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Interstitial Lung Disease
- 1. BY DR. ANIMESH ARYA CONSULTANT PULMONOLOGIST MAX HOSPITAL AND BALAJI ACTION MEDICAL INSTITUTE NEW DELHI
- 2. 58 YRS FEMALEOBESE H/O DOE FOR 2 YRS PROGRESSIVE ADMITTED FOR INVESTIGATION AND BEING TREATED FOR HYPOXIC RESPIRATORY FAILURE
- 14. Lung window shows bilateral reticularity in the right and the left mid zones anterioly ( right>left ) with A haze suggestive of interestitial thickening with interestitial pnuemonitis. Some alveolar opacities are also seen amidst the above suggestive of the alveolar exudates. The apical basal segments of both lower lobes also show reticularity with haze suggestive of interestitial thickening and interestitial pnuemonitis The right lower zone shows an area of rounded cystic air spaces suggestive of areas of bronchiectasis. Some area of bronchiectasis are also seen in the above apical basal patches of pnuemonitis. A tiny emphysematous bulla is seen in the right upper zone peripharally.basal early honeycombing is seen.
- 15. Fob andlavage and TBLB Treatment with steroids Pulse therapy VS. regular dose OLB and the treat /not treat Supportive
- 16. THE LUMEN OF ALVEOLI SHOW DESQUAMMATED EPITHILIAL CELLS THE WALL IS THICKENED EXPANDED AT CERTAIN FOCII BY COLLAGENOUS TISSUE WHILE AT PLACES THERE ARE LYMPHOID AGGREGATES. CERTAIN ALVEOLAR SPACES SHOW PLASMA CELL, LYMPHOCYTES AND FEW HISTOCYTE COLLECTIONS. THE TERMINAL BRONCHIOLES ARE THICKENED, WITH COLLAGENOUS WALL AROUND IT. SECTION B FROM THE MIDDLE LOBE SHOW CERTAIN NORMAL LOOKING ALVEOLI WHILE AT PLACES THE INTERESTITIUM IS EXPANDED LYMPHOMONONUCLEAR CELLS
- 17. FEATURES OFDIP IN THE MIDDLE LOBE AND FEATURES OF DIP AND UIP IN THE LOWER LOBE
- 18. A CRPdiagnosis of UIP made with acute exacerbation Treated with steroids 30 mg /day and azathioprine with oxygen and diuretics and LTOT Uneventful course initially with worsening to resting hypoxia without oxygen and gr. IV dyspnoea
- 19. INCRESED ABDOMINAL FULLNESS, ACIDITY, FREQUENCY OF STOOLS AND SHORTNESS OF BREATH ON WALKING UPTO ABOUT A KM. GR I – II DYSPNOEA in may2006 NO H/O WHEEZING. CHEST PAIN H/OCOUGH, EXPECTORATION,SLIGHTLY YELLOWISH TO HGIC NO PEDAL OEDEMA, NO CHEST PAIN , PND,ORTHOPNOEA NO SIGNIFICANT PAST HISTORY PATIENT IS EX SMOKER – 30 PACK-YEARS TILL ABOUT 6 MONTHS BACK ALCOHOLIC –LEFT SINCE 3 MONTHS
- 20. AS PER RECORD AVAILABLE WITH US GPE/ NAD, S/E NAD PREVIOUS HISTORY- TMT IN MAY 2005- NAD REFERRED TO CHEST SPECIALST
- 21. HB-16.2 TLC-6800, P71,L22,M1,E6, ESR =10MM URINE –NAD BLOOD SUGAR 131 CXR
- 24. FVC=3.52/3.58 (98%) FEV1=2.83/2.88 (98%) FET==6.81 Seconds PEFR=8.08/7.02 lps (115%) FEF 25-75 =2.89/3.14 (92%)
- 30. Non contrast HRCT scan was done Multiple patchy foci of consolidation showing a predominantly peripheral distribution seen at the lung bases bilaterally. The largest lesion is seen in the right lower lobe abutting the pleura with an adjacent area of ground glass density. Other lesions are significantly smaller and are seen in both the lower lobes as well as lingula and the middle lobe B/L peripherally distributed patchy consolidation/infiltrates are also seen No lymphadenopathy Radiological D/D – nonspecific shadows, ?infective, ?BOOP, ?EP,?OP
- 31. WAIT ANDWATCH AS PT. IS MILDLY SYMPTOMATIC? INVESTIGATE? WHICH INVESTIGATIONS?
