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MSK II Exam 2 > Kinder Vasculitis > Flashcards

Kinder Vasculitis Flashcards

1
Q

Common Presentations of Vasculitis

A

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

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2
Q

clinical manifestations of large vessel vaculitis

A 
limb claudication
asymmetric blood pressure
absence of pulses
bruits
aortic dilation
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3
Q

clinical manifestations of medium vessel vasculitis

A 
cutaneous nodules
ulcers
arthritis
livedo reticularis
digital gangrene
mononeuritis multiplex
microanerysms
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4
Q

clinical manifestations of small vessel vasculitis

A 
purpura
vesiculobullous lesions
urticaria
glomerulonephritis
alveolar hemorrhage
arthritis
cutaneous extravascular necrotizing granulomas
splinter hemorrhages
uveitis, episcleritis, or scleritis
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5
Q

Giant Cell Arteritis and Polymyalgia Rheumatica

A

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

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6
Q

Giant Cell Arteritis Presenting Manifestations- in order of frequency

A 
ESR
anemia
headache
jaw claudication
constitutional symptoms
polymyalgia rheumatica
visual loss
abnormal temporal artery
arthritis
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7
Q

Giant Cell Arteritis Physical Examination

A

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

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8
Q

Giant Cell Arteritis main symptoms

A

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

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9
Q

Pollymyalgia Rheumatica

A 
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

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10
Q

Diagnostic Criteria for Polymyalgia Rheumatica

A

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

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11
Q

Giant Cell Arteritis Visual Symptoms

A

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

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12
Q

Giant Cell Arteritis Atypical Manifestations

A

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
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13
Q

Giant Cell Arteritis labs

A

Elevated ESR, often around 100 mm/h in Giant Cell Arteritis

Mild anemia

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14
Q

Giant Cell Arteritis Treatment

A

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

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15
Q

Takayasu Arteritis

A

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

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16
Q

Takayasu Arteritis Clinical Findings: Vascular Manifestations

A 
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
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17
Q

Takayasu Arteritis Clincal Findings: Constitutional and Musculoskeletal Symptoms

A 
50% of patients
Asthenia
Weight loss
Fever
Myalgia
Arthralgia
Thoracic back pain – inflammation of the aorta
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18
Q

how to distinguish Takayasu from Giant Cell?

A

age. Young- takayaus

old- Giant Cell

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19
Q

Takayasu Arteritis labs and imaging

A

Laboratory
Elevated ESR and CRP in 80% of patients
Anemia 50% of patients
Thrombocytosis 33% of patients

Imaging:
MRI
CT
Ultrasound
Angiography
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20
Q

Takayasu Arteritis Diagnosis

A 
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

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21
Q

Takayasu Arteritis Treatment

A

Prednisone

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22
Q

Granulomatosis with Polyangiitis

A

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
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23
Q

Granulomatosis with Polyangiitis- who and what affected

A

Small to medium sized blood vessels
Arterial and venous circulations
Male and female equally affected
Mean age of onset is 50 years

