Learning from Mistakes - Lesson from the Masters. Dr.Sukumar Mukherjee

Transcription

1 IRACON 25 th Nov,2016 Learning from Mistakes - Lesson from the Masters Case Study Dr.Sukumar Mukherjee MD FRCP(London) FRCP ( Edin) FSMF FICP Ex-Prof and HOD of Medical College Kolkata

2 Disclosures None

3 References Kasper D, Hauser S, Jameson J C : Harrisons Principles of Internal Medicine 19 th Edition Vol 2 P Mc Graith E, Barber C : CMAJ, Nov 2010, Bilateral Plural Effusion Hochberg MC, Silman A, Smolen J et al : Rheumatology Fourth ed.2008, P , P D Sen, David Isenberg,ANCA in SLE, Lupus 2003, Vol 12 ;651 New Diagnostic and Classification criteria of ANCA associated Vasculitis (DCVAS), ACR Annual Meeting, Washington 2016 Randa YE, Arrayhani M et al, C-ANCA in SLE : An overlapping Syndrome?, African JMCR, Vol 2, P , Feb 2014

4 Mistakes are the STEPPING STONES to Learning All men make mistakes but only wise men learn from their mistakes Sir Winston Churchill

5 Despite Significant Progress In Rheumatology Immunology Molecular & Cellular Biology Newer Diagnostic & assessment tools Tissue characterization Newer Biologics However, confusion, consensus or discordance in decision making still a ground reality!

6 Theme Changing Goal Post in Clinical Decision

7 Case Vignette Phase 1 RS 72 F; Chronology of events: 2009 : Late onset chronic bronchial asthma on intermittent steroid inhalation. Non Diabetic, Normotensive & euthyroid : Bilateral TKR. April 2015 : Worsening dry cough with SOBE. No fever, haemoptysis or wt loss May-June 2015 : bilateral pleural effusion diagnosed. Screened for heart failure,chronic hepatic, renal,thyroid and malignant disease. Pleural fluid straw coloured lymphocytic exudate, normal sugar raised protein(3 grms/dl) LDH ( 704 ) ADA 36. Negative microbiology and malignant cells. Hb 11.5, ESR 70, CRP 9.2, TB Gold negative, TST 10 mm, ANF 1/80 Positive, other autoantibodies negative

8 Case Contd July -August 2015 : Emperical standard ATD started along with steroids on and from 13 th Aug and continued till 6 th Feb 2016 Oct 2015 : Pt responded well and steroid withdrwan Nov 2015 : Recurrence of bilateral pleural effusion more on right. No fever no wt loss, Dec 2015 : retapped pleural effusion and found to have Lymphocytic exudate with elevated protein and LDH.Pleural fluid for ANF and Gene Xpert were not available. ATD continued & steroid restarted.

9 Imaging chest : Pleural Effusion Mar-April 2015

10 Case Contd... Phase 2 Jan2016 : Recurrence of cough with SOBE, mild dysphonia, no fever, myalgia, arthralga, synovitis, uveitis, skin rash. oral ulcers Marginal wt loss. Chest xray shows pleural effusion again.

11 Chest Xray in Jan 2016

12 Bilateral Pleural Effusion Jan 2016

13 CT Scan Chest Jan 2016

14 Autoantibodies Jan 2016 Autoantibodies Results ANF (Hep 2) 1/640 Centromere DS-DNA (Crinthdia) 1/10 +ve C C4 52 Anti CCPAb -ve Rheumatoid Factor -ve Anti U1RNP -ve Anti Sclero 70 -ve Antinucleosome -ve Anti SM -ve Anti RO -ve

15 Case Contd Comorbidities : Osteoporosis (Tscore : -2.8), LAHB, Low Vit D. A presumptive diagnosis of SLE with recurrent pleural effusion was made and Omnacotril 30 mg/d with HCQ 400 mg /d was initiated She remained well Feb 2016 : admitted in Mumbai Hosp with aggravation of cough, SOBE without fever, reaspiration of pleural fluid was done and the nature of fluid was suggestive of lymphocytic exudate. No CVD, CLD or renal ds were found. Discharged on Omnacortil, HCQ and Antibiotics April 2016 : Nonbloody thick nasal discharge, CXR showed Encysted pleral effusion and left pleural thickening, treated with antibiotics and anti allergics

16 Still not quite right about the diagnosis? However SLE may be a possibility (SLICC Criteria)

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18 Case Contd Phase 3 June-July 2016 : recurrence of cough,sobe and thick nasal discharge Now she has been found to have hearing loss with left sided conductive deafness Xray PNS Bilateral pan sinusitis CT Scan PNS bilateral maxillary and sphenoidal sinusitis, bilateral nasal spur

19 Paranasal Sinusitis June-July 2016 Pleural effusion June July 2016

20 CT Scan June-July 2016

21 Test Result Hb 10.2 WBC 8200/cumm ESR 80mm CRP 2.54 Platelet 2.3 AN7(Hep2) 1/640Centromere +ve DS-DNA (Crithidia) -ve C-ANCA (PR3)IgG >100 An/ml P-ANCA (MPO)IgG -ve (4.2An/ml) Urine Normal

22 Again utter confusion or consensus about the diagnosis? Pt declined to go for pleural biopsy or sinus endoscopic tissue biopsy

23 DCVAS Revised classification of GPA based on scoring ACR Annual Meeting, November 2016 Clinical Laboratory Nasal Discharge -3 Abnormal CXR -2 Nasal polyp -4 CANCA-5 Cartilage Involvement -2 PANCA 1 Hearing loss-1 Biopsy - 3 Red eyes -1 Eosinophil 1X10⁹ - 3 Summation score more than 5 strongly suggestive of GPA

24 CXR Oct 2016

25 Limited expressions of GPA occur, especially disease confined to the upper or lower respiratory tract, or the eye. These pts may have no identifiable evidence of systemic vasculitis, but when they exhibit clinical and pathologic changes identical to those seen in GPA respiratory tract involvement, and especially if they are ANCA positive, they should be included in the GPA category - CHCC 2012

26 Randa YE, Arrayhani M et al, C-ANCA in Systemic Lupus Erythematosus : An overlapping Syndrome?, African JMCR, Vol 2, P , Feb 2014

27 Summary Points Elderly lady with symptomatic recurrent pleural effusion with exclusion of infection, CVD, CLD, CKD and malignancy Increased inflammatory markers.treated initially with standard ATD Persistent and high titre positive ANF with variable DS-DNA New Development of nasal discharge and left conductive deafness Recurrent pleural effusion with out lung nodules or cavitation on imaging Presence of significantly positive C- ANCA and negative P ANCA Normal urinary findings

28 So the diagnosis? ANCA associated with limited non renal GPA Or SLE and GPA overlap syndrome? with comorbidities

29 Acknowledgement My Patients (RS) Dr Somnath Bhar,MRCP Ms Pampita Chakraborty, PhD Fellow Mr Amarnath Mukherjee

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31 Questions for Vote Pleural fluid sugar is higher than 60ml/dL in the following conditions except A.Active Rheumatoid Arthritis B.Systemic Lupus Erythematosus C.Parapneumonic effusion

32 Which type of ANCA is commonly associated with SLE and Vasculopathy? A. C-ANCA (PR3) B. Atypical ANCA C. P-ANCA(MPO)

33 Which drug is inappropriate in the treatment of index patient A. Mycophenolate B. Methotrexate C. Corticosteroids D. Cyclophosphamide