Dr. Gülcihan Özkan Y edikule Teaching Hospital for Chest Diseases and Thoracic Surgery

1. CASE PRESENTATION Dr. Gülcihan Özkan Yedikule TeachingHospitalfor ChestDiseasesandThoracicSurgery

2. M.F. • 62 yearsold • Female • Place of birth: Erzincan (EasternAnatolia) • Retired (formertextileworker)

3. anamnesis • Complaints: Fever, left chest pain, cough, bloody sputum. • History: She had a 2 month history of fever, cough, leftsidedpleuritic chest pain increasing with position. She also noted mild hemoptysis a few times during this period. Referred to our hospital from another hospital when no radiological regression with 10 days antibiotherapy was seen.

4. HISTORY • 1988 Thyroidoperation • 1991 Total abdominalhysterectomy + oopherectomy • 1992 car accident (leftpatellafracture) • Medication:Betahistin, trimetazidine, ginkgoglycosidesusageforvertigofor 1 year. • 5 packs-yearcigarettes (ex-smokerfor 10 years) • Hypertensionanddiabetes in her mother.

5. PHYSICAL EXAMINATION • BP: 100/70mmHg • HR: 86/min • RR: 20/min • Normal heartsounds • Endinspiratoryfinecrackles at posterior 1/3 lowerpart.of rightlung • Tenderness on lefthipocondrium

6. LABORAToryResults • Hemogram: N • ESR: 124 mm/h • CRP: 5.8 • Urineexam: N • Urinesediment: 1-2 leucocyte • Glc: 101 • Urea: 24 • Creatinine: 0.6 • ALT: 24 • AST: 26 • LDH: 131 • ALP: 133 • GGT: 56 • Electrolytes: N

12. ThoraxcT • Bilateralpleuralcalcifications • Periphericparenchymalnodules in leftlungupperlobeanterior, lowerlobeapical, andinferiorlingularsegments • Bilateral peripheral, patchy, amorphousparenchymal infiltrates with air bronchograms inlingulaandlower lobes (moreextensive on right)

13. What is yourdiagnosis? • Infections • Idiopathicinterstitialpneumonias • Eosinophiliclungdiseases • Collagenousdiseases • All of them

14. Sputum AFB smearwasnegative (2 times) • Gram stain of sputum : 10-12 PMNL, 7-8 epithelialcell, gram-positivecocci • Bacterialculture of sputum: Normal flora bacteria. • PPD: (-) • No parasites on stoolexam

15. PERIpheralbloodsmear • Lymphocyte : % 40 • Neutrophil : % 50 • Eosinophil : % 6 • Monocyte : % 4 • Normochromicnormocytaryerythrocytes, anisositosisandpoikilocytosis • Thrombocytessufficient

16. PFT-DLCO • FVC : 1.4 (%54) • FEV 1 : 2.1 (%64) • FEV1/FVC: %99 • DLCO : 15 (%67) • DLCO/VA : 6 (%132)

17. ArterialBloodGases • pH : 7.40 • pCO2 : 38.2 mmHg • pO2 : 79 mmHg • HCO3 : 23.8 mmol/l • O2 Sat. : % 95.6

18. ENA Profile-Vasculitic markers • ANA: (-) • Anti ds-DNA (IgGMA): 9.48 • AntikardiolipinIgM: 7.2 • AntikardiolipinIgG: 4.9 • Anti SSA: 0.45 • Anti SSB: 0.50 • Anti JO1: 0.43 • Anti SCL70: 0.71 • p-ANCA: (-) • c-ANCA: (-) • MPO ANCA: 1.0 • PR3 ANCA: 1.74

19. Serum ACE: Normal • Serum Ca: 8.6 • 24 hoururineCa: Normal • Total IG E: 958 IU/ml (<100)

20. BRONchOScOPy • LTC: Normal • Bothrightandleftbronchialsystemwere normal • Lavagefromrightlowerlobefor AFB andcytologicexam • BAL and TBB fromrightlowerlobeposteriorsegment

21. AFB smear of bronchiallavage : (-) • AFB smear of post-bronchoscopicsputum: (-)

22. BAL • Total cellcount: 538/mm3 • Lymphocytes : %70 • Neutrophils : % 5 • Macrophages : % 23 • Eosinophils : % 2 • CD4/CD8 : 0.25

23. PATHOLOGY • Bronchiallavage: Endobronchialepithelialcells, alveolarmacrophagesand a fewmaturelymphocytes • Post-bronchoscopicsputum: Normal respiratorycytology • TBB: Focalfibroticexpansion in alveolaryseptae, sparsemononuclearinflammatorycellsaccumulation • Comment: Nonspesificinterstitialpneumonia

24. Rheumotologyconsultation • No primaryrheumotologicdisease

27. WhatWIllwe do now? • Radiologicfollow-up • Waitfor TB culture • Re-bronchoscopy • Openlungbiopsy • Start corticosteroidtherapy

28. M. Tuberculosisgrew on culture of postbronchoscopicsputum • Antituberculoustherapywasstarted

29. At thebeginning of antitbtherapy 1 monthlater

31. So, what do yourecommend? • Continue Anti-TB therapy • Openlungbiopsy • Re- bronchoscopy • Corticosteroidtherapy • Follow-upwithoutanytherapy

32. Openlungbiopsydecisionwasmade…

33. Mixttypeinflammatorycellinfiltrationandfoamymacrophages in alveolarseptaeandalveolarspaces

34. Eosinophilegatherings

35. Organizingpneumoniaandlambertosis

36. Openlungbiopsy Wedgeresectionfromleftlowerlobeanteriorandlingula: • Polymorphonuclear, lymphocytic, andplasmocyticinflammatorycellinfiltrationalongwitheosinophils in alveolarspaceandseptae • Desquamationfindings in alveolarspaces • Raregianrcellformation • Increase in type II-pneumocytes • Fibrin exudation in somealveolarspaces, organizingpneumoniapattern • Focalfibrosis in alveolarseptae • Focalalveolarbronchiolisation (lambertosis)

37. PATHOLOgycONSuLTAtiON • Fibrosis in septae and subpleuralareas,in some places wedge-style fibrosis • Common Masson bodies with organizing pneumonia • Type-II pneumocyte proliferation and atypical appearance in some places • Focal eosinophilic gatherings in interstitium • Bronchiectasis, constrictive bronchiolitis and bronchiolar metaplasia areas were seen • Foamy histiocytic and pneumonic infiltrations in alveoli of lower lobe anterior and lingula • In a field, granulomawithforeign bodytype giant cells

38. COMMENT: A chronicdiseasewithacuteexacerbations • DIAGNOSIS: Chronicinterstitialpneumonia

39. diagnosis ?? • Chroniceosinophilicpneumonia (CEP) • Criptogenicorganizingpneumonia (COP)

41. Diagnosis CryptogenicOrganizingPneumonia

42. 1st month of corticosteroidtherapy

43. 6th month of corticosteroidtherapy

45. pFT-DLCO • FEV1 : 1.6 (% 82) • FVC : 1.7 (% 70) • FEV1/FVC : % 77 • DLCO : 18 (% 87) • DLCO/VA : 5.8 (%129)

46. After 3 years of follow-upwithouttreatment No relapse