler After the histopathological information clinicians 3-12% better, radiologists10-12% better than the pathologists Correct diagnosis % (reference: pathologists C: clinical information, D: discussion, P: pathology findings, Cs: consensus,

  10. Thomeer M, et al. Eur Respir J 2008;31:585-591 36 investigators from 6 European countries examined 179 HRCT and 82 OLB/TBL. Local investiagators sent their speciamens to 2 pathological investigation committee. Evaluations were classified as Very Suggestive, Probable and Unlikely. assessment of 512 HRCT : -67 Unlikely (12.6%), -203 Probable (38.2%), - 258 Very Suggestive (48.5%) assessment of 44 OLB, 38 TBL specimens: - 76 Very Suggestive (42.7%), - 66 Probable (37.1%), - 33 Unlikely (18.5%) UIP was diagnosed in 84% of the 82 biopsies, 92.7% of the 165 HRCT’s. When FVC (> 60% or <60%) was added to HRCT review correct diagnosis % increased to 100%. Clinical diagnosis proportion: 87.2% Agreement was 0.40 in HRCT, and 0.30 in histology

  11. Nicholson AG, et al. Thorax 2004;59:500-505 Raghu G, et al. Chest 1999;116:1168-1174 Hanninghake GW, et al. AJRCCM 2001;164:193-196 Ryu JH, et al. Mayo Clin Proc 2007;82:976-986 In UK, 10 pathologists retrospectively examined 133 lobar biopsy specimens of cases provided with age, sex and the biopsy site information, without findings of infection,. Confidence in the primary diagnosis of the pathologists was around 5%. The agreement between the primary diagnosis of the pathologists was kappa: 0.38, and increased to kappa: 0.43 with confidence level >70%, when multiple biopsies were taken. For IPF agreement increased from kappa: 0.42 to 0.49. Kappas were 0.76 to 0.82 for sarcoidosis , and 0.29 to 0.32 for NSIP, respectively. For UIP and NSIP, the differences between pathologists were significant in the end stage disease. According to these results, distinction in the histopathological diagnosis exists between routine pathological investigations.

  12. Lettieri CJ, et al. Respiratory Medicine (2005) 99, 1425–1430 Specialist Pathologists recorded distinct diagnoss in 52.3%, (kappa = 0.21). This caused a change in the treatment in 60%. Sensitivity and specificity of the general pathologists for IPF were 76.5% and 66.7%, respectively.

  13. Visscher DW, Myers JL. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006;3:322 • Three major problems reaching a diagnosis of UIP, -The first is sampling, “indeterminate pathologic findings”, -Presence of fibrotic changes resembling UIP in other conditions, -Microscopic findings, that resemble other conditions. • These problems need for clinical and radiographic correlation

  14. “This review is an attempt to address these controversies and doing so provide the pathologist with a straighforward and practical approach to diagnosing the chronic interstitial pneumoniast” Katzenstein ALA, et al. Human Pathology 2008;39:1275-1294 UIP Diagnostic Criteria 1. patchy involvement pattern of the parenchyma, non-uniform, distributed unevenly, 2. distorted structure, honey combing and scar, variable distribution of fibroblast foci and collagen deposition UIP – can be mixed up with Asbestosis, HP, CVD, if the biopsy is taken from a single lobe small specimen could be consistent with NSIP. UIP has a heterogenous distribution. Hipersensitivite Pnomonisi – can be mixed up with UIP. HP peribronchiolar epitheloid histiocyte non-nekrotising granuloma, mültinüclear giant cells, honey combing and fibrosis in advanced cases hardly distinguished from UIP. Since there is no entity like unclassified interstitial fibrosis, pathologists have difficulty in recording an appropriate diagnosis. This is especially the case for fibrotic NSIP.

  15. Katzenstein ALA, et al. Diagnosis of usual interstitial pneumonia and distinction from other fibrosing interstitial lung disease. Human Pathology 2008;39:1275-1294 • Debate on the role of pathology in the diagnosis of IIP is one of the hot topics for the time being. More accurate and confirmed diagnoses are required for the consideration of novel, expensive treatments. Unclassified interstitial fibrosis cannot be offered as a diagnostic entity. This view could be inducing the pathologists to record an incorrect diagnosis.

