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VASCULITIS George R. Mount, CPT USA MC Rheumatology Fellow Walter Reed AMC
Goals <ul><li>Overview </li></ul><ul><li>Clinical patterns </li></ul><ul><li>Diagnostic aids </li></ul><ul><li>Treatment p...
Introduction <ul><li>Inflammation and necrosis of blood vessels </li></ul><ul><li>Occlusion and ischemia </li></ul><ul><li...
Vasculitis: Primary or Secondary? <ul><li>Primary : Vasculitis is the  principal feature  of the disease </li></ul><ul><li...
Classification of Vasculitis <ul><li>No universally accepted classification system </li></ul><ul><ul><li>Vessel size </li>...
Classification of Vasculitis <ul><li>Large Vessel </li></ul><ul><ul><li>Giant cell arteritis </li></ul></ul><ul><ul><li>Ta...
Classification of Vasculitis www.rheumtext.com  – Hochberg et al (eds)
When to Suspect a Vasculitis <ul><li>Unexplained ischemia: </li></ul><ul><ul><li>Claudication, limb ischemia, angina, TIA,...
When to Suspect a Vasculitis <ul><li>Other suggestive features: </li></ul><ul><ul><li>Glomerulonephritis </li></ul></ul><u...
When to Suspect a Vasculitis <ul><li>Systemic illness – must exclude alternative diagnoses: </li></ul><ul><ul><li>Sepsis <...
General Approach to Diagnosis <ul><li>Attempt to exclude other processes </li></ul><ul><li>Consider the age, gender, ethni...
Demographic Associations
Clues to Diagnosis
Clues to Diagnosis
Clues to Diagnosis
Clues to Diagnosis
Clues to Diagnosis
Approach to Diagnosis: Labs <ul><li>Determine organ involvement </li></ul><ul><li>Exclude other diseases </li></ul><ul><ul...
Approach to Diagnosis: ANCAs 101 <ul><li>Antibodies directed against neutrophil granule constituents </li></ul><ul><li>c-A...
Approach to Diagnosis: Biopsy <ul><li>Blind biopsy generally low yield </li></ul><ul><ul><li>Less than 20% </li></ul></ul>...
Approach to Diagnosis: Angiogram <ul><li>If biopsy is impractical </li></ul><ul><li>Important in large vessel vasculitis <...
Approach to Diagnosis: Angiogram
Prognosis <ul><li>Untreated is a rapidly progressive, usually fatal, disease </li></ul><ul><li>The prognosis is determined...
Treatment <ul><li>Determined by: </li></ul><ul><ul><li>Type and severity of organ involvement </li></ul></ul><ul><ul><li>R...
Case <ul><li>A previously healthy 22yo male college student had an URI 2 weeks ago, RX with PCN </li></ul><ul><li>He devel...
Case
Case <ul><li>What is the most likely cause of renal disease in this patient: </li></ul><ul><li>A. Lupus nephritis </li></u...
Case <ul><li>What is the most likely cause of renal disease in this patient: </li></ul><ul><li>A. Lupus nephritis </li></u...
Henoch-Schonlein Purpura <ul><li>Small vessels, post capillary venules  </li></ul><ul><li>Palpable purpura, arthralgias, a...
Henoch-Schonlein Purpura <ul><li>GI involvement in 85% </li></ul><ul><ul><li>Severe cramping, pain, nausea, vomiting, blee...
Henoch-Schonlein Purpura <ul><li>Renal involvement (10-50%) </li></ul><ul><ul><li>Renal disease more severe in adults </li...
Henoch-Schonlein Purpura <ul><li>Uncommon </li></ul><ul><ul><li>Testicular involvement </li></ul></ul><ul><ul><li>Pulmonar...
Henoch-Schonlein Purpura <ul><li>Usually single episodes < 4 weeks duration </li></ul><ul><li>40% recurrence rate after pe...
Case <ul><li>50yo woman  presents with 1 week of fever, chills, chest pain, cough, dyspnea and paresthesias in LE   </li><...
Case <ul><li>Lab: WBC 12.8 (N 30%, L 25%, Eos 40%) </li></ul><ul><li>Blood/sputum cultures: negative </li></ul><ul><li>ANA...
Case
Case She is treated with empiric antibiotics over 3 days with no improvement in symptoms. What is the most likely diagnosi...
Case She is treated with empiric antibiotics over 3 days with no improvement in symptoms. What is the most likely diagnosi...
Churg-Strauss Vasculitis <ul><li>Necrotizing, granulomatous vasculitis of small arteries and venules </li></ul><ul><li>Pri...
Churg-Strauss: Criteria- 4/6 <ul><li>Asthma </li></ul><ul><li>Eosinophilia (>10%) </li></ul><ul><li>Mono/ Polyneuropathy <...
Churg-Strauss Vasculitis <ul><li>Asthma precedes vasculitis </li></ul><ul><li>Confusion with Wegener’s </li></ul><ul><ul><...
