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Dr Prashant Chowbey, MD(Internal Medicine) Dr Mandeep Singh Saini, MD(Respiratory Diseases) A Case Report
<ul><li>NAME BK </li></ul><ul><li>AGE 54 YR </li></ul><ul><li>SEX Female </li></ul><ul><li>OCCUPATION Housewife </li></ul>...
Chief Complaints <ul><li>Fever x 3 months </li></ul><ul><li>Cough x 3 months </li></ul>
History of Present Illness <ul><li>Fever x 3 months </li></ul><ul><li>Low grade </li></ul><ul><li>Intermittent </li></ul><...
History of Present Illness <ul><li>Cough x 3 months </li></ul><ul><li>Progressively increasing  </li></ul><ul><li>Mucoid e...
History of Present Illness contd. <ul><li>Anorexia x 3 month </li></ul><ul><li>Pain and redness in both eyes and decreasin...
<ul><li>History of Past Illness  </li></ul><ul><li>No history of tuberculosis, </li></ul><ul><li>hypertension or diabetes ...
<ul><li>Family History   </li></ul><ul><li>Brother was treated for Pulmonary TB 5 Years back, and cured </li></ul><ul><li>...
General Physical Examination <ul><li>Moderately built and  nourished  </li></ul><ul><li>Conscious, co-operative, well orie...
General Physical Examination  contd . <ul><li>No clubbing </li></ul><ul><li>No significant lymphadenopathy </li></ul><ul><...
Systemic Examinations <ul><li>CHEST:  </li></ul><ul><li>Inspection : normal </li></ul><ul><li>Palpation : normal </li></ul...
<ul><li>CVS  : NAD </li></ul><ul><li>Abdomen  : NAD </li></ul><ul><li>CNS  : NAD </li></ul>
ENT Examination <ul><li>Oral Cavity WNL </li></ul><ul><li>IDL WNL </li></ul><ul><li>Nose Congestion in both nares </li></ul>
Investigations <ul><li>Hb 7.4 gm% </li></ul><ul><li>TLC 22500/cmm </li></ul><ul><li>DLC N 71, L 26, M 01, E 02 </li></ul><...
Investigations  contd. <ul><li>FBS 77 gm% </li></ul><ul><li>LFT </li></ul><ul><li>S.Bilirubin   0.6 mg% Alk. Phosphatase 2...
Investigations  contd. <ul><li>Sputum for AFB (D S)  :3, negative </li></ul><ul><li>ECG   : normal  </li></ul>
Chest x-ray (PA)
Differential Diagnosis <ul><li>Wegener Granulomatous </li></ul><ul><li>Septic Emboli </li></ul><ul><li>Pulmonary Tuberculo...
Special Investigations <ul><li>cANCA Positive </li></ul><ul><li>CECT Thorax </li></ul><ul><li>X-ray PNS </li></ul><ul><li>...
CECT Thorax
 
X-ray PNS
Histopathology
 
<ul><li>Inflammation and necrosis of blood vessels </li></ul><ul><li>Granulomatous  </li></ul><ul><li>Vasculitis </li></ul>
Final Diagnosis <ul><li>Wegner Granulomatosis </li></ul>
A Discussion
Wegener Granulomatosis, a discussion <ul><li>Originally described by Klinger in 1931 </li></ul><ul><li>Wegener described i...
Classical Wegener’s Granulomatosis <ul><li>It is characterized by </li></ul><ul><li>necrotizing granulomas in the respirat...
Incidence and Prevalence <ul><li>Uncommon disease  </li></ul><ul><li>Unknown incidence-approx. 5-12 new cases per million ...
Histopathology <ul><li>Hallmarks  </li></ul><ul><ul><li>necrotizing vasculitis  </li></ul></ul><ul><ul><ul><li>small arter...
Pathogenesis <ul><li>Unclear </li></ul><ul><li>Significant increase in HLA-DR2 </li></ul><ul><li>Indications that depositi...
ANCA <ul><li>In 1985 van der Voude et al reported an association of Wegner Granulomatosis with antineutrophil cytoplasm an...
ANCA <ul><li>It shows sensitivity of 65% and specificity of 77% with positive predictive value of test is 45% </li></ul><u...
Histopathology <ul><li>Endobronchial disease:  </li></ul><ul><ul><li>active form  </li></ul></ul><ul><ul><li>fibrous scarr...
Clinical Features <ul><li>Diverse </li></ul><ul><li>Patient may present in initially to practitioners from almost any bran...
Manifestation Percent at Disease Onset Percent Throughout Course of Disease Kidney Glomerulonephritis 18 77 Ear/Nose/Throa...