  16. Katzenstein ALA, et al. Human Pathology 2008;39:1275-1294 • 4.1. How is honeycomb change defined microscopically, and what is its relationship, if any, • to peribronchiolar metaplasia? • 4.2. Because focal fibroblast proliferation is a feature of both fibroblast foci and BOOP, can they • always be distinguished • 4.3. Are fibroblast foci specific for UIP? • 4.4. Because NSIP tends to occur in individuals slightly younger than those with UIP, and NSIP-like • areas can be found in UIP, is NSIP an early form of UIP? • 4.5. How much honeycomb change is too much to diagnose fibrosing NSIP? • 4.6. How much interstitial inflammation can be present in UIP before another diagnosis • is entertained?

  17. Katzenstein ALA, et al. Human Pathology 2008;39:1275-1294 • 4.7. When discordant biopsy results are encountered from different lobes, which is the correct diagnosis? • 4.8. Because biopsies from one lobe may occasionally show NSIP in cases with otherwise typical UIP in other lobes, can NSIP be accurately diagnosed on a biopsy taken from a single lobe? • 4.9. Once fibrosis becomes extensive, how important is it to separate fibrosing NSIP, chronic HP, and scarred LCH from UIP? • 4.10. Can idiopathic interstitial pneumonias other than UIP be diagnosed on TBB? • 4.11. If the experts do not always agree, can these diseases be accurately diagnosed? • 4.12. Can ordinary (nonpulmonary) pathologists diagnose UIP and related idiopathic interstitial pneumonias?

  18. Katzenstein ALA, et al. Human Pathology 2008;39:1275-1294 • 4.13. By dividing interstitial lung disease into fibrotic predominant and cellular predominant variants, are we reverting back to the classification schemes of the 1970s and 1980s? • 4.14. What terminology should be used on the pathology report when diagnosing the idiopathic interstitial pneumonias?

  19. Fujita J, et al. Idiopathic non-specific interstitial pneumonia: as an ‘‘autoimmune interstitial pneumonia.’’ Respir Med 2005;99:234–240. Selman M, et al. Gene expression profiles distinguish idiopathic pulmonary fibrosis from hypersensitivity pneumonitis. Am J Respir Crit Care Med 2006;173:188–198. Sahin H, et al. Chronic hypersensitivity pneumonitis: CT features comparison with pathologic evidence of fibrosis and survival. Ra Radiology 2007;244:591–598. • Investigations have revealed a predominant regulatory gene of extracellular matrix and chemokine activity regardless of the form of IIP as UIP or NSIP • The slight difference between NSIP and UIP is surprizing for the clinical differences between IIP’s. Others showed different transcript levels. The transcripts of familial IIP, which constitutes severe forms is different from sporadic IIP’s. • In IPF, immune cells other than macrophages, and dendritic cells are active. There is an association between pathological pulmonary fibrosis and inflammation.

  20. IPF Tanısı İçin Biyopsi Gerekmez • Farklı loblardan alına biyopsi örnekleri birbirlerinden farklı histopatolojik örnekleri temsil edebilmektedir. • Histopatolojik bulgular NSIP ve HP ile benzerlik gösterebilir. • Histopatolojik UIP tanısı ile klinik tablo ve hastalığın doğal seyri, tedaviye yanıtı her zaman paralellik göstermez. • Biyopsi tanısı zamanla değişebilmektedir. • UIP’nin kesin tanısı için histopatolojik spesifik bir belirteç yoktur. • Hastalığın doğal seyri, klinik belirti bulguları, laboratuar sonuçları, görüntüleme paternleri, tanı yönünden histopatolojiye göre daha fazla yardımcıdır.

  21. IPF Pathogenesis • Mutation in Telomerase Reverse Transcriptase (TERT) has been implicated for the pathogenesis. • Stem/progenitor cell replace the injured and apoptotic cells in the tissue . • Mesenchymal cells in the lung and alveolar type II cells express telomerase. This enzyme plays a critical role in the enhancement of the telomerase length. • When the telomerase shortens the renewal capacity of the stem cell decreases and the cell gets aged • Presence of mutant telomerase in the parenchymal cells could be responsible for the cellular death and/or limited regeneration capacity. • Short telomerase phenotype could be found in IPF. • However, telomerase length and telomerase action could be specific to the cell and autonomous

