Vasculitis Syndromes
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Transcript of Vasculitis Syndromes
Vasculitis Syndromes
Polymyalgia Rheumatica,Giant Cell Arteritis, Wegener’s Granulomatosis,
Polyarteritis Nodosa
What is Vasculitis?
• Disease characterized by inflammation of blood vessel walls, leading to altered blood flow through obstructed walls. This causes ischemia and tissue damage.
• In addition there is an intense inflammatory rxn causing further systemic signs and symptoms
• Can be fatal
You Should Suspect Vasculitis • Unexplained signs and sxs• Multisystem disease• Unexplained elevated ESR/CRP• Skin lesions (palpable purpura)• Ischemic vascular changes (Raynaud’s, gangrene, livedo, claudication)• Glomerulonephritis• Mononeuritis multiplex• Intestinal angina• Inflammatory ocular diease• Arthalgias/arthritis, myalgias• Sudden visual loss/headache
Select Vasculitides
• Polymyalgia Rheumatica
• Giant Cell or Temporal Arteritis
• Wegener’s Granulamatosis
• Polyarteritis Nodosa
Polymyalgia Rheumatica (PMR)
• Most ‘benign‘ of the group• Common: 50/100,000, age > 50, average age
75. Highest prevalence in northern European ancestry, females>>males
• Cause unknown
PMR Clinical Presentation
• Usually abrupt onset• Intense morning stiffness and pain that can last all
day involving the shoulders and hip girdle area• No small joint involvement• Muscle strength normal• Fatigue and anorexia common• Elevated CRP and ESR; anemia of chronic disease,
elevated platelets• 15% get GCA (more later)
PMR Treatment
• Low Dose Steroids (10-20 mg/day)– The only drug that works– Look to normalize the CRP and ESR; if they
continue to be elevated, rethink the dx (?paraneoplastic syndrome or GCA)
– Usually self-limited: 65% of patients able to taper off Prednisone by 1 year, >85% in 2 years
– Disease flares not uncommon as prednisone is tapered and may require dose adjustments
Giant Cell Arteritis
• Can occur exclusively but often seen with PMR• Rare: 15/100,000• Age >50• Cause unknown• Involves the medium/large blood vessels of
the head and neck including the blood vessels that supply the optic nerve
GCA Pathophysiology
• Unknown trigger causes inflammatory response with the release of IL-1 and IL-6.
• This leads to systemic symptoms and the infiltration of inflammatory cells into the adventitia of the temporal and other involved arteries
• Typical histologic pattern: Giant Cells
GCA Clinical Presentation• Variable• Scalp tenderness• Temporal headaches• Jaw Claudication• Sudden loss of vision• +/- PMR sxs• Rare- upper extremity claudication due to subclavian
involvement• Constitutional sxs: FUO, wt loss, fatigue• Bounding OR absent temporal artery pulses• Rarely subclavian bruits
GCA Diagnostic Studies
• Temporal Artery Biopsy is the gold standard• Elevated ESR and CRP, usually levels higher
than in PMR• Anemia • Elevated LFTs not uncommon
Treatment of GCA
• High dose Steroids (60 mg/day) is the only drug that works
• Slow taper over time usually 1-2 years. Some patients require low dose (<10 mg/day) chronically
GCA Complications
• Blindness • Scalp Necrosis• Lingual Infarction• Aortic Dissection/Aneurysm
• Complications from high dose steroids: osteoporosis, cataracts, elevated blood sugars, wt. gain etc.
Wegener’s Granulomatosis (WG)
• Potentially fatal vasculitis involving small vessels
• Rare: 3-14/million, more common in whites, any age but rare in children
• Pathology shows necrotizing granulomas usually in upper airways, lungs and kidneys
WG Pathophysiology
• Complex immunopathogenic events in which the production and activity of ANCAs (usually c-ANCA) play a central role. These autoantibodies interact with primed neutrophils to cause vascular injury and necrosis.
• Histologic lesions show granulomas
WG Clinical Presentation• Variable, multisystem involvement• Organs:
– Eyes: episcleritis/scleritis, proptosis due retro-orbital mass– CNS: rare mass lesion– Upper airway: otitis media, nasal chondritis, sinusitis with purulent
drainage and epistaxis, ulcerations, subglottic stenosis– Kidney: neprotic syndrome, proteinuria, renal failure– Skin: palpable purpura due to leukocytoclastic vasculitis, pyoderma
gangrenosum, panniculitis– Lung: cough, hemoptysis, hemorrhage, resp failure– Cardiac: pericarditis, conduction abnormalities– Systemic: fever, night sweats, wt loss, fatigue
Palpable Purpura
Eye Involvement
Face Involvement
WG Diagnostic Studies
• Presence of c-ANCA (cytoplasmic staining pattern antineutrophil cytoplasmic antibodies + clinical picture is often enough to make the diagnosis. It is + 80-90% of generalized WG.
• If the c-ANCA is -, tissue biopsy of lung or kidney is recommended.
• “Limited” refers to disease limited to the airways; c-ANCA often is -.
Additional labs
• Elevated CRP and ESR• Anemia, leukocytosis, & thrombocytosis• Elevated Cr• Active urine sediment with red cell casts,
hematuria and proteinuria
WG Clinical Course/Progression
• Prior to immunosuppression therapies, WG was uniformly fatal. Now survival rates almost 90% with aggressive treatment.
• High dose steroids and Cyclophosphamide are cornerstone of therapy. Methotrexate or Azathioprine sometimes used as steroid sparing agents.
Polyarteritis Nodosa (PAN)
• Medium vessel vasculitis• Can be caused by Hep B• 5/million cases• Peak incidence 50’s & 60’s, slightly more
common in males
PAN Pathophysiology
• In Hep B assoc cases immune complexes play significant role
• In non Hep B cases, the pathophysiology is less understood
PAN Clinical Presentation
• Systemic: fever, fatigue, wt loss• Abdominal pain due to mesenteric angina/ischemia• Mononeuritis multiplex• Myalgias/arthalgias/mild arthritis• Hypertension• Skin: livedo reticularis, palpable purpura, fingertip
ulceration, subcutaneous nodules• Testicular pain or tenderness
Livedo reticularis
Subcutaneous Nodules
Complications of PAN
• Chronic renal failure• Bowel perforation• Stroke/cerebral hemorrhage due to HTN• Foot/wrist drop
Wrist Drop
Labs of PAN
• Elevation of acute phase reactants (ESR, CRP etc)
• Absence of ANCA• Elevated transaminases, decreased albumin• +/- Hep B• Urine: proteinuria and hematuria without
casts
Imaging Studies of PAN
• Mesenteric and/or renal angiography is the test of choice
• Biopsies seldom done
Angiogram
PAN Treatment
• High dose steroids and Cyclophosphamide• Methotrexate or Azathioprine is used as
steroid sparing agents later once the disease is controlled
• Treatment for Hep B with antivirals. Sometimes plasma exchange is used to remove immune complexes