Differential Diagnosis <ul><li>Polyarteritis </li></ul><ul><li>SLE </li></ul><ul><li>Churg Strass syndrome </li></ul><ul><...
Investigation  <ul><li>cANCA </li></ul><ul><li>Biopsy of the involved organs mostly upper respiratory tract and lung </li>...
Treatment <ul><li>Cyclophosphamide (2mg/Kg/d) orally for 1 year after remission </li></ul><ul><li>Prednisolone (1mg/Kg/d) ...
Prognosis <ul><li>Without treatment the disease is fatal within 2 years after diagnosis  </li></ul><ul><li>90% are improve...
Follow Up
Thank You
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  1. 1. Dr Prashant Chowbey, MD(Internal Medicine) Dr Mandeep Singh Saini, MD(Respiratory Diseases) A Case Report
  2. 2. <ul><li>NAME BK </li></ul><ul><li>AGE 54 YR </li></ul><ul><li>SEX Female </li></ul><ul><li>OCCUPATION Housewife </li></ul><ul><li>RESIDENCE Vidisha </li></ul><ul><li>ADMISSION 23/11/2007 </li></ul><ul><li>DISCHARGE 09/12/2007 </li></ul><ul><li>Presently on follow up. </li></ul>
  3. 3. Chief Complaints <ul><li>Fever x 3 months </li></ul><ul><li>Cough x 3 months </li></ul>
  4. 4. History of Present Illness <ul><li>Fever x 3 months </li></ul><ul><li>Low grade </li></ul><ul><li>Intermittent </li></ul><ul><li>No diurnal variations </li></ul><ul><li>No rigors or chills </li></ul>
  5. 5. History of Present Illness <ul><li>Cough x 3 months </li></ul><ul><li>Progressively increasing </li></ul><ul><li>Mucoid expectoration approx. 50ml/day </li></ul><ul><li>No diurnal variation </li></ul><ul><li>Hemoptysis x 1 ½ months </li></ul><ul><li>blood tinged sputum </li></ul>
  6. 6. History of Present Illness contd. <ul><li>Anorexia x 3 month </li></ul><ul><li>Pain and redness in both eyes and decreasing vision for last 1month </li></ul><ul><li>Epistaxis x 4 days </li></ul><ul><li>few drops only </li></ul><ul><li>Loss of weight </li></ul><ul><li>No history of nausea/vomiting </li></ul><ul><li>No history of urinary/bowel trouble </li></ul>
  7. 7. <ul><li>History of Past Illness </li></ul><ul><li>No history of tuberculosis, </li></ul><ul><li>hypertension or diabetes mellitus </li></ul><ul><li>Personal History </li></ul><ul><li>Non smoker, non alcoholic, vegetarian </li></ul><ul><li>Married and has 2 children </li></ul>
  8. 8. <ul><li>Family History </li></ul><ul><li>Brother was treated for Pulmonary TB 5 Years back, and cured </li></ul><ul><li>Treatment History </li></ul><ul><li>She was taking ATT and some cough syrup for 1 month before admission with no improvement </li></ul>
  9. 9. General Physical Examination <ul><li>Moderately built and nourished </li></ul><ul><li>Conscious, co-operative, well oriented to time, person and place </li></ul><ul><li>Multiple hypo pigmented rounded lesions on lower half of the face </li></ul><ul><li>Anaemic </li></ul><ul><li>Redness of both eyes with thick discharge </li></ul><ul><li>No cyanosis </li></ul>
  10. 10. General Physical Examination contd . <ul><li>No clubbing </li></ul><ul><li>No significant lymphadenopathy </li></ul><ul><li>No pedal edema </li></ul><ul><li>JVP not raised </li></ul><ul><li>Pulse 78/minute </li></ul><ul><li>Blood Pressure 110/76 mmHg </li></ul><ul><li>Resp. Rate 14/minute </li></ul>
  11. 11. Systemic Examinations <ul><li>CHEST: </li></ul><ul><li>Inspection : normal </li></ul><ul><li>Palpation : normal </li></ul><ul><li>Percussion : normal </li></ul><ul><li>Auscultation : Crepitations in the left infraclavicular area </li></ul>
  12. 12. <ul><li>CVS : NAD </li></ul><ul><li>Abdomen : NAD </li></ul><ul><li>CNS : NAD </li></ul>
  13. 13. ENT Examination <ul><li>Oral Cavity WNL </li></ul><ul><li>IDL WNL </li></ul><ul><li>Nose Congestion in both nares </li></ul>