  22. ILD (DPLD) Diagnosis • History 1-Natural history/chronology of the disease, -acute, chronic and episodic (Eosinophilic pneumonia, vasculitides, HP or COP) 2-Respiratory risk factors and aetiological agents -Sigara -RB/ILD, DIP, LCH, Goodpasture synd., IPF (odds ratio 1.6-2.9) –HP and Sarcoidosis less likely -Occupation -Hobbies, Environment, Travel –Antigens for HP, Eosinophilic lung disease -Family History -IPF, Sarcoidosis -HIV Risk Factors -Opportunistic infections, LIP - Rheumatological Symtoms -Past Medical Hystory –Previous Malignancy, CT, RT, Surgery, -Vasculitis -Drug Hystory Symptoms -Cough, Sarcoidosis, HP, COP, IIPs, -Wheezing, Eosinophilic pneumonia, Churg-Strauss syndrome, -Pleural Inflamation, CVDs, Asbestos exposure,Drugs, • Physical Examination -Digital Clubbing, IPF, HP, CVD/ILD -Crackles (velcro) IPF, HP, Sarcoidosis -Inspiratory Squeaks, HP, NSIP, -Disease severity and cardiopulmonary capacity -Extrapulmonary Signs

  23. DPLD (ILD) Sınıflandırması • Known Cause -Hypersensitivity pneumonitis, -Connective Tissue Diseases associated ILDs, -Drug-induced ILDs, -Smoking-related ILDs Pulmonary Langerhans cell histiocytosis?, Respiratory bronchiolitis-associated ILD? (RB-ILD), Desquamative interstitial pneumonia? (DIP), Acute eosinophilic pneumonia, -Radiation-induced ILDs, -Toxic inhalation-induced ILDs • Unknown Cause Idiopathic Interstitial Pneumonias -Idiopathic pulmonary fibrosis -Non-Specific interstitial pneumonia -Acute interstitial pneumonia -Lymphocytic interstitial pneumonia -RB-ILD, -DIP, -Cryptogenic organizing pneumonia Eosinophilic Pneumonias Pulmonary Lymphangioleiomyomatosis

  24. ILD Klinik Karakteristikleri • ÖYKÜ 1-Hastalığın doğal ve kronolojik seyri –akut, -kronik, -epizodik (Eozinofilik pnomoni, -vaskülit/pulmoner hemoraji, -HP, -COP 2-Risk Faktörleri, Etyolojik Nedenler -Sigara RB-ILD, DIP, LCH, Goodpasture Send., IPF(odds ratio 1.6-2.9) -HP ve Sarkoidozis zayıf olasılık, -Meslek, -Hobiler/çevre/seyahat -HP antijeni, Eozinofilik hastalık -Aile Hikayesi IPF ve Sarkoidozis, -HIV Risk Faktörü, -Opurtunistik infeksiyon , LIP, -Romatolojik Belirtiler, -Önceki Tibbi Öykü, -Malignite, KT, RT, Cerrahi girişim, Vaskülit/Hemoraji, -İlaç Tedavileri, • BELİRTİLER -Öksürük, -Sarkoidozis, HP, COP, IIP’ler, -Hışıltılı >Soluk, -Eozinofilik pnomoni, Churg –Strauss sendromu, -Hemotezi, -Wegener’s granülomatozu, Goodpasture send. -Plörezi, -KVH’lar, Asbestoz teması, İlaçlar, • FİZİK MUAYENE -Çomak parmak, -IPF, RK.HP, KVH/ILD, -Velcro benzeri crackles, -IPf, HP, Sarkoidozis, -İnspiratuar Squeaks, -HP, NSIP, -Hastalığın Şiddeti, Efor kapasitesi, -Solunum Fonksiyonları, Genelde restriktif bozukluk, Küçük hava yolları için FEF25-75 , FEF75,, FEF85 ve FVC ( FVC < % 60 ileri evre), Tlco < % 40 ileri evre hastalık

  25. Shigeki Misumi and David A. Lynch Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia Imaging Diagnosis, Spectrum of Abnormalities, and Temporal Progression Proc Am Thorac Soc 2006;3:307–314 Features Helpful in CT Diagnosis of Fibrotic Lung Diseases ______________________________UIP______DIP_____NSIP____Kr_HP_____ Subpleural predominance ++ ++ 0 ± Peribronchovascular predominance 0 0 +++ 0 Ground glass 0 +++ +++ +++ Reticular 0 0 0 0 Honeycombing ++ 0 0 0 Nodules 0 0 0 +++ Mosaic attenuation/air trapping 0 0 0 +++ Cysts 0 +++ 0 0 0 = feature is not helpful in the CT diagnosis, +++ = feature is very importante CT Diag.

