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Pod Med I > 10) Vasculitis, Sjogren's Syndrome, and Vasculopathies > Flashcards

10) Vasculitis, Sjogren's Syndrome, and Vasculopathies Flashcards

1
Q

Sjogren’s Syndrome

A
  • An immune-mediated disorder of the exocrine glands
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2
Q

Exocrine pancreas glands

A
  • Produce secretions needed for the surface of an organ rather than the bloodstream
  • ie: mammary and sweat glands
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3
Q

Sjogren’s Syndrome symptoms

A
  • Classic triad of dry eye (conjunctivitis, xerophthalmia), dry mouth (dysphagia, xerostomia), and arthritis
  • Parotid enlargement, increased risk of B-cell lymphoma
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4
Q

Sjogren’s Syndrome high yield facts

A
  • Predominantly affects women between 40 and 60 years of age
  • Associated with rheumatoid arthritis
  • Sicca syndrome
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5
Q

Sicca syndrome

A
  • Keratoconjuntivitis and xerostomia without connective tissue disease
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6
Q

Sjogren’s syndrome treatment

A
  • Artificial tears for dry eye
  • Sugarless mints, gum for dry mouth
  • NSAIDS, Plaquenil (hydroxychloroquine) for arthritis and myalgias
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7
Q

Common manifestation ofSjögren’s syndrome

A
  • Vasculitisthat is usually manifested as rash or peripheral neuropathy
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8
Q

Vasculitides

A
  • Heterogeneous group of clinical syndromes characterized by inflammation of the blood vessels
  • History and physical examination is highly variable, but usually vasculitis occurs over a few days or a few weeks
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9
Q

Vasculitis high yield facts

A
  • Fever and rash are common
  • Mild synovitis occurs in 20% of patients
  • Arterial pulses may be abnormal
  • Multiple peripheral neuropathies may occur (especially in the lower extremities)
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10
Q

Vasculitis

A
  • Vascular inflammation with inflammatory cell infiltrate and clear-cut vascular damage with fibrinoid necrosis
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11
Q

Vasculitis may be associated with

A
  • Infections
  • Medications
  • Chemicals
  • Foods
  • Connective tissue and other inflammatory diseases
  • Malignancies
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12
Q

Etiologic factors for vasculitis caused by infections

A
  • Strep
  • TB
  • Hepatitis A-C
  • Herpes
  • Influenza
  • Candida
  • M. leprae
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13
Q

Etiologic factors for vasculitis caused by medications

A
  • PCN
  • Sulfonamides
  • Tamoxifen
  • Streptomycin
  • OCPs
  • Thiazides
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14
Q

Etiologic factors for vasculitis caused by chemicals

A
  • Insecticides

- Petroleum products

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15
Q

Etiologic factors for vasculitis caused by foods

A
  • Milk

- Gluten

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16
Q

Etiologic factors for vasculitis caused by connective tissue and other inflammatory diseases

A
  • RA
  • SLE
  • Dermatomyositis
  • Sjogren’s
  • UC
  • CF
  • HIV
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17
Q

Etiologic factors for vasculitis caused by malignancies

A
  • Lymphomas
  • Myeloma
  • Lung CA
  • Colon CA
  • Renal CA
  • Prostate CA
  • Breast CA
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18
Q

Vasculitis is characterized by

A
  • Inflammation and destruction of blood vessels

- Predominant feature or a component of a systemic process

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19
Q

Predominant manifestations of vasculitis are palpable

A
  • Purpura
  • Pitechiae
  • Vesicles
  • Pustules
  • Plaques
  • Ulcers
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20
Q

Vasculitis is distinct from

A
  • The inflammatory process in many chronic wounds
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21
Q

Palpable purpura

A
  • Non-blanchable hemorrhages

- Raised and palpable

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22
Q

Presentation of vasculitis is dependent on

A
  • Size vessels involved (urticarial, palpable purpura, nodules, livedo reticularis, mononeuritis multiplex, necrosis of organs, claudication)
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23
Q