  14. 14. Investigations <ul><li>Hb 7.4 gm% </li></ul><ul><li>TLC 22500/cmm </li></ul><ul><li>DLC N 71, L 26, M 01, E 02 </li></ul><ul><li>S.Cr 0.9 mg% </li></ul><ul><li>B.Urea 24 mg% </li></ul><ul><li>Na + 142 meq/l </li></ul><ul><li>K + 4.8 meq/l </li></ul><ul><li>ESR 65 mm/hr </li></ul>
  15. 15. Investigations contd. <ul><li>FBS 77 gm% </li></ul><ul><li>LFT </li></ul><ul><li>S.Bilirubin 0.6 mg% Alk. Phosphatase 206 IU/L AST 49 IU/L ALT 90 IU/L Total Proteins 6.6 g% Albumin 3.8 g% </li></ul><ul><li>Urine Routine normal </li></ul><ul><li>RA factor positive </li></ul>
  16. 16. Investigations contd. <ul><li>Sputum for AFB (D S) :3, negative </li></ul><ul><li>ECG : normal </li></ul>
  17. 17. Chest x-ray (PA)
  18. 18. Differential Diagnosis <ul><li>Wegener Granulomatous </li></ul><ul><li>Septic Emboli </li></ul><ul><li>Pulmonary Tuberculosis </li></ul><ul><li>Ca lung - primary/secondary </li></ul>
  19. 19. Special Investigations <ul><li>cANCA Positive </li></ul><ul><li>CECT Thorax </li></ul><ul><li>X-ray PNS </li></ul><ul><li>Fiber Optic Bronchoscopy </li></ul><ul><ul><li>Trans Bronchial Lung Biopsy </li></ul></ul>
  20. 20. CECT Thorax
  21. 22. X-ray PNS
  22. 23. Histopathology
  23. 25. <ul><li>Inflammation and necrosis of blood vessels </li></ul><ul><li>Granulomatous </li></ul><ul><li>Vasculitis </li></ul>
  24. 26. Final Diagnosis <ul><li>Wegner Granulomatosis </li></ul>
  25. 27. A Discussion
  26. 28. Wegener Granulomatosis, a discussion <ul><li>Originally described by Klinger in 1931 </li></ul><ul><li>Wegener described its different clinical and pathological entity in 1936 </li></ul><ul><li>It represents a necrotizing granulomatous vasculitis involving the upper and lower airways, often associated with glomerulo- nephritis </li></ul><ul><li>Only upper and lower airway involvement in 28% of patients </li></ul><ul><li>Pathologically, a triad of necrosis, granulomatous inflammation, and small vessel vasculitis is seen </li></ul>
  27. 29. Classical Wegener’s Granulomatosis <ul><li>It is characterized by </li></ul><ul><li>necrotizing granulomas in the respiratory tract </li></ul><ul><li>generalized focal necrotizing vasculitis </li></ul><ul><li>focal necrotizing glomerulonephritis </li></ul><ul><li>When one element of triad is absent it is usually the renal or upper respiratory tract component </li></ul>
  28. 30. Incidence and Prevalence <ul><li>Uncommon disease </li></ul><ul><li>Unknown incidence-approx. 5-12 new cases per million population per annum (Carruthers DM et al’1996) </li></ul><ul><li>Male to female ratio is 1:1 (some series show a little male predominance 3:2) </li></ul><ul><li>Mean age of onset is approximately 40 years </li></ul><ul><li>15 percent of patients are less than 19 years of age </li></ul><ul><li>Very rare before adolescence </li></ul>
  29. 31. Histopathology <ul><li>Hallmarks </li></ul><ul><ul><li>necrotizing vasculitis </li></ul></ul><ul><ul><ul><li>small arteries and veins together </li></ul></ul></ul><ul><ul><li>granulomatous formation </li></ul></ul><ul><ul><ul><li>intravascular/extravascular </li></ul></ul></ul><ul><li>Lung involvements </li></ul><ul><ul><li>nodular cavitary infiltrates </li></ul></ul><ul><ul><ul><li>multiple </li></ul></ul></ul><ul><ul><ul><li>bilateral </li></ul></ul></ul><ul><li>On biopsy: necrotizing granulomatous vasculitis </li></ul>
  30. 32. Pathogenesis <ul><li>Unclear </li></ul><ul><li>Significant increase in HLA-DR2 </li></ul><ul><li>Indications that deposition of circulating immune complexes in vessel walls initiates the vasculitis </li></ul><ul><li>The immune complexes can </li></ul><ul><ul><li>trigger the complement cascade </li></ul></ul><ul><ul><li>cause granuloma formation </li></ul></ul>
