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Vasculitis Overview

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Vasculitis Overview Vasculitis Overview Presentation Transcript

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