Treatment for vasculitis

A
  • Immune based and underlying cause (ie infection, drug, systemic disease, malignancy)
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24
Q

Diagnostic laboratory studies for infection

A
  • CBC
  • ESR
  • VDRL, HIV, PPD
  • C-reactive protein
  • Gram stain
  • Special stains for AFB, fungus
  • Routine culture
  • AFB, anaerobic, fungal culture
  • Xrays, nuclear med studies, CT, MRI (osteomyelitis, deep abscess, infected prosthesis)
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25
Diagnostic immunologic laboratory studies
- ESR - VDRL - Antinuclear antibodies - Rheumatoid factor - Protein electrophoresis - Immune complex - Complement (CH50, C3, C4) - a-ANCA, p-ANCA (Anti-neutrophil cytoplasmic antibodies) - Hepatitis panel
26
Invasive diagnostic studies for selected patients
- Temporal artery, skin, muscle, sural nerve biopsy - Angiography - Renal biopsy - Lung biopsy
27
Differential diagnosis for vasculitis includes diverse disorders that result in
- Organ ischemia - Localized inflammation - Constitutional symptoms
28
The pattern of expression of a specific vasculitic disorder
- May evolve or change over time independent of therapeutic interventions
29
Drugs of choice used to treat vasculitis
- DMARDS (Disease Modifying Drugs; entire immune system) | - Biologics (target specific steps in the inflammatory process)
30
Other options for treatment of vasculitis
- Colchicine - Dapsone (test G6PD) - Steroids - Imuran - Methotrexate - Cytoxan - Cytophosphamide - Chlorambucil
31
Vasculopathy
- Thrombi within vessels in the absence of inflammation
32
Vasculopathy may be caused by
- Atrophie Blanche | - Thrombotic disorders
33
Thrombotic disorders contributing to vasculopathy
- Protein C - Protein S - Antithrombin III - Cryofibrinogen (malignancy, myeloma, luekemia) - Factor V Leiden - Homocystine - Anticardiolipin - Lupus
34
General metabolic laboratory diagnostic studies for vasculopathy
- Renal (BUN/Cr) - Liver function - Hepatitis panel - Glycemic control (HbA1C) - CV risk (lipids, homocysteine) - Gout (uric acid) - Thyroid fx (myxedema) - Calcium (calcinosis)
35
Hematologic laboratory diagnostic studies for vasculopathy
- CBC - ESR (nonspecific inflammation) - Infection (C-reactive protein) - Coagulopathy (antithrombin III, protein C, S, Factor V, IgG, IgM anticardiolipin antibodies, lupus anticoagulants) - Sickle cell or other hemoglobinopathy (Hemoglobin electrophoresis) - Cryoglobulinemia (cryoglobulins, C2, C4, end organ dysfunction, hep panel)
36
Biopsy or arteriography may confirm diagnosis of
- Vasculitis
37
Antineutrophil cytoplasmic antibody (ANCA) is present in most cases of
- Wegener’s granulomatosis
38
Skin biopsies diagnostic in
- Hypersensitivity vasculitis
39
Forms of vasculitis
- Takayasu’s arteritis - Giant cell (temporal) arteritis - Polyarteritis Nodosa - Wegener’s Granulomatosis - Hypersensitivity vasculitis - Polymyalgia rheumatica
40
Other forms of vasculitis
- Churg-Strauss Syndrome - Henöch-Schönlein purpura - Cryoglobulinemia (Vasculopathy) - Thromboangiitis obliterans
41
Takayasu’s Arteritis
- AKA “pulseless disease” - Thickening of the aortic arch and/or proximal blood vessels, causing weak pulses in upper extremities and ocular disturbances - Primarily affects young Asian females - Fever night sweats myalgia, arthritis, skin nodules
42
Takayasu's Arthritis diagnosis
- Suspect in young women with a history of systemic inflammatory illness, or bruits over large arteries - Arteriography confirms diagnosis
43
Takayasu's Arthritis treatment
- Corticosteroids - “Steroid-sparing’ agents (nonbiologic and biologic DMARDS) - Combination therapy for resistant cases - Endovascular/Vascular bypass procedures/angioplasty
44