Churg-Strauss Vasculitis <ul><li>p-ANCA (MPO): 70% </li></ul><ul><li>More responsive to steroids alone </li></ul>
Case <ul><li>A 65yo woman c/o 6 months of malaise, 9lb weight loss, recurrent sinusitis, and a persistent cough. On exam, ...
Case <ul><li>A 65yo woman c/o 6 months of malaise, 9lb weight loss, recurrent sinusitis, and a persistent cough. On exam, ...
Wegener’s Granulomatosis <ul><li>Necrotizing, granulomatous vasculitis small vessels </li></ul><ul><li>Affects persons of ...
Wegener’s Granulomatosis <ul><li>Nasal/ sinus disease destructive </li></ul><ul><li>Renal follows respiratory </li></ul><u...
Wegener’s Granulomatosis <ul><li>Criteria:  2 or more </li></ul><ul><ul><li>Nasal/oral ulcers OR purulent/ bloody discharg...
Wegener’s Granulomatosis <ul><li>c-ANCA </li></ul><ul><ul><li>> 90% + in patients with classic symptoms </li></ul></ul><ul...
Wegener’s Granulomatosis <ul><li>Outcomes: </li></ul>Intervention   Survival None   50% at 5 months Glucocorticoids   50% ...
Distinguishing CSV & WG CSV WG Asthma +++   uncommon Eosinophils +++   occ /modest Atopy +++   uncommon Upp airway destruc...
Case <ul><li>A 29yo woman presents with a 4yr h/o skin ulcers on her lower extremities. A previous punch biopsy showed thr...
Case <ul><li>A 29yo woman presents with a 4yr h/o skin ulcers on her lower extremities. A previous punch biopsy showed thr...
Polyarteritis Nodosa  <ul><li>Primary systemic necrotizing vasculitis </li></ul><ul><ul><li>Small/ medium sized arteries <...
Polyarteritis Nodosa <ul><li>Any age </li></ul><ul><li>Peak years: 30-60 </li></ul><ul><li>M:F Ratio  2:1 </li></ul>
<ul><li>Fever, malaise, weight loss </li></ul><ul><li>Arthritis/ arthralgia (50%) </li></ul><ul><li>Skin lesions  </li></u...
<ul><li>Anemia, leukocytosis, thrombocytosis </li></ul><ul><li>ESR elevation </li></ul><ul><li>Hypocomplementemia (25%) </...
PAN-Diagnosis <ul><li>Biopsy – Symptomatic sites </li></ul><ul><ul><li>Skin, Sural nerve, Muscle, Liver, Testes, Temporal ...
 
PAN- Prognosis &Treatment <ul><li>Untreated - 85% mortality at 5 years </li></ul><ul><li>Treatment - 80% survival at 5 yea...
Case <ul><li>A 32yo Korean woman presents with a 30lb weight loss, low-grade fevers and arthralgias. She notes back pain b...
Case <ul><li>A 32yo Korean woman presents with a 30lb weight loss, low-grade fevers and arthralgias. She notes back pain b...
Takayasu’s Arteritis <ul><li>Large vessel </li></ul><ul><li>Unknown etiology </li></ul><ul><li>Aorta/branches </li></ul><u...
Takayasu’s Arteritis <ul><li>Women in reproductive years </li></ul><ul><ul><li>10X more than men </li></ul></ul><ul><ul><l...
Takayasu’s Arteritis <ul><li>98% have stenotic lesions, 27% aneurysms </li></ul><ul><li>Subclavian & aortic arch most comm...
<ul><li>Arterial stenoses/organ ischemia </li></ul><ul><ul><li>Claudication </li></ul></ul><ul><ul><li>Transient cerebral ...
<ul><li>In the absence of complications (retinopathy, HTN, aortic v. insuff), 15 yr survival 95% </li></ul><ul><li>Most re...
Case <ul><li>A 78yo woman c/o headache for the past 8 days and notes the onset of double vision and blurring, lasting 15mi...
Case <ul><li>A 78yo woman c/o headache for the past 8 days and notes the onset of double vision and blurring, lasting 15mi...
 
Giant Cell Arteritis <ul><li>“ Temporal arteritis” & “Cranial arteritis” </li></ul><ul><li>Large vessel, granulomatous art...
<ul><li>Segmental vessel inflammation </li></ul><ul><li>Multinucleate giant cell, lymphocytic predominance…PMN’s rare </li...
<ul><li>Nonspecific constitutional symptoms- fever,malaise, fatigue </li></ul><ul><li>Headache (> 2/3) </li></ul><ul><li>S...
Giant Cell Arteritis <ul><li>Lab </li></ul><ul><ul><li>ESR – Marked elevation </li></ul></ul><ul><ul><li>CRP elevation </l...
<ul><li>Biopsy </li></ul><ul><ul><li>Vascular involvement not uniform </li></ul></ul><ul><ul><li>Length ?: If normal TA ex...
<ul><li>Prednisone 1mg/kg/d </li></ul><ul><ul><li>Taper 10%/month after Sx/lab resolved </li></ul></ul><ul><ul><li>Slow ta...