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24
Q

Granulomatosis with Polyangiitis Clinical Findings

A

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

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25
Granulomatosis with Polyangiitis Treatment
Severe Disease Rituximab or Cyclophosphamide + Glucocorticoids Limited Disease Methotrexate + Glucocorticoids
26
Microscopic Polyangiitis
Most common cause of pulmonary-renal syndrome of alveolar hemorrhage and glomerulonephritis Combination of two or more of the following - Fatigue, myalgias, weight loss, and fevers - Migratory arthralgias or arthritis, either pauciarticular or polyarticular - Palpable purpura, sometimes with skin ulcerations - Sensorimotor mononeuritis multiplex - Alveolar hemorrhage associated with hemoptysis and respiratory compromise - Glomerulonephritis – pauci-immune ANCA (MPO-ANCA)
27
Microscopic Polyangiitis- who and what?
``` Necrotizing vasculitis Small blood vessels (capillaries, arterioles, or venules) Tropism for kidneys and lungs Mean age of diagnosis 60 years Male/Female 1:1 ```
28
Microscopic Polyangiitis major symptoms
``` Glomerulonephritis 80% - Pauci-immune Weight loss 70% Mononeuritis multiplex 60% Fevers 55% Cutaneous vasculitis 60 % Alveolar Hemorrhage 12% ```
29
Microscopic Polyangiitis- organs
No upper respiratory manifestations like Granulomatous Polyangiitis Lungs - Capillaritis with alveolar hemorrhage Kidneys - 80% of patients have renal involvment - Proteinuria, microscopic hematuria, and red cell casts Nervous System - Distal, asymmetric, axonal polyneuropathy (mononeuritis multiplex)
30
Microscopic Polyangiitis- skin
Small Vessel: Palpable purpura Vesiculobullous lesions Splinter hemorrhages ``` Medium Vessel: Nodules Ulcers Livedo reticularis Digital gangrene ```
31
Microscopic Polyangiitis Treatment
Rituximab + Glucocorticoids | Cyclophosphamide + Glucocorticoids
32
Eosinophilic Granulomatosis with Polyangiitis
Asthma, eosinophilia, and systemic vasculitis Clinical Features - Allergic rhinitis and nasal polyposis - Reactive airway disease - Peripheral eosinophilia (10-60% of Leukocytes) - Fleeting pulmonary infiltrates and occasional pulmonary hemorrhage - Vasculitis neuropathy - Congestive Heart Failure P-ANCA in 50%
33
Eosinophilic Granulomatosis with Polyangiitis phases
Prodrome Phase - Asthma and allergic rhinitis often lasts for several years Eosinophilia/Tissue Infiltration Phase - Peripheral eosinophilia - Tissue infiltration of heart, lung, gastrointestinal tract, and other tissues Vasculitis Phase - Heart, lungs, peripheral nerves, and skin
34
Eosinophilic Granulomatosis with Polyangiitis Clinical Findings
Lungs - Asthma in 90% of patients - Eosinophilic infiltration in early disease - Necrotizing vasculitis - Granuloma Peripheral Nerves - Vasculitis neuropathy in 77% of patients - Foot drop or wrist drop Heart - CHF Kidneys - Less likely to cause end-stage renal disease Skin: Palpable purpura, Papules, Ulcers, Vesiculobullous lesions, Nodular skin lesions –, “Churg-Strauss granuloma”, Splinter hemorrhage, Digital ischemia, Gangrene of digits
35
Eosinophilic Granulomatosis with Polyangiitis labs
Eosinophilia up to 60% or white blood cell count Elevated serum IgE Elevated ESR and CRP P-ANCA elevated in 50%
36
Eosinophilic Granulomatosis with Polyangiitis Treatment
``` Glucocorticoids Cyclophosphamide Azathioprine Mycophenolate mofetil Methotrexate ```
37
Polyarteritis Nodosa
Subacute onset - Fever, weight loss, malaise, and arthralgias - Lower extremity nodules and ulcerations - Mononeuritis multiplex - Intestinal angina - -- Postprandial pain secondary to mesenteric vessel disease Cutaneous polyarteritis nodosa -Variant in which vasculitis is limited to the skin presents as nodules that break down to ulcers
38
Polyarteritis Nodosa dx