Giant Cell (Temporal) Arteritis classification criteria (must have 3/5)
- Age > 50 at disease onset - New headache - Temporal artery abnormality (tender or decreased pulse) - Elevated Westergren ESR > or equal to 50 mm/Hr - Abnromal artery biopsy with mononuclear cell infiltrate, granulomatous infection, usually with multinucleated giant cells
45
Giant Cell (Temporal) Arteritis treatmenr
- Corticosteroids | - Cytotoxic or immunosuppressive agents
46
Polyarteritis Nodosa (PAN)
- Blood vessel disease characterized by inflammation of small and medium-sized arteries (vasculitis) - Prevents them from bringing oxygen and food to organs
47
Most cases of Polyarteritis Nodosa (PAN) occur in
- 4th or 5th decade of life, although it can occur at any age
48
Polyarteritis Nodosa (PAN) may be the first sign of
- TB - Bacterial/fungal infection - Sarcoidosis - IBS - Cancer
49
Polyarteritis Nodosa (PAN) high-yield facts
- Typically involves renal and visceral vessels - Fever, weight loss, malaise, abdominal pain, headache, myalgia, hypertension - Diagnosed by skin biopsy
50
ACR classification criteria for Polyarteritis Nodosa (1-5)
- Weight loss >4 kg - Livedo Reticularis - Testicular pain or tenderness - Myalgias, weakness, or leg tenderness - Mononeuropathy or polyneuropathy
51
ACR classification criteria for Polyarteritis Nodosa (6-10)
- Diastolic BP >90 mmHg - Elevated BUN or creatinine - Hepatitis B virus - Arteriographic abnormality - Biopsy of small or medium artery containing PAN
52
Polyarteritis Nodosa is a systemic vasculitis characterized by
- necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcations
53
Polyarteritis Nodosa is a systemic vasculitis results in
- Microaneurysm formation - Aneurysmal rupture with hemorrhage - Thrombosis - Consequently, organ ischemia or infarction
54
Polyarteritis Nodosa treatment
- Corticosteroids - Azathioprine - Cyclophosphamide - Colchicine - Cyclophosphamide - IV immunoglobulin - Penicillin to prevent flairs of strep tonsillitis or cellulitis
55
Although identical skin lesions are common in systemic PAN, cutaneous PAN should be considered
- A separate disease - Distinguished from systemic PAN - Clinical course and management of these conditions differ from each other
56
Systemic PAN is a vasculitis that causes destructive inflammation of medium-sized muscular arteries of multiple systems including
- Liver - Kidney - Heart - Lung - Gastrointestinal tract - Musculoskeletal - Nervous systems
57
Systemic PAN is a potentially life-threatening form of vasculitis, whereas cutaneous PAN
- Usually runs a chronic but relatively benign course
58
Wegener's Granulomatosis high yield facts
- Focal necrotizing vasculitis and necrotizing granulomas in the lung and upper airway - Necrotizing glomerulonephritis - CXR may reveal large nodular densities - Hematuria
59
Wegener's Granulomatosis is now called
- Granulomatosis with polyangiitis (GPA)
60
ACR classification criteria for Wegener's Granulomatosis (must have 2/4)
- Nasal of oral inflammation (oral ulcers or bloody nasal drainage) - Abnormal chest radiograph (nodules, fixed infiltrates, cavities) - Urinary sediment (>5 RBC/hpf or RBC casts) - Granulomatous inflammation on biopsy (in wall of artery or arteriole, perivascular, or extravascular)
61
Wegener's Granulomatosis treatment
- Corticosteroids - Methotrexate - Cyclophosphamide - Combinations of two antibiotics ( trimethoprim and sulfamethoxazole) - Rituximab (Biologic) - CellCept (mycophenolate) - Others - Team approach
62
Hypersensitivity Vasculitis
- Vasculitis of small vessels, especially arterioles and venules secondary to an immune response to exogenous substances
63
Most common cause of Hypersensitivity Vasculitis