<ul><li>30% - relapse with steroid taper, esp prednisone < 20 mg/d </li></ul><ul><li>25% - vertebral compression fractures...
Common Vaculitis Presentations (aka Board Buzz Words…) <ul><li>Oral & genital ulcers = Behcet’s </li></ul><ul><li>Upper/lo...
Common Vaculitis Presentations (aka Board Buzz Words…) <ul><li>Palpable purpura = small vessel leukocytoclastic vasculitis...
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Vasculitis Overview

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Vasculitis Overview

  1. 1. VASCULITIS George R. Mount, CPT USA MC Rheumatology Fellow Walter Reed AMC
  2. 2. Goals <ul><li>Overview </li></ul><ul><li>Clinical patterns </li></ul><ul><li>Diagnostic aids </li></ul><ul><li>Treatment paradigms </li></ul><ul><li>Cases and questions </li></ul>
  3. 3. Introduction <ul><li>Inflammation and necrosis of blood vessels </li></ul><ul><li>Occlusion and ischemia </li></ul><ul><li>Immunologic mechanisms </li></ul><ul><li>Multi-system disease with constitutional symptoms and inflammatory laboratory indices </li></ul>
  4. 4. Vasculitis: Primary or Secondary? <ul><li>Primary : Vasculitis is the principal feature of the disease </li></ul><ul><li>Secondary : Vasculitis is a complication of another disease or toxin (e.g. RA, infection, malignancy) </li></ul>
  5. 5. Classification of Vasculitis <ul><li>No universally accepted classification system </li></ul><ul><ul><li>Vessel size </li></ul></ul><ul><ul><li>Histopathology </li></ul></ul><ul><ul><li>Dominant organ involvement </li></ul></ul><ul><li>Overlap </li></ul>
  6. 6. Classification of Vasculitis <ul><li>Large Vessel </li></ul><ul><ul><li>Giant cell arteritis </li></ul></ul><ul><ul><li>Takayasu’s </li></ul></ul><ul><li>Medium Vessel </li></ul><ul><ul><li>Polyarteritis Nodosa </li></ul></ul><ul><ul><li>Kawasaki’s </li></ul></ul><ul><li>Small Vessel </li></ul><ul><ul><li>Wegener’s, Microscopic polyangitis, Henoch-Scholein purpura, Cryoglobulemic, hypersensitivity </li></ul></ul>
  7. 7. Classification of Vasculitis www.rheumtext.com – Hochberg et al (eds)
  8. 8. When to Suspect a Vasculitis <ul><li>Unexplained ischemia: </li></ul><ul><ul><li>Claudication, limb ischemia, angina, TIA, stroke, mesenteric ischemia, cutaneous ischemia </li></ul></ul><ul><ul><li>Especially in a young individual </li></ul></ul><ul><li>Multiorgan dysfunction: </li></ul><ul><ul><li>Systemically ill patient </li></ul></ul><ul><ul><li>Other suggestive features </li></ul></ul>
  9. 9. When to Suspect a Vasculitis <ul><li>Other suggestive features: </li></ul><ul><ul><li>Glomerulonephritis </li></ul></ul><ul><ul><li>Palpable purpura </li></ul></ul><ul><ul><li>Peripheral neuropathy </li></ul></ul><ul><ul><li>Established autoimmune disease </li></ul></ul>
  10. 10. When to Suspect a Vasculitis <ul><li>Systemic illness – must exclude alternative diagnoses: </li></ul><ul><ul><li>Sepsis </li></ul></ul><ul><ul><li>Drug toxicity </li></ul></ul><ul><ul><li>Malignancy </li></ul></ul><ul><ul><li>Coagulopathy </li></ul></ul>
  11. 11. General Approach to Diagnosis <ul><li>Attempt to exclude other processes </li></ul><ul><li>Consider the age, gender, ethnicity of the patient </li></ul><ul><li>Determine which organ systems are involved </li></ul><ul><li>Estimate the size of the vessels involved </li></ul>
  12. 12. Demographic Associations
  13. 13. Clues to Diagnosis
  14. 14. Clues to Diagnosis
  15. 15. Clues to Diagnosis
  16. 16. Clues to Diagnosis
  17. 17. Clues to Diagnosis
  18. 18. Approach to Diagnosis: Labs <ul><li>Determine organ involvement </li></ul><ul><li>Exclude other diseases </li></ul><ul><ul><li>Routine labs: CBC, BMP, UA, ESR, CRP, LFTs </li></ul></ul><ul><ul><li>Infection w/u: cultures, viral serologies (HBV, HCV, HIV) </li></ul></ul><ul><ul><li>Autoimmune serologies: ANA, RF, ANCAs, ENA, ds DNA, C3/C4 </li></ul></ul><ul><ul><li>Misc: CK, anti-GBM, SPEP, Cryoglobulins </li></ul></ul>
