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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
Q

Diagnostic immunologic laboratory studies

A
  • ESR
  • VDRL
  • Antinuclear antibodies
  • Rheumatoid factor
  • Protein electrophoresis
  • Immune complex
  • Complement (CH50, C3, C4)
  • a-ANCA, p-ANCA (Anti-neutrophil cytoplasmic antibodies)
  • Hepatitis panel
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26
Q

Invasive diagnostic studies for selected patients

A
  • Temporal artery, skin, muscle, sural nerve biopsy
  • Angiography
  • Renal biopsy
  • Lung biopsy
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27
Q

Differential diagnosis for vasculitis includes diverse disorders that result in

A
  • Organ ischemia
  • Localized inflammation
  • Constitutional symptoms
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28
Q

The pattern of expression of a specific vasculitic disorder

A
  • May evolve or change over time independent of therapeutic interventions
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29
Q

Drugs of choice used to treat vasculitis

A
  • DMARDS (Disease Modifying Drugs; entire immune system)

- Biologics (target specific steps in the inflammatory process)

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30
Q

Other options for treatment of vasculitis

A
  • Colchicine
  • Dapsone (test G6PD)
  • Steroids
  • Imuran
  • Methotrexate
  • Cytoxan
  • Cytophosphamide
  • Chlorambucil
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31
Q

Vasculopathy

A
  • Thrombi within vessels in the absence of inflammation
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32
Q

Vasculopathy may be caused by

A
  • Atrophie Blanche

- Thrombotic disorders

33
Q

Thrombotic disorders contributing to vasculopathy

A
  • Protein C
  • Protein S
  • Antithrombin III
  • Cryofibrinogen (malignancy, myeloma, luekemia)
  • Factor V Leiden
  • Homocystine
  • Anticardiolipin
  • Lupus
34
Q

General metabolic laboratory diagnostic studies for vasculopathy

A
  • Renal (BUN/Cr)
  • Liver function
  • Hepatitis panel
  • Glycemic control (HbA1C)
  • CV risk (lipids, homocysteine)
  • Gout (uric acid)
  • Thyroid fx (myxedema)
  • Calcium (calcinosis)
35
Q

Hematologic laboratory diagnostic studies for vasculopathy

A
  • 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
Q

Biopsy or arteriography may confirm diagnosis of

A
  • Vasculitis
37
Q

Antineutrophil cytoplasmic antibody (ANCA) is present in most cases of

A
  • Wegener’s granulomatosis
38
Q

Skin biopsies diagnostic in

A
  • Hypersensitivity vasculitis
39
Q

Forms of vasculitis

A
  • Takayasu’s arteritis
  • Giant cell (temporal) arteritis
  • Polyarteritis Nodosa
  • Wegener’s Granulomatosis
  • Hypersensitivity vasculitis
  • Polymyalgia rheumatica
40
Q

Other forms of vasculitis

A
  • Churg-Strauss Syndrome
  • Henöch-Schönlein purpura
  • Cryoglobulinemia (Vasculopathy)
  • Thromboangiitis obliterans
41
Q

Takayasu’s Arteritis

A
  • 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
Q

Takayasu’s Arthritis diagnosis

A
  • Suspect in young women with a history of systemic inflammatory illness, or bruits over large arteries
  • Arteriography confirms diagnosis
43
Q

Takayasu’s Arthritis treatment

A
  • Corticosteroids
  • “Steroid-sparing’ agents (nonbiologic and biologic DMARDS)
  • Combination therapy for resistant cases
  • Endovascular/Vascular bypass procedures/angioplasty
44
Q

Giant Cell (Temporal) Arteritis classification criteria (must have 3/5)

A
  • 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
Q

Giant Cell (Temporal) Arteritis treatmenr

A
  • Corticosteroids

- Cytotoxic or immunosuppressive agents

46
Q

Polyarteritis Nodosa(PAN)

A
  • Blood vessel disease characterized by inflammation of small and medium-sized arteries (vasculitis)
  • Prevents them from bringing oxygen and food to organs
47
Q

Most cases of Polyarteritis Nodosa (PAN) occur in

A
  • 4th or 5th decade of life, although it can occur at any age
48
Q

Polyarteritis Nodosa(PAN) may be the first sign of

A
  • TB
  • Bacterial/fungal infection
  • Sarcoidosis
  • IBS
  • Cancer
49
Q

Polyarteritis Nodosa (PAN) high-yield facts

A
  • Typically involves renal and visceral vessels
  • Fever, weight loss, malaise, abdominal pain, headache, myalgia, hypertension
  • Diagnosed by skin biopsy
50
Q

ACR classification criteria for Polyarteritis Nodosa (1-5)

A
  • Weight loss >4 kg
  • Livedo Reticularis
  • Testicular pain or tenderness
  • Myalgias, weakness, or leg tenderness
  • Mononeuropathy or polyneuropathy
51
Q