- 32. CONSULTED ANOTHER CHEST PHYSICIAN AND TREATED WITH AB. FOR A WEEK AND REVIEW AGAIN AND INVESIGATE FOR AFB SMEAR AND CULTURE FOR THREE DAYS, AND SPUTUM CULTURE SP CULTURE NEGATIVE, SP AFB NEG FOR 3 DAYS HIS COUGH WORSENED AND THE SPUTUM WAS MORE BLOOD TINGED HE THEN REPORTED TO US FOR FOB AND BIOPSY WITH INTENT TO RULE OUT MALIGNANCY (SUGGESTED BY SOMEBODY)
- 33. MX TEST, ACE LEVEL ,HIV, TEMP RECORD AND BRONCHOSCOPY FOB PERFORMED MILD PURULENT SECRETIONS COMING OUT OF APICAL AND POSTERIOR BASAL SEGMENTS OF RLL, APICAL SEGMENT WAS NAROWED SENT FOR CYTOLOGY, AFB CULTURES AND TBLB TAKEN FROM THE RLL APICAL SEGMENT
- 34. CYTOLOGY, INCONCLUSIVE AFB SMEAR -NEG BIOPSY- A PIECE OF BRONCHIAL MUCOSA, IT HAS PSEUDOSTRATIFIED LINING DISCONTINUOUS AT PLACES THERE IS SUBMUCOUSAL LYMPHONUCLEAR INFILTRATE, FEW FOAMY CELLS.AND A FEW ILL DEFINED GRANULOMAS IMP: EPITHELOID GRANULOMATOUS INFLAMMATION BRONCHIAL TISSUE - ?CAUSE
- 35. TUBERCULOSIS ALVEOLARSARCOID OP PUMONARY VASULITIS ???? IIP
- 36. PATIENT WAS TOLD OF THE REPORT AND HE DISCUSSED THIS WITH THREE DIFFERENT PULMONOLOGISTS AND THE OPINION WAS VARIED ABOUT 1. ATT, 2. ATT AND STEROIDS 3. STEROIDS 4 .WAIT AND WATCH FOR CULTURE HOWEVER PATIENT WAS STARTED ON ANTITUBERCULAR TREATMENT WITH 4 DRUGS WITH THE CONSENT OF THE PT.
- 39. His symptoms worsened and the patient became dyspnoeic, with worsening hemoptysis and loss of weight His CXR did not show much change , RATHER WORSENED He was suggested to take steroids and discontinue ATT but he again went to at least three more pulmonologists and all agreed to same approach In the mean time to get sputa for AFB for 3 more days, c-ANCA, p-ANCA, ACE levels, complement levels, repeat urine exam and ENT exam All investigations came out to be normal. Meanwhile pt’s dyspnoea worsened. He was suggested repeat CT scan thorax and PFT
- 41. FVC: 2.04 vs 3.52 FEV1: 1.61 vs 2.83
- 51. CT Report (18-8-05): There is marked volume loss of both lower lobes which show areas of collapse consolidatoion in both the lung bases. Evidence of thickened interstitium with ground glass density are also seen. Similar changes are evident in few areas of middle lobe and lingula. A few scattered infiltrates are seen in the upper lobes but these portions of lung are mostly spared. There is no lymph nodes and or pleural effusion There is marked progression of lesions as compared to the previous scan of 30-6-05. No definitive radiological opinion was volunteered.
- 55. Patient was advised to undergo OLB at this stage for a conclusive diagnosis Pt refused procedure and continued on steroids Went to AIIMS and subjected to FOB and TBLB again ON 26/08/05 Biopsy result-shows respiratory epithelium, subepithelium showing mild thickening of the interalveolar septae and sparse chronic inflammatory infiltrate. The possibility of inflammatory lung disease can not be ruled out in the small biopsy.