- Reactions to drugs are the most common cause | - Onset is usually abrupt and occurs after exposure to the etiologic agent
64
Most common clinical manifestation of Hypersensitivity Vasculitis
- Palpable purpura
65
Hypersensitivity Vasculitis diagnosis
- Biopsy confirms the diagnosis - Identification of the offending agent - Lesions generally resolve after a period of days or weeks
66
Polymyalgia Rheumatica patients usually affected
- At least 50 years old | - Usually caucasian
67
Polymyalgia Rheumatica characteristics
- Muscle pain lasting for at least 1 month (shoulders, pelvic girdle) - Severe morning stiffness and gelling - No muscle atrophy or true weakness - Erythrocyte sedimentation rate >40 mm/Hr (at least 100 mm/Hr in many patients) - Rapid relief with small doses of glucocorticoids
68
Polymyalgia Rheumatica differential diagnostic possibilities
- Temporal arteritis - Viral myalgia - Rheumatoid Arthritis - Polymystosis - Multiple Myeloma - Osteoarthritis - Fibrositis - Depression - Occult infection - Occult malignancy - Endocrinopathy
69
Bueger's Disease is also known as
- Smoker’s disease | - Thromboangiitis obliterans
70
Bueger's Disease etiology
- Idiopathic, segmental, thrombosing vasculitis of intermediate and small peripheral arteries and veins
71
Bueger's Disease findings
- Intermittent claudication - Superficial nodular phlebitis - Raynaud’s phenomenon - Severe pain - Gangrene
72
Diseases which may mimic Buerger’s Disease
- Atherosclerosis - Endocarditis - Other types of vasculitis - Severe Raynaud's phenomenon associated with connective tissue disorders (e.g., lupus or scleroderma) - Clotting disorders of the blood - Others
73
The commonly followed diagnostic criteria are outlined below although the criteria tend to differ slightly from author to author. Olin (2000) proposes the following criteria:
- 20–40 years old and male (recently few females diagnosed) - Current/recent tobacco use - Presence of distal extremity ischemia (indicated by claudication pain at rest, ischemic ulcers or gangrene) documented by noninvasive vascular testing such as US - Exclusion of other autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests - Exclusion of a proximal source of emboli by echocardiography and arteriography - Consistent arteriographic findings in the clinically involved and noninvolved limbs
74
Angiograms in Bueger's Disease reveal
- “Corkscrew” appearance of arteries that result from vascular damage, particularly the arteries in the region of the wrists and ankles - Collateral circulation gives "tree root" or "spider leg" appearance - May also show occlusions (blockages) or stenosis (narrowings) in multiple areas of both the arms and legs
75
Buerger's Disease treatment
- Quit smoking
76
Adjunctive treatments for Buerger's Disease
- In acute cases, drugs and procedures which cause vasodilation are effective in reducing pain experienced by patient, but do not help in changing the course of disease - Epidural anesthesia and hyperbaric oxygen therapy also have vasodilator effect - In chronic cases, lumbar sympathectomy may be occasionally helpful
77
Sjogren's Syndrome summary
- Immune-mediated disorder of the exocrine glands | - classic triad of dry eye (conjunctivitis, xerophthalmia), dry mouth (dysphagia, xerostomia), and arthritis
78
Vasculidities summary
- The vasculidities are a heterogeneous group of clinical syndromes characterized by inflammation of the blood vessels - Biopsy or arteriography confirms diagnosis of a vasculitis - Corticosteroids and immunosuppressives are the mainstays of treatment
79
Polymyalgia Rheumatica summary
- Commonly associated with temporal arteritis | - Causes muscle pain in the shoulders and pelvic girdle

Pod Med I (3 decks)

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