learning from mistakes – lessons from the masters case based discussion - dr sukumar mukherjee
TRANSCRIPT
Learning from Mistakes - Lesson from the Masters
Case Study
IRACON 25th Nov,2016
Dr.Sukumar MukherjeeMD FRCP(London) FRCP ( Edin) FSMF FICP
Ex-Prof and HOD of Medical College Kolkata
Disclosures
None
References • Kasper D , Hauser S , Jameson J C : Harrisons’ Principles of
Internal Medicine 19th Edition Vol 2 P 1716-1719
• Mc Graith E , Barber C : CMAJ , Nov 2010 , Bilateral Plural Effusion
• Hochberg MC , Silman A , Smolen J et al : Rheumatology Fourth ed.2008 , P 1287-88 , P 1535-36
• 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 -022-023 , Feb 2014
Mistakes are the STEPPING STONES to Learning
“All men make mistakes but only wise men learn from their mistakes”
Sir Winston Churchill
Despite Significant Progress In Rheumatology
ImmunologyMolecular & Cellular BiologyNewer Diagnostic & assessment toolsTissue characterizationNewer Biologics
However, confusion , consensus or discordance in decision making – still a
ground reality !
Theme
Changing Goal Post in Clinical Decision
Case Vignette – Phase 1 • RS 72 F; Chronology of events:
• 2009 : Late onset chronic bronchial asthma on intermittent steroid inhalation . Non Diabetic , Normotensive & euthyroid.
• 2012 : 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
Case Contd…• July -August 2015 : Emperical standard ATD
started along with steroids on and from 13th Aug and continued till 6th 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.
• Jan 2016 : Diagnosis recurrent bilateral pleural effusion – unresolved
Imaging chest : Pleural EffusionMar-April 2015
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 .
Chest Xray in Jan 2016
Bilateral Pleural Effusion Jan 2016
CT Scan Chest Jan 2016
AutoantibodiesJan 2016
Autoantibodies Results ANF (Hep 2) 1/640 CentromereDS-DNA (Crinthdia) 1/10 +veC3 143.9C4 52Anti CCPAb -veRheumatoid Factor -veAnti U1RNP -veAnti Sclero 70 -veAntinucleosome -veAnti SM -veAnti RO -ve
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
• Still not quite right about the diagnosis ?
However SLE may be a possibility (SLICC Criteria)
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
Paranasal Sinusitis
June-July 2016
Pleural effusionJune – July 2016
CT ScanJune-July 2016
Test ResultHb 10.2WBC 8200/cummESR 80mmCRP 2.54Platelet 2.3AN7(Hep2) 1/640Centromere +veDS-DNA (Crithidia) -veC-ANCA (PR3)IgG >100 An/mlP-ANCA (MPO)IgG -ve (4.2An/ml)Urine Normal
• Again utter confusion or consensus about the diagnosis ?
• Pt declined to go for pleural biopsy or sinus endoscopic tissue biopsy
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
DCVAS
CXR Oct 2016
• 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
• Randa YE , Arrayhani M et al , C-ANCA in Systemic Lupus Erythematosus : An overlapping Syndrome ? , African JMCR , Vol 2 , P -022-023 , Feb 2014
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
• So the diagnosis ? ANCA associated with limited non
renal GPA Or
SLE and GPA overlap syndrome ?with
comorbidities
Acknowledgement
• My Patients (RS)• Dr Somnath Bhar ,MRCP• Ms Pampita Chakraborty , PhD
Fellow • Mr Amarnath Mukherjee
Questions for Vote • Pleural fluid sugar is higher than 60ml/dL in the
following conditions except A.Active Rheumatoid Arthritis B.Systemic Lupus ErythematosusC.Parapneumonic effusion
Which type of ANCA is commonly associated with SLE and Vasculopathy ?
A. C-ANCA (PR3)B. Atypical ANCAC. P-ANCA(MPO)
Which drug is inappropriate in the treatment of index patient
A. MycophenolateB. MethotrexateC. CorticosteroidsD. Cyclophosphamide