Connective Tissue Diseases Flashcards Preview

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Flashcards in Connective Tissue Diseases Deck (15):
1

Epidemiology of lupus
Gender
Age
Race

9x more common in women. Associated w/ estrogen.
Peak onset is 15-45 y/o.
More common / severe in AA's

2

Mnemonic for lupus criteria

SOAP BRAIN MD. Need at least 4/11.
•Serositis
•Oral / nasal ulcers. Painless. Nasal ulcers often on septum.
•Arthritis (non-erosive)
•Photosensitivity – When skin cells die, immune system is upregulated, which may lead to a flare
•Blood – hemolytic anemia (Coombs positive), leucopenia, lymphopenia, thrombocytopenia, anemia of chronic disease
•Renal – proteinuria, cellular casts, increased mesangial cells / matrix, basement membrane abnormalities, and immune complex deposits of IgG, IgM, IgA, and complement in the glomerulus.
•ANA
•Immunologic – DNA, Sm, Antiphospholipid
•Neurologic – seizure, psychosis
•Malar rash – spares the nasolabial folds
•Discoid rash – raised plaques w/ keratotic scaling that may be permanently scarring / disfiguring

3

4 things that increase risk for lupus

Smoking, silica dust, dogs, UV light

4

Mechanism of hydroxychloroquine (3)

•Reduces phagocytosis of self-antigens by increasing intracellular pH and disrupting low-affinity binding of self-proteins while preserving high-affinity binding of exogenous antigens.
•Reduces TLR activation by increasing lysosomal pH
•Blocks proliferative responses of T cells after stimulation by auto-Ags, thus decreasing cytokine release.

5

Treating lupus joint pain

NSAIDs and hydroxychloroquine

6

Treating lupus synovitis

Low dose prednisone, MTx, or azathioprine

7

Treating life-threatening disease of kidney, brain, heart, or lung w/ lupus.

High dose prednisone + cyclophosphamide or mycophenolate

8

6 things that may trigger lupus flares

Sun, stress, sulfa drugs, surgery, infection, and pregnancy

9

3 markers of increased mortality w/ lupus

Male gender, lupus anticoagulant, and severe SLE

10

Sjogren syndrome
Gender ratio
Age
Pathogenesis
Clinical manifestations
Labs

9x more common in females
Peak onset is 60 y/o
Lymphocytic infiltration of exocrine glands --> ocular / oral dryness.
Clinical manifestations: dry eyes, dry mouth, parotid swelling, arthralgia, arthritis, LAD
Oral dryness may cause tooth decay
Labs: RF (75%), SSA (Ro) 70%, SSB (La) 50%, ANA (50%)

11

Scleroderma
Gender
Age
In which form is ILD or pulmonary HTN more common?
What causes increased mortality?

9x more common in females.
Peak age of onset is 40-60 y/o
ILD is more common in diffuse. Pulmonary HTN is more common in CREST.
Increased mortality from pulmonary fibrosis and pulmonary HTN.

12

Limited CREST
Labs

•Calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, and telangiectasias.
• Ca deposits are often on finger pad
• No skin tightening proximal to elbows / knees (except face)
• Anti-centromere ANA in 75% of pxs. >50% have Scl-70 Abs

13

Diffuse scleroderma diagnosis criteria

•Diagnosis requires 1 major or 2 minor criteria
• Major criterion: proximal scleroderma w/ skin tightening over face, hands, forearm, and trunk
• Minor criteria: sclerodactyly, digit pitting, finger pad atrophy, bibasilar pulmonary fibrosis.

14

Dermatomyositis / Polymyositis
Gender
Age
Diagnostic criteria
Labs
Increased mortality?

2x more common in females.
Peak onset is age 40-50
Bohan / Peter Criteria
• Symmetric proximal muscle weakness
• Elevated muscle enzymes (CK)
• Myopathic changes on EMG
• DM shows inflammatory cells in perifascicular regions
• PM shows inflammatory cells in muscle fascicles
• Typical rash of dermatomyositis
•Labs: ANA is positive in 80% of pas
•Increased risk of death from cancer, infection, respiratory failure, and CVD.

15

4 rashes common to Dermatomyositis / Polymyositis

•Gottron’s sign suggests DM – erythematous, scaly eruption over extensor surfaces of fingers. May mimic psoriasis.
•Heliotrope rash is red / purple eruption on upper eyelid + swelling
•Shawl sign – diffuse, flat red lesion occurring over chest / shoulders or in a V-shaped distribution over anterior neck / chest. Occurs in DM.
•Mechanic’s hands in PM – roughened / cracking of skin of tips / lateral fingers → dirty-appearing lines