Vasculitides Flashcards
Clinical features of Polyarteritis nodosa
Sudden sharp pain in peripheral nerve distribution, later with weakness (foot drop). Cutaneous manifestations: palpable purpura, infarctive ulcers, Livedo reticularis, Digital tip infarctions, Livedo Reticularis, Mononeuritis Multiplex, Cutaneous Nodules
Management of Polyarteritis nodosa
Treatment: High dose steroids (1 mg/kg/day), Immunosuppressives, such as cyclophosphamide or azathioprine, Plasma exchange; Treat viral hepatitis if present.
Polyarteritis nodosa
Skin, peripheral nerves, joints, intestinal tract most commonly involved. Lungs spared. Uncommon- incidence 5 to 10 per million per year, Twice as common in men. ……. Assoc w/ Hep B (more commonly prior to vaccine)
Can affect myriad of organs (GU, abd, ocular), Musculoskeletal – arthralgias»_space; synovitis, “Mononeuritis multiplex” >50%.
Pathology of Hypersensitivity vasculitis (LCV)
Extravasated RBCs, fibrinoid necrosis
Clinical features of Hypersensitivity vasculitis (LCV)
Pts with LCV complain of: itching, burning sensation
pain, asymptomatic lesions …………… Exam: Palpable purpura most common, Lesions usually round 1-3 mm diameter, May coalesce, plaque like, May ulcerate
Management of Hypersensitivity vasculitis (LCV)
Treat the cause (if you can find it!); Generally results in rapid clearing in 2 wks. Treat chronic disease that primarily involves the skin with nontoxic modalities. Try to minimize systemic steroids, Avoid immunosuppressives. Colchicine or dapsone (inhib neutrophil fxn)
Hypersensitivity vasculitis (LCV)
Leukocytoclastic vasculitis (LCV) - histopathologic term denoting small-vessel vasculitis.“Cutaneous vasculitis” “LCV” “Hypersensitivity vasculitis: all terms are used interchangeably. Many possible causes exist for this condition, but a cause is not found in as many as 50% of patients . The most commonly identified cause is a drug reaction ……. Various infections such as Hep C. Collagen Vascular Diseases (RA, Sjogrens, Lupus). Inflammatory Bowel Disease. Malignancy accounts for less than 1% of cases of cutaneous vasculitis.
Cryoglobulinemia
Cryoglobulins – immunoglobulins & complement. Precipitation of blood proteins at temps <37C, Dissolve upon warming – tend to see peripheral involvement
Etiology of Cryoglobulinemia
Chronic immune stimulation, defective clearance. Associated with hepatitis B & C
Clinical features of Cryoglobulinemia
Prognonis is good, 70% at 10 yrs. Severe manifestations:
Glomerulonephritis , tends to be assoc Hep C, GI (abd pain, bleeding), Rapidly progressive neuropathy, CHF
Management of Cryoglobulinemia
Labs: + cryoglobulins, Diminished complement, +RF, Hepatitis B & C, Renal failure, Lymphoproliferative d/o……….
Treatment: tx underlying hepatitis, malignancy, Prednisone (tapered rapidly), Rituxan if severe.
Labs and Imaging of Wegner’s Granulomatosis
Renal: increased Scr, proteinuria, hematuria, red blood cell casts; + c-ANCA PR3 80-90%, most often with active disease; Anemia; Elevated inflammtory markers; Chest CT - nodules and infiltrations that often cavitate
Diagnosis of Wegner’s Granulomatosis
Suspect with upper or lower respiratory tract involvement and nasal/oral ulcerations; PR3 positive c-ANCA; Biopsy of involved tissues - necrotizing granulomatous inflammatory process
Clinical Manifestations of Wegner’s Granulomatosis
Nonspecific findings- fever, malaise, weight loss, arthralgias, myalgias, chronic rhinitis or worsening sinusitis. Pain over sinus areas. ………… Purulent or bloody nasal discharge. Nasal or mucosal ulcerations. Saddle-nose deformity …….. Pulmonary: chest pain, dyspnea, hemoptysis, pulmonary hemorrhage, Tracheal lesions and subglottic involvement -> stenosis. ………. Eye- uveitis, episcleritis, proptosis
Management of Wegner’s Granulomatosis
High dose steroids (1mg/kg or IV 1g/d x3d). Immunosuppressives, such as Cyclophosphamide or Rituximab. Diffuse alveolar hemorrhage – plasmapharesis
Relapses common, maintenance therapy with less toxic drugs (methotrexate or azathioprin)
Giant Cell Arteritis (temporal arteritits)
Granulomatous arteritis of the aorta and its major branches - extracranial branches of the carotid artery; patients > 50 years old, associated polymyalgia rheumatica
Pathology of Giant Cell Arteritis (temporal arteritits)
Relatively common vasculitis: Onset > 50 yrs (avg age ~70 yrs), Male:female ratio: 1:4. Strong assoc PMR (prox pain & stiffness)
Clinical Features of Giant Cell Arteritis (temporal arteritits)
Nonspecific symptoms: fatigue, low-grade fever, weight loss. Headache is the most common symptom (classically over temporal regions), Jaw claudication ……….. Scalp tenderness over arteries, esp Temporal. Impaired vision (most common neurologic sign (40% of cases)); ischemic optic neuritis can result in blindness pale, swollen disc on ophthalmologic exam. [diplopia]. New carotid artery bruits, or bruits over axillary/brachial arteries. Temple Artery Induration
Management of Giant Cell Arteritis (temporal arteritits)
Treatment - high dose steroids (1 mg/kg). Given high stakes, Initiation of steroids should NOT be delayed until biopsy is done. Diagnostic utility of biopsy declines with each day on steroids
Takayasu’s Arteritits
Granulomatous inflammation of the aorta & major branches, patients <40 years old
Pathology of Takayasu’s Arteritits
Similar to GCA in presentation, histopathology, and lg vessel involvement but differs epidemiologically. Most frequent in young Asian women. Proximal aorta and branches most common.
Clinical Manifestations of Takayasu’s Arteritits
Symptoms: dizziness, amaurosis, diplopia, angina (narrowing of coronary ostia), arm claudication, leg claudication, intra-abdominal ischemia (mesenteric arteries), cerebral ischemia (cervical arteries)……….. Physical: Asymmetric blood pressure >10 mm/Hg in nearly all pts. Hypertension (40%)- renal artery stenosis. Bruits- carotid, subclavian, etc. Aortic valve insufficiency (aortic root dilatation).
Management of Takayasu’s Arteritits
Labs: Normochromic anemia, Elevated ESR; EKG- may have ischemia; CXR- may have widening of thoracic aorta; Imaging- Arteriography. ……….. Treatment: prednisone 1 mg/kg/day & immunosuppressive.
Dx of LCV
Age>16 years at disease onset, Medication at disease onset, Palpable Purpura, Maculopapular rash, Biopsy -arterioles and venuoles with granulocytes in perivascular space with extravascular RBCs and fibrinoid necrosis
