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Systemic Disorders Flashcards

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1
Q

What are the clinical features of systemic lupus erythematous?

A

I’M DAMN SHARP

Immunoglobbulins (anti-dsDNA, anti-Sm, antiphospholipid antibodies)
Malar rash

Discoid rash (photosensitive inflammation of skin)
Antinuclear antibody
Mucosal ulcers
Neurologic disorders (cerebral lupus)

Serositis (pleuritis, pericarditis, Libman Sacks endocarditis)
Hematologic disorders
Arthritis
Renal disorders (proliferative glomerulonephritis)
Photosensitivity

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2
Q

What is Libman Sacks endocarditis? What is it associated with?

A

1) Non infectious vegetations on both sides of the heart valve due to hypercoagulability state associated with antiphospholipid antibodies
2) Systemic Lupus Erythematous

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3
Q

Is ANA sensitive or specific? What does that indicated when there is a positive result?

A

1) Highly sensitive (think: SNOUT)
2) A positive test result means that the pt. may have SLE, however, it is not specific so many pt. have a positive ANA but do not have SLE

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4
Q

What drug is used in the most severe manifestations of SLE?

A

1) Cyclophosphamide

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5
Q

What is the main drug used in treatment of SLE?

A

1) Glucocorticoid steroids

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6
Q

What does a positive Scl-70 antibody? What is Scl-70 Antibody?

A

1) Indicated of diffuse cutaneous systemic sclerosis

2) Anti-DNA topoisomerase I antibody

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7
Q

What are the common findings of limited cutaenous systemic sclerosis?

A

CREST syndrome

1) Calcinosis
2) Raynaud’s phenomenon
3) Esophageal involvement
4) Sclerodactylyl
5) Telangiectasia

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8
Q

How do you differentiate between diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis?

A

1) Diffuse cutaneous systemic sclerosis occurs in the skin proximal to the knees and elbows; limited has distal occurrence
2) Diffuse cutaneous systemic sclerosis has renal complications; limited has pulmonary hypertension
3) Diffuse cutaneous systemic sclerosis has a very poor prognosis compared to limited
4) Diffuse = Scl-70 antibody; Limited = anti-centromere antibody

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9
Q

What are major complications of systemic sclerosis?

A

1) Pulmonary hypertension (more common in localized form)
2) Hypertensive renal crisis (more common in diffuse form)
3) Sclerodactylyl
4) Raynaud’s phenomenon
5) Arthalgia
6) Smooth muscle atrophy and fibrosis of the lower 2/3 of the esophagus

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10
Q

Autoimmune disorder characterized by lymphocytic infiltration of the salivary and lacrimal glands leading to glandular fibrosis and exocrine failure

A

Sjogren’s Syndrome

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11
Q

What test is used to diagnose Sjogren’s syndrome by measuring tear flow over 5 minutes?

A

Schirmer tear test

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12
Q

Xerophthalmia, Xerostomia, arthritis, enlarged parotid, and positive for Anti-Ro and Anti-La antibodies

A

Sjogren syndrome

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13
Q

Symmetrical proximal muscle weakness; positive anti Jo1 antibodies and cutaneous involvement (Gottron lesion, heliotrope rash, malar rash)

A

Dermatomyositis

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14
Q

Small vessel vasculitis; most common in children; usually follows an URI; classic triad (palpable purpura, arthalgia, and abdominal pain); caused by IgA complex deposition

A

Henoch-Schonlein purpura

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15
Q

What is important to note about the areas that polyarteritis nodosa (PAN) affects?

A

1) It does not affect the pulmonary vasculature

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16
Q

What are the findings of polyarteritis nodosa?

A

1) Transmural inflammation of the vasculature with fibrinoid necrosis
2) Lesions are in different stage simultaneously

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17
Q

What is the cause of polyarteritis nodosa?