  19. 19. Approach to Diagnosis: ANCAs 101 <ul><li>Antibodies directed against neutrophil granule constituents </li></ul><ul><li>c-ANCA </li></ul><ul><ul><li>Stains cytoplasm (hence “c”) </li></ul></ul><ul><ul><li>Main target antigen: proteinase-3 </li></ul></ul><ul><ul><li>Highly specific (>90%) for Wegener’s </li></ul></ul><ul><li>p-ANCA </li></ul><ul><ul><li>Stains perinuclear (hence “p”) </li></ul></ul><ul><ul><li>Main target antigen: myeloperoxidase </li></ul></ul><ul><ul><li>A/w MPA and Churg-Strauss </li></ul></ul>
  20. 20. Approach to Diagnosis: Biopsy <ul><li>Blind biopsy generally low yield </li></ul><ul><ul><li>Less than 20% </li></ul></ul><ul><li>“ Go where the money is.” </li></ul><ul><ul><li>Approx. 66% success in symptomatic organs </li></ul></ul><ul><ul><li>Examples: Temporal artery biopsy, kidney biopsy, sural nerve biopsy, testicular biopsy </li></ul></ul><ul><li>Vasculitic lesions tend to be focal and segmental </li></ul>
  21. 21. Approach to Diagnosis: Angiogram <ul><li>If biopsy is impractical </li></ul><ul><li>Important in large vessel vasculitis </li></ul><ul><li>Patient with abdominal pain </li></ul><ul><ul><li>Renal or mesenteric vasculitis </li></ul></ul>
  22. 22. Approach to Diagnosis: Angiogram
  23. 23. Prognosis <ul><li>Untreated is a rapidly progressive, usually fatal, disease </li></ul><ul><li>The prognosis is determined by the extent and number of organs involved </li></ul><ul><li>Morbidity and mortality can be prevented if recognized and treated early </li></ul>
  24. 24. Treatment <ul><li>Determined by: </li></ul><ul><ul><li>Type and severity of organ involvement </li></ul></ul><ul><ul><li>Rate of disease progression </li></ul></ul><ul><li>Corticosteroids </li></ul><ul><ul><li>Usually 1mg/kg initially </li></ul></ul><ul><ul><li>Pulse dose corticosteroids </li></ul></ul><ul><li>Immunosuppressive therapy </li></ul><ul><ul><li>Cytoxan, Methotrexate, Imuran </li></ul></ul>
  25. 25. Case <ul><li>A previously healthy 22yo male college student had an URI 2 weeks ago, RX with PCN </li></ul><ul><li>He develops abdominal pain, bilateral ankle pain & swelling with raised purpuric lesions over lower extremities </li></ul><ul><li>Labs: </li></ul><ul><ul><li>creatinine 3.0 mg/dL, BUN 46 mg/dL </li></ul></ul><ul><ul><li>Urinalysis: 4+ proteinuria, 2+ RBC’s, sev. RBC casts/ hpf </li></ul></ul>
  26. 26. Case
  27. 27. Case <ul><li>What is the most likely cause of renal disease in this patient: </li></ul><ul><li>A. Lupus nephritis </li></ul><ul><li>B. Acute post-streptococcal glomerulonephritis </li></ul><ul><li>C. Henoch-Schonlein purpura </li></ul><ul><li>D. Allergic interstitial nephritis </li></ul><ul><li>E. Goodpasture’s syndrome </li></ul>
  28. 28. Case <ul><li>What is the most likely cause of renal disease in this patient: </li></ul><ul><li>A. Lupus nephritis </li></ul><ul><li>B. Acute post-streptococcal glomerulonephritis </li></ul><ul><li>C. Henoch-Schonlein purpura </li></ul><ul><li>D. Allergic interstitial nephritis </li></ul><ul><li>E. Goodpasture’s syndrome </li></ul>
  29. 29. Henoch-Schonlein Purpura <ul><li>Small vessels, post capillary venules </li></ul><ul><li>Palpable purpura, arthralgias, abdominal pain, renal disease </li></ul><ul><li>Males=females </li></ul><ul><li>Mean age 5 yrs. </li></ul><ul><li>Preceding URI in 2/3 (1-3 weeks) </li></ul><ul><li>Tissue deposition of IgA -containing immune complexes (skin, kidneys, bowel) </li></ul>
  30. 30. Henoch-Schonlein Purpura <ul><li>GI involvement in 85% </li></ul><ul><ul><li>Severe cramping, pain, nausea, vomiting, bleeding </li></ul></ul><ul><ul><li>Major hemorrage or intussuseption is an uncommon but life-threatening complication in children </li></ul></ul>
  31. 31. Henoch-Schonlein Purpura <ul><li>Renal involvement (10-50%) </li></ul><ul><ul><li>Renal disease more severe in adults </li></ul></ul><ul><ul><li>Determines prognosis </li></ul></ul><ul><ul><li>Many recover with no therapy </li></ul></ul><ul><ul><li>Asymptomatic hematuria  proteinuria & renal insufficiency (cresentic GN) </li></ul></ul><ul><ul><li>< 0.5% progress to ESRD </li></ul></ul>
