Kinder Vasculitis Flashcards
Common Presentations of Vasculitis
Constitutional
- Fever, weight loss, fatigue, and weakness
Musculoskeletal
- Arthralgias, arthritis, and myalgias
Cutaneous
- Palpable purpura, nodules, urticaria, livedo reticularis, phlebitis, and ischemia
Pulmonary
- Hemorrhage, nodules, infiltration, and cavities
Renal
- Nephritis, infarction, and hypertension
Neurologic
- Cephalgia, mononeuritis multiplex, and stroke
Lab
- Anemia, increased ESR, abnormal LFT, and hematuria
clinical manifestations of large vessel vaculitis
limb claudication asymmetric blood pressure absence of pulses bruits aortic dilation
clinical manifestations of medium vessel vasculitis
cutaneous nodules ulcers arthritis livedo reticularis digital gangrene mononeuritis multiplex microanerysms
clinical manifestations of small vessel vasculitis
purpura vesiculobullous lesions urticaria glomerulonephritis alveolar hemorrhage arthritis cutaneous extravascular necrotizing granulomas splinter hemorrhages uveitis, episcleritis, or scleritis
Giant Cell Arteritis and Polymyalgia Rheumatica
Almost all patients with Giant Cell Arteritis (temporal arteritis) are older than 50 years with average age of 72 years
Women are twice as likely as men to develop the disorder
Most common in whites or Americans of Scandinavian origin
Patients with PMR only are not at risk of losing vision and require low doses of prednisone (under 20 mg/day)
Patients with GCA are at risk for losing vision and require higher doses of prednisone (≥ 40 mg/day) to prevent blindness
Giant Cell Arteritis Presenting Manifestations- in order of frequency
ESR anemia headache jaw claudication constitutional symptoms polymyalgia rheumatica visual loss abnormal temporal artery arthritis
Giant Cell Arteritis Physical Examination
Temporal artery may be enlarged, difficult to compress, nodular, or pulseless
15-20% have axillary or subclavian disease
- Diminished pulses
- Unequal arm blood pressures
- Bruits above or below the clavicle
Aortic regurgitation
Giant Cell Arteritis main symptoms
Headache
- Dull, aching, and localized over the temporal area
- New or different headache
- Tenderness of the scalp, when combing or brushing hair
Jaw Claudication
- Pain in masseter muscle when chewing
- Most specific symptom
Polymyalgia Rheumatica
- Pain and stiffness in the neck, shoulders, and hip-girdle that are much worse in the morning
Pollymyalgia Rheumatica
Shoulders (70-95%) Hips (50-70%) Morning stiffness Pain is in the muscles Difficulty getting out of bed, rising, brushing hair or teeth Swelling, erythema, and heat are absent
goes with GIant Cell Arteritis
Diagnostic Criteria for Polymyalgia Rheumatica
Criteria of Healey
Pain persisting for at least 1 month and involving 2 of the following areas
Neck, shoulders, and pelvic girdle
Morning stiffness lasting > 1 hour
Rapid response to prednisone (20 mg/day or less)
Absence of other diseases causing musculoskeletal symptoms
Age older than 50 years
ESR > 40 mm/hr
Giant Cell Arteritis Visual Symptoms
1/3 of Giant Cell Arteritis Patients
Diplopia or visual loss
Visual loss can be monocular, binocular, transient, or permanent
Can occur abruptly
Often proceeded by episodes of blurred vision, or amaurosis fugax
On average develops 5 months after onset of GCA
Visual loss usually secondary to occlusion of the posterior ciliary artery, a branch of the ophthalmic artery
Giant Cell Arteritis Atypical Manifestations
Fever of unknown origin
Cough
Mononeuritis multiplex
- Shoulder
Large Artery Disease
- Aorta, vertebral, carotid, subclavian, and axillary arteries
- Symptoms
- – TIA, stroke, hand ischemia, arm or leg claudication