  31. 33. ANCA <ul><li>In 1985 van der Voude et al reported an association of Wegner Granulomatosis with antineutrophil cytoplasm antibodies (ANCA) in blood </li></ul><ul><li>ANCA is also positive in common conditions as </li></ul><ul><ul><ul><ul><li>TB </li></ul></ul></ul></ul><ul><ul><ul><ul><li>lung cancer </li></ul></ul></ul></ul><ul><ul><ul><ul><li>pulmonary emboli </li></ul></ul></ul></ul><ul><ul><ul><ul><li>pulmonary fibrosis </li></ul></ul></ul></ul><ul><ul><ul><ul><li>HIV infection </li></ul></ul></ul></ul><ul><ul><ul><ul><li>other autoimmune diseases </li></ul></ul></ul></ul>
  32. 34. ANCA <ul><li>It shows sensitivity of 65% and specificity of 77% with positive predictive value of test is 45% </li></ul><ul><li>cANCA is more sensitive </li></ul>
  33. 35. Histopathology <ul><li>Endobronchial disease: </li></ul><ul><ul><li>active form </li></ul></ul><ul><ul><li>fibrous scarring </li></ul></ul><ul><ul><li>leads to obstruction with atelactasis </li></ul></ul><ul><li>Upper airway lesions reveal </li></ul><ul><ul><li>inflammation </li></ul></ul><ul><ul><li>Necrosis </li></ul></ul><ul><ul><li>granuloma formation </li></ul></ul><ul><ul><li>+/- vasculitis </li></ul></ul><ul><li>Renal involvement is seen as focal or segmental glomerulitis that may evolve into a RPGN </li></ul>
  34. 36. Clinical Features <ul><li>Diverse </li></ul><ul><li>Patient may present in initially to practitioners from almost any branch of medicine </li></ul><ul><li>Vary from a very acute systemic illness to a chronic illness </li></ul><ul><li>NIH series shows that at presentation </li></ul><ul><ul><li>most patients had evidence of upper airway involvement </li></ul></ul><ul><ul><li>fewer than half had lung involvement, although this developed in almost all eventually </li></ul></ul><ul><ul><li>uncommon but developed in almost of 80% within 2 years </li></ul></ul>
  35. 37. Manifestation Percent at Disease Onset Percent Throughout Course of Disease Kidney Glomerulonephritis 18 77 Ear/Nose/Throat Sinusitis Nasal disease Otitis media Hearing loss Subglottic stenosis Ear pain Oral lesion 73 51 36 25 14 1 9 3 92 85 68 44 42 16 14 10 Lung Pulmonary infiltrates Pulmonary nodules Hemoptysis Pleuritis 45 25 24 12 10 85 66 58 30 28 Eyes Conjunctivitis Dacrocystitis Scleritis Proptosis Eye pain Visual loss Retinal lesions Corneal lesions Iritis 5 1 6 2 3 0 0 0 0 18 18 16 15 11 8 4 1 2 Others Arthralgia/arthritis Fever Cough Skin abnormalities Weight loss Peripheral neuropathy CNS disease Pericarditis Hyperthyroidism 32 23 19 13 15 1 1 2 1 67 50 46 46 35 15 8 6 3
  36. 38. Differential Diagnosis <ul><li>Polyarteritis </li></ul><ul><li>SLE </li></ul><ul><li>Churg Strass syndrome </li></ul><ul><li>Sarciodosis </li></ul><ul><li>Infectious granulomatoses </li></ul><ul><li>Nasopharyngeal lymphoma </li></ul><ul><li>Pulmonary Hodgkin’s disease </li></ul><ul><li>Goodpasture syndrome </li></ul><ul><li>Polychondritis </li></ul><ul><li>Behcet’s disease </li></ul>
  37. 39. Investigation <ul><li>cANCA </li></ul><ul><li>Biopsy of the involved organs mostly upper respiratory tract and lung </li></ul><ul><li>Renal biopsy </li></ul><ul><li>Radiology (sinuses, lungs) </li></ul>
  38. 40. Treatment <ul><li>Cyclophosphamide (2mg/Kg/d) orally for 1 year after remission </li></ul><ul><li>Prednisolone (1mg/Kg/d) orally for 1 month followed by on alternate days and to be tapered off in 6 months </li></ul><ul><li>Co-trimoxazole </li></ul><ul><li>Hydration </li></ul>
  39. 41. Prognosis <ul><li>Without treatment the disease is fatal within 2 years after diagnosis </li></ul><ul><li>90% are improved with treatment </li></ul><ul><li>75% get full remission </li></ul><ul><li>Half of the patients who achieved full remission relapse in one or more times </li></ul><ul><li>Most of the patients who relapsed achieve remission with treatment </li></ul>
  40. 42. Follow Up
  41. 43. Thank You

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