  32. 32. Henoch-Schonlein Purpura <ul><li>Uncommon </li></ul><ul><ul><li>Testicular involvement </li></ul></ul><ul><ul><li>Pulmonary hemorrhage </li></ul></ul><ul><ul><li>CNS complications </li></ul></ul>
  33. 33. Henoch-Schonlein Purpura <ul><li>Usually single episodes < 4 weeks duration </li></ul><ul><li>40% recurrence rate after period of wellness </li></ul><ul><li>Treatment </li></ul><ul><ul><li>Supportive measures </li></ul></ul><ul><ul><li>Corticosteroids for GI vasculitis and hemorrhage </li></ul></ul><ul><ul><li>? CS early in nephritis </li></ul></ul>
  34. 34. Case <ul><li>50yo woman presents with 1 week of fever, chills, chest pain, cough, dyspnea and paresthesias in LE </li></ul><ul><li>PMHx: bronchial asthma (7 yrs), allergic rhinitis </li></ul><ul><li>PE: 100 F, HR 98, BP 120/70, RR 16/min </li></ul><ul><ul><li>wheezes/ rhonchi bilaterally </li></ul></ul><ul><ul><li>mild peripheral weakness in LE </li></ul></ul><ul><ul><li>sensory dysesthesia in stocking distribution bilaterally </li></ul></ul>
  35. 35. Case <ul><li>Lab: WBC 12.8 (N 30%, L 25%, Eos 40%) </li></ul><ul><li>Blood/sputum cultures: negative </li></ul><ul><li>ANA neg, p-ANCA + 1:20 </li></ul><ul><li>CXR: patchy bilateral infiltrates </li></ul>
  36. 36. Case
  37. 37. Case She is treated with empiric antibiotics over 3 days with no improvement in symptoms. What is the most likely diagnosis: A. Eosinophilic bronchitis B. Idiopathic hypereosinophilic syndromes C. Churg-Strauss syndrome D. Wegener’s granulomatosis E. Polyarteritis nodosa
  38. 38. Case She is treated with empiric antibiotics over 3 days with no improvement in symptoms. What is the most likely diagnosis: A. Eosinophilic bronchitis B. Idiopathic hypereosinophilic syndromes C. Churg-Strauss syndrome D. Wegener’s granulomatosis E. Polyarteritis nodosa
  39. 39. Churg-Strauss Vasculitis <ul><li>Necrotizing, granulomatous vasculitis of small arteries and venules </li></ul><ul><li>Prior asthma </li></ul><ul><ul><li>Started on leukotriene inhibitors and weaned off steroids </li></ul></ul><ul><li>Allergic rhinitis </li></ul><ul><li>Eosinophilia </li></ul><ul><li>Pulmonary infiltrates </li></ul><ul><li>Intra/extravascular granulomas </li></ul>
  40. 40. Churg-Strauss: Criteria- 4/6 <ul><li>Asthma </li></ul><ul><li>Eosinophilia (>10%) </li></ul><ul><li>Mono/ Polyneuropathy </li></ul><ul><li>Pulmonary Infiltrates – Non-fixed </li></ul><ul><li>Paranasal sinus abnormality </li></ul><ul><li>Extravascular eosinophils </li></ul>
  41. 41. Churg-Strauss Vasculitis <ul><li>Asthma precedes vasculitis </li></ul><ul><li>Confusion with Wegener’s </li></ul><ul><ul><li>Nasal/sinus DZ is NON-destructive </li></ul></ul><ul><ul><li>Pulmonary nodules less common </li></ul></ul>
  42. 42. Churg-Strauss Vasculitis <ul><li>p-ANCA (MPO): 70% </li></ul><ul><li>More responsive to steroids alone </li></ul>
  43. 43. Case <ul><li>A 65yo woman c/o 6 months of malaise, 9lb weight loss, recurrent sinusitis, and a persistent cough. On exam, she is afebrile, the mid-portion of the nasal bridge has a flattened appearance, and both sides of the nasal septum are ulcerated. RF is positive and ESR is 66. </li></ul><ul><li>Which of the following tests would be most helpful in determining the diagnosis: </li></ul><ul><li>A. Nasal septum biopsy </li></ul><ul><li>B. Chest radiograph </li></ul><ul><li>C. Measurement of ANCA antibodies </li></ul><ul><li>D. Sputum culture </li></ul><ul><li>E. Measurement of anti-GBM antibodies </li></ul>
  44. 44. Case <ul><li>A 65yo woman c/o 6 months of malaise, 9lb weight loss, recurrent sinusitis, and a persistent cough. On exam, she is afebrile, the mid-portion of the nasal bridge has a flattened appearance, and both sides of the nasal septum are ulcerated. RF is positive and ESR is 66. </li></ul><ul><li>Which of the following tests would be most helpful in determining the diagnosis: </li></ul><ul><li>A. Nasal septum biopsy </li></ul><ul><li>B. Chest radiograph </li></ul><ul><li>C. Measurement of ANCA antibodies </li></ul><ul><li>D. Sputum culture </li></ul><ul><li>E. Measurement of anti-GBM antibodies </li></ul>
