Connective Tissue Diseases Flashcards
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
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
4 things that increase risk for lupus
Smoking, silica dust, dogs, UV light
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.
Treating lupus joint pain
NSAIDs and hydroxychloroquine
Treating lupus synovitis
Low dose prednisone, MTx, or azathioprine
Treating life-threatening disease of kidney, brain, heart, or lung w/ lupus.
High dose prednisone + cyclophosphamide or mycophenolate
6 things that may trigger lupus flares
Sun, stress, sulfa drugs, surgery, infection, and pregnancy
3 markers of increased mortality w/ lupus
Male gender, lupus anticoagulant, and severe SLE
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%)
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.
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
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.
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.
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