A

Immune complex mediated

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18
Q

Necrotizing vasculitis involving the lungs, kidneys, and skin; p-ANCA positive

A

Microscopic polyangiitis

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19
Q

Granulomatous, necrotizing vasculitis with eosinophilia; p-ANCA positive

A

Chrug-Strauss vasculitis

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20
Q

Focal nectrotizing vasculitis with necrotizing granulomas in the lung; also causes rapid progressive cresentric glomerulonephritis; c-ANCA positive

A

Wegener’s granulomatosis

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21
Q

Vasculitis that is common in asian children that presents with lymphadenitis, strawberry tongue, and hand-foot erythema

A

Kawasaki disease

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22
Q

Vasculitis affecting the temporal artery that commonly is involved with the elderly; has a segmental focal granulomas; may lead to irreversible blindness

A

Temporal (giant cell) vasculitis

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23
Q

Also known as the “Pulseless disease” due to the formation of granulomas in the aortic arch that causes decreased blood flow to the extremities; common in Asian females <40

Study These Flashcards
A

Takayasu’s arteritis

24
Q

Idiopathic immune mediated disease with wide spread non-caseating granulomas; bilateral hilar lymphadenopathy, elevated ACE, hypercalcemia, Lupus pernio

25
Why is there hypercalcemia in sarcoidosis?
It is due to an elevated alpha 1 hydroxylase 25-hydroxycholecalciferol
26
Lesion on nose associated with sarcoidosis
Lupus pernio
27
Idiopathic systemic disease characterized by recurrent oral and genital ulcers that are extremely painful; also demonstrates pathergy phenomenon
Behcet's Disease
28
Pathergy Phenomenom
1) Sterile pustules form where pt. gets stuck with needle
29
Systemic disorder with inflammatory destructive lesions of cartilage in the ear, nose, and throat; auricular inflammation, fever, arthritis
Relapsing polychondritis
30
Systemic inflammatory disorder characterized by high fever of unknown origin, quickly fading rash, leukocytosis, and arthritis; high levels of ferritin
Adult Still Disease
31
What are the findings for anti-phospholipid syndrome
1) IgG or IgM anti-cardiolipin 2) Recurrent thrombosis 3) Fetal loss in 1st trimester 4) Thrombocytopenia 5) Lupus anticoagulannt (causes clots)
32
Lupus like syndrome with anti-histone antibodies; presents with fever, arthalgia, and serositis; occurs after an addition to a new drug
Drug induced Lupus
33
What medications may cause drug induced lupus?
1) Hydralazine 2) Methyldopa 3) Isoniazid 4) Anti-TNF alpha 5) Interferon alpha
34
What complications are generally not found in drug induced lupus
1) Renal or neurological symptoms
35
What is the main treatment for SLE?
1) Glucocorticoids
36
What are complications of glucocorticoids?
1) Cushing syndrome 2) Avascular necrosis of the femoral head 3) Immunosuppression
37
High anti-ribonucleoprotein
Mixed Connective Tissue disease
38
What is a pt. with Sjogren's syndrome at an increased risk for?
1) Increased risk of lymphoma
39
Elderly, loss of sight, new headache, ESR >50
Temporal arteritis
40
What is treatment for Temporal arteritis
1) Gluococorticoids
41
Palpable purpura, Raynaud's phenomenon, arthritis; strongly association with Hepatitis C; proteins precipitate with lower temperature
Cryoglobulinemia
42
What are the causes of death for relapsing polychondritis?
1) Airway inflammation leading to respiratory failure 2) Infection complications 3) Inflammation of vessels with aneurysm rupture
43
Uveitis and and recurrent oral and genital ulcers
Behcet's disease
44
ANA
SLE (nonspecific, sensitive)
45
Anti SSA/Ro
Sjogren
46
Anti-Scl70
Scleroderma (diffuse)
47
Anti-dsDNA
SLE (specific)
48
Anti-histone
Drug-induced Lupus
49
Anti-centromere
CREST syndrome
50
Anti SSB/La
Sjogren
51
Anti-RNP
Mixed connective tissue disease
52
Anti-Jo1 (tRNA synthetase)
Polymyositis/Dermatomyositis
53
Pain and stiffness in shoulders and hips, often with fever, malaise, and weight loss; does not causes muscular weakness; associated with temporal arteritis
Polymyalga rheumtica
54
Pt with a rash/skin manifestation with visible blood vessels or new insidious onset of difficulty swallowing. What is the disease? What antibody is present?
1) CREST Syndrome | 2) Anti-centromere antibody
55
How do you differentiate between Wergner's granulomatosis and Sarcoidosis?
1) Sarcoidosis consists of non-caseating granulomas that are found in a bilateral hilar lymphadnopathy pattern 2) Wergner's consists of caseating granulomas found in the lung

Musculoskeletal (5 decks)

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