  45. 45. Wegener’s Granulomatosis <ul><li>Necrotizing, granulomatous vasculitis small vessels </li></ul><ul><li>Affects persons of any age </li></ul><ul><li>No significant sex predilection </li></ul><ul><li>Upper and lower respiratory tracts </li></ul><ul><li>Glomerulonephritis </li></ul><ul><li>Frequently vasculitis of other organs </li></ul>
  46. 46. Wegener’s Granulomatosis <ul><li>Nasal/ sinus disease destructive </li></ul><ul><li>Renal follows respiratory </li></ul><ul><ul><li>May progress rapidly </li></ul></ul><ul><li>Non-specific abnormalities </li></ul><ul><ul><li>Conjunctivitis, scleritis, episcleritis </li></ul></ul><ul><ul><li>Proptosis (15%) </li></ul></ul>
  47. 47. Wegener’s Granulomatosis <ul><li>Criteria: 2 or more </li></ul><ul><ul><li>Nasal/oral ulcers OR purulent/ bloody discharge </li></ul></ul><ul><ul><li>Abnormal CXR - nodules, focal infiltrates, cavities </li></ul></ul><ul><ul><li>Abnormal urine sediment (microhematuria, RBC casts) </li></ul></ul><ul><ul><li>Granulomatous inflammation </li></ul></ul>
  48. 48. Wegener’s Granulomatosis <ul><li>c-ANCA </li></ul><ul><ul><li>> 90% + in patients with classic symptoms </li></ul></ul><ul><ul><li>Facilitates clinical DX </li></ul></ul><ul><ul><li>Does not eliminate need for BX </li></ul></ul><ul><ul><li>Not for intensification of therapy </li></ul></ul><ul><li>Open biopsy </li></ul><ul><ul><li>Paranasal, nasal, larynx, lung </li></ul></ul><ul><ul><li>Renal biopsy rarely distinctive enough to be definitive </li></ul></ul>
  49. 49. Wegener’s Granulomatosis <ul><li>Outcomes: </li></ul>Intervention Survival None 50% at 5 months Glucocorticoids 50% at 1 year GCS + Cytoxan 80% at 8 years
  50. 50. Distinguishing CSV & WG CSV WG Asthma +++ uncommon Eosinophils +++ occ /modest Atopy +++ uncommon Upp airway destruction uncommon + Pulmonary nodules occasional ++ Renal failure + ++
  51. 51. Case <ul><li>A 29yo woman presents with a 4yr h/o skin ulcers on her lower extremities. A previous punch biopsy showed thrombotic lesions in small blood vessels of the dermis. Treatment has mostly focused on wound care. On exam the ulcers are noted, as is livedo reticularis, a decreased right hand grip, and a right foot drop. </li></ul><ul><li>Which of the following is the most likely diagnosis: </li></ul><ul><ul><li>Lymphoma, with a paraneoplastic syndrome </li></ul></ul><ul><ul><li>Takayasu’s arteritis </li></ul></ul><ul><ul><li>Systemic lupus erythematosus </li></ul></ul><ul><ul><li>Polyarteritis nodosa </li></ul></ul><ul><ul><li>Kawasaki’s disease </li></ul></ul>
  52. 52. Case <ul><li>A 29yo woman presents with a 4yr h/o skin ulcers on her lower extremities. A previous punch biopsy showed thrombotic lesions in small blood vessels of the dermis. Treatment has mostly focused on wound care. On exam the ulcers are noted, as is livedo reticularis, a decreased right hand grip, and a right foot drop. </li></ul><ul><li>Which of the following is the most likely diagnosis: </li></ul><ul><ul><li>Lymphoma, with a paraneoplastic syndrome </li></ul></ul><ul><ul><li>Takayasu’s arteritis </li></ul></ul><ul><ul><li>Systemic lupus erythematosus </li></ul></ul><ul><ul><li>Polyarteritis nodosa </li></ul></ul><ul><ul><li>Kawasaki’s disease </li></ul></ul>
  53. 53. Polyarteritis Nodosa <ul><li>Primary systemic necrotizing vasculitis </li></ul><ul><ul><li>Small/ medium sized arteries </li></ul></ul><ul><ul><li>Very rarely veins </li></ul></ul><ul><ul><li>Never large elastic arteries </li></ul></ul><ul><ul><li>May be a manifestation of other disease </li></ul></ul><ul><ul><ul><li>RA, Sjogren’s, Hepatitis B or C </li></ul></ul></ul><ul><ul><li>Limited  progressive/ fulminant </li></ul></ul>
  54. 54. Polyarteritis Nodosa <ul><li>Any age </li></ul><ul><li>Peak years: 30-60 </li></ul><ul><li>M:F Ratio 2:1 </li></ul>
  55. 55. <ul><li>Fever, malaise, weight loss </li></ul><ul><li>Arthritis/ arthralgia (50%) </li></ul><ul><li>Skin lesions </li></ul><ul><li>Neurologic (Peripheral > central) (50-70%) </li></ul><ul><li>Renal (70%) </li></ul><ul><li>HTN (25%) </li></ul><ul><li>Cardiac (50%) </li></ul><ul><li>GI (50%) </li></ul><ul><li>“ Classic PAN”- Rare lung disease </li></ul>Polyarteritis Nodosa