  26. Shigeki Misumi and David A. Lynch Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia Imaging Diagnosis, Spectrum of Abnormalities, and Temporal Progression Proc Am Thorac Soc 2006;3:307–314 Prevalance of Imaging Features in Fibrotic Lung Diseases __________________________________UIP_______DIP______NSIP_____Kr_HP____ Subpleural predominance ++ ++ 0 ± Peribronchovascular predominance 0 0 +++ 0 Ground glass ± +++ +++ +++ Reticular +++ +++ +++ +++ Honeycombing ++ ±±± Nodules 0 0 0 ++ Mosaic attenuation/air trapping 0 0 0 +++ Cysts 0 ++ 0 0 0 = feature does not occur, +++ = feature is present in all or almost all cases

  27. Ryu JH, et al. Diagnosis of Interstitial Lung DiseasesMayo Clin Proc 2007; 82;976-986 Diagnosis of Interstitial Lung Diseases Radyolojik Bulgular Konsolidasyon; Akut: DAH, AIP, Akut Eoz.Pnomoni, COP, İlaçlarla ILD, Kronik: Kr. Eoz.Pnom. COP, PAP, Sarcoidosis, Lenfoma Retiküler Patern; Akut: Pulm. Ödem Kronik: IPF, HP, Asbestozis, Sarkoidozis, İlaçlarla ILD Nodüler Patern <1cm / çap Akut: HP, Sarkoidozis, İnfeksiyon Kronik: Sarkoidozis, HP, Resp. Bronşiyolitis, Alveolar Mikrolitiyazis. Kistik Akut: Pnomosistitis pnomonisi, Septik embolizm Kronik: LCH, LAM, Lenfositik interstisyel pnomoni, Bal peteği-IPF Buzlu Cam Opasiteleri Akut: DAH, HP, AIP, İlaçlara bağlı ILD, Kronik: NSIP, HP, Resp. Bronşiyolitis ve ILD, DIP, PAP, İlaçlara bağlı ILD

  28. ILD (DPLD) Tanısı • Pulmonary Function Tests -Spirometry, Diffusion capacity, oximetry, - Arterial blood gas and pH -Cardiopulmonary exercise test, • Laboratory Tests -Complete blood cell count -Chemistry panel, -Serologic tests fo HP, -Tests for CVD, -Antineutrophil cytoplasmic antibodies, -BNP, proBNP, • Imaging Studies -CXR, -Previous imaging studies, -Chest CT and HRCT • Bronchoscopy

  29. Ryu JH, et al. Diagnosis of Interstitial Lung DiseasesMayo Clin Proc 2007; 82;976-986 • Thickened Interlobular Septa -Acute: Congestive heart failure, Pulmonary edema, -Chronic: Sarkoidosis, PAP •  Associated Findings • Traction Bronchiectasis: IPF, Asbestosis, Other chr. Fibrotic disorders, • Lymphadenopathy: Sarkoidosis, Berilliosis, Infections, Lymphangitis cars. Lymphoma, • Air Trapping: HP, Resp. Bronchiolitis associated ILD, DIP, Sarkoidosis, • Pleural Effusion or Thickening: Asbestosis, KVH-ILD, LAM, Lymphoma, Lymnfangitic carcinomatosis, drug-induced ILD • ILD’nin bilinen histopatolojik patern sayısı sınırlıdır. Bu paternlerin tanısal özellikleri farklıdır. Bazı biyopsi spesmenler tanısal özellikler taşırken bazıları nonspesifik anormallikler gösterir.

  30. Ryu JH, et al. Diagnosis of Interstitial Lung DiseasesMayo Clin Proc 2007; 82;976-986 • İnterlobüler Septal Kalınlaşma -Akut: Kalp yetmezliği, Pulmoner ödem, -Kronik: Sarkoidozis, PAP •  Diğer Bulgular • Traksiyon Bronşiyektazisi: IPF, Asbestozis, Diğer kr. Fibrotik bozukluklar, • Lenfadenomegali: Sarkoidozis, Berilyozis, Silikozis, İnfeksiyon, Lenfanjitis kars. Lenfoma, • Air Trapping: HP, Resp. Bronşiyolitis ve ILD, DIP, Sarkoidozis, • Plevral Sıvı veya Kalınlaşma: Asbestozis, KVH-ILD, LAM, Lenfoma, Lenfanjitis, İlaca bağlı ILD • ILD’nin bilinen histopatolojik patern sayısı sınırlıdır. Bu paternlerin tanısal özellikleri farklıdır. Bazı biyopsi spesmenler tanısal özellikler taşırken bazıları nonspesifik anormallikler gösterir.