Angiogram or biopsy of an involved organ required for diagnosis - May reveal microaneurysms in the kidneys or GI tract - Biopsies of the skin and peripheral nerves (with sampling of adjacent muscle) are the least invasive way of confirming the diagnosis Necrotizing inflammation of muscular arterioles and medium-sized arteries - Spares capillaries and no venous involvement - No glomerulonephritis Renovascular Hypertension and Renal infarctions - Spares lungs -No granuloma formation - Men and women equally affected
39
Polyarteritis Nodosa Clinical Manifestations
Pain - Myalgias, arthritis, peripheral nerve infarction, testicular ischemia, or mesenteric vasculitis Skin and Joints - Livedo racemosa - Nodules, papules, and ulcerations - Digital ischemia leading to gangrene - Arthralgias of knees, ankles, elbows, and wrists occur in 50% of patients Mononeuritis multiplex - 60% of patients - Most commonly in sural, peroneal, radial, and ulnar nerves - First symptoms may be a foot or wrist drop Gastrointestinal Tract - Postprandial abdominal pain (”intestinal angina”) - Mesenteric infarction - Aneurysmal rupture - Mesenteric angiography demonstrates multiple microaneurysms Intraparenchymal Renal Inflammation - 40% of patients - Renal and interlobar arteries - Microaneurysms in the kidney or wedge shaped infarctions - Renin mediated hypertension
40
Polyarteritis Nodosa labs
Biopsy - Skin, symptomatic nerve, or muscles - Nodules, papules, and edges of ulcers Angiography - Segmental necrosis leads to aneurysm formation - Microaneurysms in mesenteric and renal vasculature Labs - Hepatitis B associated with minority of cases
41
Polyarteritis Nodosa Treatment
Glucocorticoids Cyclophosphamide HBV Associated Disease - Prednisone for two weeks followed by antiviral treatment
42
Mixed Cryoglobulinemia
Small and medium sized vessel involvement Cryoglobulins - Immunoglobulins that precipitate from the serum at low temperatures Affected Organs - Skin – most common - Joints, peripheral nerves, and kidneys Rheumatoid Factor Positive in most patients 90% of cases associated with Hepatitis C 33% of patients with Sjögren Syndrome
43
Mixed Cryoglobulinemia Diagnosis
Compatible clinical syndrome accompanied by cutaneous vasculitis Isolation of cryoglobulins from the serum Antibodies to Hepatitis C Biopsies as necessary to exclude other diagnoses
44
Mixed Cryoglobulinemia Clinical Findings
Skin - Small vessel disease - Leukocytoclastic vasculitis - Palpable purpura - Predilection for lower extremities Rheumatologic - Arthralgias - Raynaud phenomenon - Acrocyanosis Peripheral Nerve - Sensory neuropathy prior to motor mononeuritis multiplex
45
Mixed Cryoglobulinemia Labs
Cryglobulins Cryocrit - Centrifugation of serum at 4℃. Hypocomplementemia Rheumatoid Factor Positive Anti-HCV Antibodies and Quantification of HCV RNA
46
Hypersensitivity Vasculitis
Small-vessel vasculitis of the skin Leukocytoclastic angiitis Etiologies - Medications - Infections - 40% unknown Must rule out other known causes of vasculitis Most cases are self-limited, but glucocorticoids are required in some cases
47
Hypersensitivity Vasculitis Diagnosis
``` Age at onset > 16 years Medication at disease onset Palpable purpura Maculopapular rash Biopsy including arteriole and venule, showing granulocytes in a perivascular or extravascular location ```
48
Hypersensitivity Vasculitis Clinical Manifestations
Skin - Purpura, papules, urticaria/angioedema, erythema multiforme, vesicles, pustules, ulcers, and necrosis - Lower extremity and buttocks most common Joints - Arthralgias Labs - Elevated ESR or CRP in less than 50% of patients - Normal Tests CBC, Electrolytes, LFTs, UA, ANA, RF, ANCA, C3, C4, Hep B, Hep C, Cryoglobulins, and chest x-ray
49
Hypersensitivity Vasculitis Treatment
Mild - Leg elevation, NSAIDs, and antihistamines Persistent Disease - Colchicine - Hydroxychloroquine - Dapsone Refractory - Glucocorticoids - May need addition of immunosuppressive such as azathioprine
50
Behçet Disease