ACR classification criteria for Polyarteritis Nodosa (6-10)

A
  • Diastolic BP >90 mmHg
  • Elevated BUN or creatinine
  • Hepatitis B virus
  • Arteriographic abnormality
  • Biopsy of small or medium artery containing PAN
52
Q

Polyarteritis Nodosa is a systemic vasculitis characterized by

A
  • necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcations
53
Q

Polyarteritis Nodosa is a systemic vasculitis results in

A
  • Microaneurysm formation
  • Aneurysmal rupture with hemorrhage
  • Thrombosis
  • Consequently, organ ischemia or infarction
54
Q

Polyarteritis Nodosa treatment

A
  • Corticosteroids
  • Azathioprine
  • Cyclophosphamide
  • Colchicine
  • Cyclophosphamide
  • IV immunoglobulin
  • Penicillin to prevent flairs of strep tonsillitis orcellulitis
55
Q

Although identical skinlesionsare common in systemic PAN, cutaneous PAN should be considered

A
  • A separate disease
  • Distinguished from systemic PAN
  • Clinical course and management of these conditions differ from each other
56
Q

Systemic PAN is a vasculitis that causes destructive inflammation of medium-sized muscular arteries of multiple systems including

A
  • Liver
  • Kidney
  • Heart
  • Lung
  • Gastrointestinal tract
  • Musculoskeletal
  • Nervous systems
57
Q

Systemic PAN is a potentially life-threatening form of vasculitis, whereas cutaneous PAN

A
  • Usually runs achronicbut relativelybenigncourse
58
Q

Wegener’s Granulomatosis high yield facts

A
  • Focal necrotizing vasculitis and necrotizing granulomas in the lung and upper airway
  • Necrotizing glomerulonephritis
  • CXR may reveal large nodular densities
  • Hematuria
59
Q

Wegener’s Granulomatosis is now called

A
  • Granulomatosis with polyangiitis (GPA)
60
Q

ACR classification criteria for Wegener’s Granulomatosis (must have 2/4)

A
  • 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
Q

Wegener’s Granulomatosis treatment

A
  • Corticosteroids
  • Methotrexate
  • Cyclophosphamide
  • Combinations of two antibiotics ( trimethoprim and sulfamethoxazole)
  • Rituximab (Biologic)
  • CellCept (mycophenolate)
  • Others
  • Team approach
62
Q

Hypersensitivity Vasculitis

A
  • Vasculitis of small vessels, especially arterioles and venules secondary to an immune response to exogenous substances
63
Q

Most common cause of Hypersensitivity Vasculitis

A
  • Reactions to drugs are the most common cause

- Onset is usually abrupt and occurs after exposure to the etiologic agent

64
Q

Most common clinical manifestation of Hypersensitivity Vasculitis

A
  • Palpable purpura
65
Q

Hypersensitivity Vasculitis diagnosis

A
  • Biopsy confirms the diagnosis
  • Identification of the offending agent
  • Lesions generally resolve after a period of days or weeks
66
Q

Polymyalgia Rheumatica patients usually affected

A
  • At least 50 years old

- Usually caucasian

67
Q

Polymyalgia Rheumatica characteristics

A
  • 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
Q

Polymyalgia Rheumatica differential diagnostic possibilities

A
  • Temporal arteritis
  • Viral myalgia
  • Rheumatoid Arthritis
  • Polymystosis
  • Multiple Myeloma
  • Osteoarthritis
  • Fibrositis
  • Depression
  • Occult infection
  • Occult malignancy
  • Endocrinopathy
69
Q

Bueger’s Disease is also known as

A
  • Smoker’s disease

- Thromboangiitis obliterans

70
Q

Bueger’s Disease etiology

A
  • Idiopathic, segmental, thrombosing vasculitis of intermediate and small peripheral arteries and veins
71
Q

Bueger’s Disease findings

A
  • Intermittent claudication
  • Superficial nodular phlebitis
  • Raynaud’s phenomenon
  • Severe pain
  • Gangrene
72
Q

Diseases which may mimic Buerger’s Disease

A
  • 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
Q

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:

A
  • 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
Q

Angiograms in Bueger’s Disease reveal

A
  • “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
Q

Buerger’s Disease treatment

A
  • Quit smoking
76
Q

Adjunctive treatments for Buerger’s Disease

A
  • 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
Q

Sjogren’s Syndrome summary

A
  • Immune-mediated disorder of the exocrine glands

- classic triad of dry eye (conjunctivitis, xerophthalmia), dry mouth (dysphagia, xerostomia), and arthritis

78
Q

Vasculidities summary

A
  • 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
Q

Polymyalgia Rheumatica summary

A
  • Commonly associated with temporal arteritis

- Causes muscle pain in the shoulders and pelvic girdle

Pod Med I (3 decks)

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