  56. 56. <ul><li>Anemia, leukocytosis, thrombocytosis </li></ul><ul><li>ESR elevation </li></ul><ul><li>Hypocomplementemia (25%) </li></ul><ul><li>Hepatitis B SAg (10-54%) </li></ul><ul><li>Hep C Ab: 5% </li></ul><ul><li>p-ANCA (MPO): < 10%, c-ANCA is rare </li></ul>Polyarteritis Nodosa
  57. 57. PAN-Diagnosis <ul><li>Biopsy – Symptomatic sites </li></ul><ul><ul><li>Skin, Sural nerve, Muscle, Liver, Testes, Temporal Artery </li></ul></ul><ul><ul><li>Renal- does not allow differentiation of type of vasculitis (segmental necrotizing GN) </li></ul></ul><ul><li>ANGIO - ABDOMINAL VISCERA </li></ul><ul><ul><li>Evidence of intra-abdominal involvement </li></ul></ul><ul><ul><li>Other involved organs not available for bx </li></ul></ul>
  58. 59. PAN- Prognosis &Treatment <ul><li>Untreated - 85% mortality at 5 years </li></ul><ul><li>Treatment - 80% survival at 5 years </li></ul><ul><ul><li>Corticosteroids </li></ul></ul><ul><ul><li>Cytotoxics </li></ul></ul><ul><li>40% relapse (median 33 months) </li></ul>
  59. 60. Case <ul><li>A 32yo Korean woman presents with a 30lb weight loss, low-grade fevers and arthralgias. She notes back pain between her shoulder blades. She notes pain in her arms with any prolonged activity. Recent labs are notable for a platelet count of 800K and an ESR of 130. On exam, her HR is 100bpm and her BP is 60/40 in both arms. </li></ul><ul><li>What is the next step in her management: </li></ul><ul><ul><li>Hospitalize for further evaluation </li></ul></ul><ul><ul><li>Order blood cultures, get an ANA and RF </li></ul></ul><ul><ul><li>Perform a careful exam, listen for subclavian bruits </li></ul></ul><ul><ul><li>Administer IV saline </li></ul></ul><ul><ul><li>Advise the patient to begin Fe supplementation </li></ul></ul>
  60. 61. Case <ul><li>A 32yo Korean woman presents with a 30lb weight loss, low-grade fevers and arthralgias. She notes back pain between her shoulder blades. She notes pain in her arms with any prolonged activity. Recent labs are notable for a platelet count of 800K and an ESR of 130. On exam, her HR is 100bpm and her BP is 60/40 in both arms. </li></ul><ul><li>What is the next step in her management: </li></ul><ul><ul><li>Hospitalize for further evaluation </li></ul></ul><ul><ul><li>Order blood cultures, get an ANA and RF </li></ul></ul><ul><ul><li>Perform a careful exam, listen for subclavian bruits </li></ul></ul><ul><ul><li>Administer IV saline </li></ul></ul><ul><ul><li>Advise the patient to begin Fe supplementation </li></ul></ul>
  61. 62. Takayasu’s Arteritis <ul><li>Large vessel </li></ul><ul><li>Unknown etiology </li></ul><ul><li>Aorta/branches </li></ul><ul><li>“ Pulseless Disease” </li></ul>
  62. 63. Takayasu’s Arteritis <ul><li>Women in reproductive years </li></ul><ul><ul><li>10X more than men </li></ul></ul><ul><ul><li>Asia, Eastern Europe, Latin America </li></ul></ul><ul><li>Granulomatous Panarteritis </li></ul>
  63. 64. Takayasu’s Arteritis <ul><li>98% have stenotic lesions, 27% aneurysms </li></ul><ul><li>Subclavian & aortic arch most common, 93% </li></ul><ul><li>40- 80% renal artery stenosis </li></ul>
  64. 65. <ul><li>Arterial stenoses/organ ischemia </li></ul><ul><ul><li>Claudication </li></ul></ul><ul><ul><li>Transient cerebral ischemia/ stroke </li></ul></ul><ul><ul><li>Renal artery hypertension </li></ul></ul><ul><ul><li>CHF </li></ul></ul><ul><ul><li>Angina </li></ul></ul><ul><ul><li>MI </li></ul></ul><ul><ul><li>Mesenteric vascular insufficiency </li></ul></ul>Takayasu’s Arteritis
  65. 66. <ul><li>In the absence of complications (retinopathy, HTN, aortic v. insuff), 15 yr survival 95% </li></ul><ul><li>Most respond to steroids alone </li></ul><ul><li>40% will need cytotoxics </li></ul>Takayasu’s Arteritis