- 56. REPEAT FOBWITH BAL OPEN LUNG BIOPSY AND MICROBIOLOGICAL SAMPLING CHANGE MX WITH ADDITION OF IMMUNOSUPPRESSIVES
- 57. D/D ?? Continued on steroids and we reemphasised the need for a firm diagnosis by larger biopsy by way of OLB
- 58. Patient undergoes open lung biosy on 14/09/05 at another institute after consulting five pulmonologists in Delhi and Bombay Reported as widening of the interstitial tissue which is infiltrated by the inflammatory cells. Some of the alveolar lumina contain macrophages and chronic inflammatory cells consisting of lymphocytes - Intersitial Pneumonitis of Lung Review of OLB in AIIMS - Compatible with organising pneumonia. No granuloma seen Review at another institution - UIP
- 59. INTERSTITIAL PNEUMONIA VARIETY? UIP NSIP-SUBTYPE= CELLULAR FIBROTIC MIXED -OP-FIBROTIC NSIP
- 60. After OLBpatient was also given azathioprine besides steroids Meanwhile AFB culture on lung biopsy and bronchial aspirate came out to be negative
- 61. WAS THE CLINICAL DIAGNOSIS CORRECT? DID WE MISS AT ANY INVESTIGATION? WAS APPROACH CORRECT? FOB AND BIOPSY WERE INADEQUATE AND CONFUSING OLB DID IT GIVE US THE FINAL ANSWER? VARIANCE OF VARIOUS PATHOLOGISTS IMPRESSION
- 64. NSIP OP-NSIP ?? USUAL INTERSTITIAL PNEUMONITIS ?? UIP WITH COP ?? COP ???? OTHER
- 66. DIFFICULT TO ARRIVE AT A DIAGNOSIS BETTER CLINICAL , RADIOLOGIC AND SURICAL CORELATION IS MANDATORY OPEN LUNG BIOPSY IS THE NEED OF THE HOUR , CAN BE EASILY PERFORMED BUT PERHAPS THE APPROPRIATE INTERPRETATION OF THE FINDINGS IS STILL ELUSIVE AND MAY NOT BE THE GOLD STANDARD
- 67. 48 MALE,NON SMOKER DOE -2MONTHS REVIEWD BY PHYSICIANS NO DIAGNOSIS CXR – BASAL LINEAR SHADOWS LOSS OF LUNG VOLUME EXAM –BIBASILAR FINE CRACKLES ADVISED CT SCAN CHEST AND PFT
- 77. PATCHY GROUNDGLASS LOSS OF LUNG VOLUMES NO THICKENING OF INTERSTITIUM NO HONEY COMB INTERSTITIAL PNEUMONITIS-NSIP PFT – RESTRICTIVE DISORDER
- 78. FOB ANDLAVAGE – INCONCLUSIVE TREATMENT AS CELLULAR NSIP PULSE 1 GM OF METHYLPREDNISOLONE X 3 DAYS AND THEN START ON 10 MG WYSOLONE AND AZATHIOPRINE 50 MG/DAY
- 108. DOE –HIGH INDEX OF SUSPICION MUST R/OOTHER DISEASES A HIGH QUALITY CT-WITH A CR CONF AGOOD PFT WITH DLCO MANDATORY TIME PLANE HIGH PROBABLITY OF IIP ALGORITMIC APPROACH IF DIA CERTAIN NO NEED FOR OLB , FOB COULD BE DONE TO R/O INFECTIONS BAL ROLE IN INDIA IF OLB ATTEMPED SELECT THE SITE NO. SIZE AND GOOD PATHOLOGIST
- 109. TREATMENT AIMAT WHAT POTENTIAL CURE CELLULAR/GG-TREAT /PULSES FIBROTIC –NO GOOD ONLY SUPPORTIVE NEWER DRUGS-NAC /OTHERS