- Thoracic aortic aneurysm
- – Aortic regurgitation
- – MI
- – Dissection
Giant Cell Arteritis labs
Elevated ESR, often around 100 mm/h in Giant Cell Arteritis
Mild anemia
Giant Cell Arteritis Treatment
Prednisone 40-60 mg po q day
- Start tapering after first month by 10% every 1-2 weeks until down to 15 mg po q day, then decrease by 1 mg every 2 weeks Most patients need to be on steroids for 1-2 years
Methylprednisolone 1000 mg IV/day for 3-5 days if experiencing visual loss
Temporal Artery Biopsy – don’t delay treatment to obtain biopsy
Takayasu Arteritis
Large Vessel Arteritis
- Aorta and its major branches
Affects women during reproductive years (8:1 Women)
Presents with absent pulse, bruit, claudication, hypertension, or fever of unknown origin
ESR is usually elevated
Responds to Prednisone
Granulomatous inflammation affecting all levels of the vessel
Takayasu Arteritis Clinical Findings: Vascular Manifestations
Bruits - Carotids, supraclavicular, infraclavicular, arms, and abdomen Claudication – upper extremity Hypertension Light-headedness Unequal Blood pressures in extremeties Carotidynia Aortic Regurgitation – 20% of patients Loss of pulse
Takayasu Arteritis Clincal Findings: Constitutional and Musculoskeletal Symptoms
50% of patients Asthenia Weight loss Fever Myalgia Arthralgia Thoracic back pain – inflammation of the aorta
how to distinguish Takayasu from Giant Cell?
age. Young- takayaus
old- Giant Cell
Takayasu Arteritis labs and imaging
Laboratory
Elevated ESR and CRP in 80% of patients
Anemia 50% of patients
Thrombocytosis 33% of patients
Imaging: MRI CT Ultrasound Angiography
Takayasu Arteritis Diagnosis
Onset at age under 40 years Limb Claudication Decreased brachial artery pulse Unequal arm blood pressures (> 10 mm Hg) Subclavian or aortic bruit Angiographic evidence of narrowing or occlusion of the aorta or its primary branches, or large limb arteritis
Three of six criteria is 91% sensitive and 98% specific for Takayasu Arteritis
Takayasu Arteritis Treatment
Prednisone
Granulomatosis with Polyangiitis
Pathology with granulomatous inflammation, vasculitis, and necrosis
Clinical Features: - Fatigue, myalgias, weight loss, and fevers * Persistent upper respiratory tract and ear “infections” that do not respond to antibiotics - Orbital pseudotumors - Migratory arthritis - Nodular or cavitary lung lesions - Rapidly progressive glomerulonephritis ANCA
Granulomatosis with Polyangiitis- who and what affected
Small to medium sized blood vessels
Arterial and venous circulations
Male and female equally affected
Mean age of onset is 50 years
Granulomatosis with Polyangiitis Clinical Findings
Nose, Sinuses, and Ears
- 90% have nasal involvement
- Rhinorrhea, nasal obstruction, epistaxis
- Cartilagenous inflammation leads to septal perforation and “saddle-nose” deformity
- Conductive and sensorineural hearing loss
Eyes
- Orbital pseudotumors
- Scleritis, episcleritis, and conjunctivitis
Mouth
- “Strawberry gums” and Mouth Ulcers – both are painful
Trachea
- Subglottic stenosis
Lungs
- Pulmonary Lesions in 80% of patients
- Cough, hemoptysis, dyspnea, and pleuritic chest pain
- Pulmonary infiltrates and nodules on chest x-ray
- Cavitary lesions
Renal
- Up to 80% of patients
- Rapidly progressive glomerulonephritis
- Hematuria, red cell casts, and proteinuria
- Pauci-immune
Joints
- Migratory Arthritis
Biopsy – granulomatous inflammation, vasculitis, and necrosis
- Lung
- Kidney
- Upper Respiratory Tract
C-ANCA/PR3-ANCA
Granulomatosis with Polyangiitis Treatment
Severe Disease
Rituximab or Cyclophosphamide + Glucocorticoids
Limited Disease
Methotrexate + Glucocorticoids