  31. Ryu JH, et al. Diagnosis of Interstitial Lung DiseasesMayo Clin Proc 2007; 82;976-986 Diagnosis of Interstitial Lung Diseases Radiologic Findings Consolidation; Acute: DAH, AIP, Acute Eos.Pneumonia, COP, Drug-induced ILD, Chronic: Chr. Eos.Pneum. COP, PAP, Sarcoidosis, Lymphoma Reticular Pattern; Acute: Pulm. Odema, Chronic: IPF, CVD/ILD, Asbestosis, Sarcoidosis, HP, İlaca bağlı ILD, Cystic Airspace Acute: Pneumocystis pneumonia, Septic embolism Chronic: LCH, LAM, LIP, IPF-honeycomb lung Ground-glass Opacities Acute: DAH, HP, AIP, Drug-induced ILD, Chronic: NSIP, HP, Resp. Bronchiolitis associated ILD, DIP, PAP, Drug induced ILD

  32. Lleslie KO. Clin Chest Med2004;25:657-703, Verbeken EK. Eur Respir J 2001;32Suppl.:107S-113S Diğer Bulgular Traksiyon Bronşiyektazisi: IPF, Asbestozis, Diğer kr. Fibrotik bozukluklar, Lenfadenomegali: Sarkoidozis, Berilyozis, Silikozis, İnfeksiyon, Lenfanjitis kars. Lenfoma, Air Trapping: HP, Resp. Bronşiyolitis ve ILD, DIP, Sarkoidozis, Plevral Sıvı veya Kalınlaşma: AsbestozisILD’nin bilinen histopatolojik patern sayısı sınırlıdır. Bu paternlerin tanısal özellikleri farklıdır. Bazı biyopsi spesmenler tanısal özellikler taşırken bazıları nonspesifik anormallikler gösterir. , KVH-ILD, LAM, Lenfoma, Lenfanjitis, İlaca bağlı ILD

  33. Athol U. Wells1 and Cory M. Hogaboam. Update in Diffuse Parenchymal Lung Disease 2007 Am J Respir Crit Care Med 2008;Vol 177. pp 580–584, • NSIP initially was regarded as a confusing preliminary diagnosis and a presentation. It was classified as idiopathic NSIP and HP, and NSIP secondary to drug induced lung disease and especially to CVD • Recent information suggest that this distinction is not valid. When idiopathic NSIP is investigated precisely it is revealed that the clinical and serological abnormalities are due to the underlying CVD or its development, which did was not noticed. Another supporting evidence is the similarity of the survival between idiopathic NSIP and NSIP secondary to CVD. In contrast to that mortality in IPF is higher than that associated with CVD and UIP. • The classification has to be updated according to these views. The previous classification carries difficulties for the invesigations of new treatments. Fibrotic NSIP develops in HP, and is also a subgroup of idiopathic NSIP related to HP. The prognostic value of this pattern of HP is not well known, however recent data suggests that widespread reticular findings with or without honey combing and traction bronchiectasis in the CT illustrates the presence of fibrotic disease.

  34. Athol U. Wells1 and Cory M. Hogaboam. Update in Diffuse Parenchymal Lung Disease 2007 Am J Respir Crit Care Med 2008;Vol 177. pp 580–584, • NSIP başlangıçta bir ön tanı ve tanı kargaşası yaratan tablo şeklindeydi. İdyopatik NSIP ve HP, İlaca bağlı akciğer hastalığı ve özellikle KVH’lara sekonder NSIP olarak sınıflandırılmaktaydı. • Son bilgiler bu ayrımın yapay olduğunu düşündürmektedir. İdyopatik NSIP dikkatli araştırıldığında klinik ve serolojik anormalliklerin kuvvetle altta ayrımı yapılmamış KVH’lığı veya ona doğru gidişi düşündürdüğü görülmektedir. Bunu destekleyen bir durum, idyopatik NSIP’le KVH’lara segonder NSIP survivalları benzerdir. Zıt olarak IPF’de mortalite, KVH ve UIP birlikte bulunanlardan daha yüksektir. • Bu görüşlerle yeniden sınıflandırmalıdır. Eski sınıflandırma yeni tedavi araştırmaları için zorluklar taşımaktadır. HP’de fibrotik NSIP ve UIP gelişir, ve gene çalışmaları HP’in altında idyopatik NSIP’in bir alt grubudur. HP’deki bu paternin göreceli prognostik değeri bilinmemektedir, ancak son raporlara göre BT’deki yaygın retiküler bulgular bal peteği ve traksiyon bronşiyektazisi olsun olmasın altta bulunan fibrotik hastalığı göstermektedir.

More Related