Recurrent attack of painful oral apthous ulcers, painful genital ulcers, uveitis, and skin lesions Young adults, aged 25-35 years Prevalent in parts of Asia and Europe Blindness, central nervous system disease, and large-vessel events are the most serious complications Treatment Glucocorticoids, immunosuppressive drugs, or both
51
Behçet Disease Clinical Manifestations
``` Oral ulcers- 100% genital ulcers skin lesions arthritis GI disease thrombophlebitis CNS disease epididymitis ```
52
Behçet Disease- skin, eyes, GI
Skin Lesions - Erythema nodosum * Pathergy - -- Phenomenon of developing an aseptic nodule or ulcer 24-48 hours following a sterile needle prick to the forearm Ocular Inflammation - Panuveitis - Hallmark, appears early in course - Leads to visual loss GI - Ulcers of the bowel can lead to pain, anorexia, rectal bleeding, vomiting, and diarrhea
53
Behçet Disease other findings
Arthritis CNS - Meningitis and meningoencephalitis Large Vessel Vasculitis - Pulmonary, carotid, aortic, iliac, femoral, and popliteal arteries - -- Occlusion, aneurysm, swelling, or rupture - Peripheral thrombophlebitis Labs No specific lab findings
54
Behçet Disease diagnosis
recurrent oral ulceration- 3x over 12 months Plus TWO of the following: Recurrent genital ulceration ocular lesions (uveitis/ retinal vasculitis) Skin lesions ( Positive pathergy test
55
Behçet Disease Treatment
Recurrent oral ulcers - Topical steroids or dapsone Vision threatening uveitis or meningoencephalitis - High dose systemic steroids and anti-TNF inhibitor
56
Henoch-Schönlein Purpura
Nonthrombocytopenic purpura, caused by inflammation of blood vessels in the superficial dermis Leukocytoclastic vasculitis and deposition of IgA in the walls of the involved vessels Tetrad – not all 4 required for diagnosis - Purpura - Arthritis - Glomerulonephritis - Abdominal pain 90% in children
57
Henoch-Schönlein Purpura presentation/ path
Disease follows an upper respiratory infection by 10 days in 2/3 patients Skin pathology shows a leukocytoclastic vasculitis of small blood vessels in the superficial dermis - Necrosis is often present - No granulomas - Immunofluorescent staining shows coarse, granular IgA deposition
58
Henoch-Schönlein Purpura Classic presentation and dx confirmation
Classic presentation - Acute onset of fever - Palpable purpura on the lower extremities and buttocks - Abdominal pain, GI bleeding in 33% - Arthritis - Hematuria Diagnosis confirmed by - Direct immunofluorescence as well as conventional staining of biopsy specimens - In children, treatment can be based on clinical presentation alone
59
Henoch-Schönlein Purpura Treatment
Dapsone High Dose IV steroids + Azathioprine or Cyclophosphamide for severe nephritis Most cases self-limited and resolve in 6-8 weeks, especially in children
60
Primary Angiitis of the CNS
Common presentation - Headaches - Encephalopathy - Multiple Strokes Angiographic abnormalities are suggestive, but not specific Diagnosis requires brain biopsy Treatment - glucocorticoids
61
Secondary Vasculitis of the CNS
``` HIV Herpes zoster Hepatitis CBehçet Disease Polyarteritis nodosa Cocaine ANCA Vasculitis ```
62
Buerger Disease (Thromboangiitis Obliterans)
Typically young male smokers – mean age 40 years Moderate to heavy tobacco use Severe digital ischemia Angiography with segmental involvement of medium-sized arteries with abrupt vascular cut-offs and corkscrew collaterals Major vessel involvement occurs at the ankles and wrist
63
Buerger Disease Clinical Findings and treatment
Extremities - Predilection for the feet and toes - 60% of patients have an abnormal Allen Test - Painful digits with gangrene, tissue loss, and autoamputation Skin - Superficial thrombophlebitis - Splinter hemorrhages - Gangrene of distal tissues (toes and fingers) Treatment - Smoking cessation

MSK II Exam 2 (16 decks)

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