  66. 67. Case <ul><li>A 78yo woman c/o headache for the past 8 days and notes the onset of double vision and blurring, lasting 15min before resolving. She has lost 15lb over the past 2 months. </li></ul><ul><li>Which is the best next step in her management: </li></ul><ul><ul><li>Refer her to an ophthalmologist or neuro </li></ul></ul><ul><ul><li>Schedule a temporal artery biopsy </li></ul></ul><ul><ul><li>Test her ESR and schedule a TA biopsy </li></ul></ul><ul><ul><li>Administer sumitriptan </li></ul></ul><ul><ul><li>Administer prednisone, 60mg, immediately and schedule a TA biopsy </li></ul></ul>
  67. 68. Case <ul><li>A 78yo woman c/o headache for the past 8 days and notes the onset of double vision and blurring, lasting 15min before resolving. She has lost 15lb over the past 2 months. </li></ul><ul><li>Which is the best next step in her management: </li></ul><ul><ul><li>Refer her to an ophthalmologist or neuro </li></ul></ul><ul><ul><li>Schedule a temporal artery biopsy </li></ul></ul><ul><ul><li>Test her ESR and schedule a TA biopsy </li></ul></ul><ul><ul><li>Administer sumitriptan </li></ul></ul><ul><ul><li>Administer prednisone, 60mg, immediately and schedule a TA biopsy </li></ul></ul>
  68. 70. Giant Cell Arteritis <ul><li>“ Temporal arteritis” & “Cranial arteritis” </li></ul><ul><li>Large vessel, granulomatous arteritis </li></ul><ul><li>Extracranial vessels (arteries) >>> intracranial </li></ul><ul><li>Aortic arch vessels (10-15%) </li></ul><ul><li>Unknown etiology </li></ul><ul><li>Persons over age 50 (mean 70) </li></ul><ul><li>2-3x more common in women </li></ul>
  69. 71. <ul><li>Segmental vessel inflammation </li></ul><ul><li>Multinucleate giant cell, lymphocytic predominance…PMN’s rare </li></ul><ul><li>Thrombosis </li></ul><ul><li>Giant cells not required for diagnosis </li></ul>Giant Cell Arteritis
  70. 72. <ul><li>Nonspecific constitutional symptoms- fever,malaise, fatigue </li></ul><ul><li>Headache (> 2/3) </li></ul><ul><li>Scalp tenderness +/- nodules </li></ul><ul><li>Temporal artery tenderness </li></ul><ul><li>Visual symptoms (blindness 15%) </li></ul><ul><li>Intermittent claudication (jaw, tongue, extremities) </li></ul><ul><li>Neuropathies/TIA/Stroke (30%) </li></ul><ul><li>Respiratory Tract (10%) </li></ul><ul><li>PMR (40-60%) </li></ul>Giant Cell Arteritis
  71. 73. Giant Cell Arteritis <ul><li>Lab </li></ul><ul><ul><li>ESR – Marked elevation </li></ul></ul><ul><ul><li>CRP elevation </li></ul></ul><ul><ul><li>Anemia </li></ul></ul><ul><ul><li>Thrombocytosis </li></ul></ul><ul><ul><li>Increased LAE </li></ul></ul><ul><ul><li>Increased Alkaline phosphotase </li></ul></ul>
  72. 74. <ul><li>Biopsy </li></ul><ul><ul><li>Vascular involvement not uniform </li></ul></ul><ul><ul><li>Length ?: If normal TA exam, obtain 3-5 cm sample and examine at multiple levels </li></ul></ul><ul><ul><li>Negative biopsy </li></ul></ul><ul><ul><li>Consider contralateral bx if first bx normal </li></ul></ul><ul><ul><li>When ? .….ASAP </li></ul></ul>Giant Cell Arteritis
  73. 75. <ul><li>Prednisone 1mg/kg/d </li></ul><ul><ul><li>Taper 10%/month after Sx/lab resolved </li></ul></ul><ul><ul><li>Slow taper at 15 mg/d </li></ul></ul><ul><ul><li>Lab and Sx </li></ul></ul><ul><ul><li>Long term steroids </li></ul></ul><ul><li>Steroid sparing with MTX </li></ul>Giant Cell Arteritis
  74. 76. <ul><li>30% - relapse with steroid taper, esp prednisone < 20 mg/d </li></ul><ul><li>25% - vertebral compression fractures </li></ul><ul><li>50% - other serious steroid toxicities, e.g. HTN, diabetes, CHF, cataracts….. </li></ul>Giant Cell Arteritis
  75. 77. Common Vaculitis Presentations (aka Board Buzz Words…) <ul><li>Oral & genital ulcers = Behcet’s </li></ul><ul><li>Upper/lower airway disease and glomerulonephritis = Wegener’s </li></ul><ul><ul><li>Septal perforation, epistaxis </li></ul></ul><ul><ul><li>Recurrent sinus infections </li></ul></ul><ul><li>Young female with arm/leg fatigue and HTN = Takayasu’s </li></ul>
  76. 78. Common Vaculitis Presentations (aka Board Buzz Words…) <ul><li>Palpable purpura = small vessel leukocytoclastic vasculitis </li></ul><ul><li>Hepatitis B = PAN </li></ul><ul><li>Hepatitis C = cryoglobulinemia </li></ul>
  77. 79. Questions?

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