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Flashcards in Systemic connective tissue disease Deck (16)
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Describe the epidemiology of lupus

More common in women, African Americans, associated with estrogen therapy


List the 11 criteria for diagnosing lupus

S: Serositis, inflammation of the pericardial or pleural lining
O: Oral or nasal ulcers
A: Non-erosive arthritis of at least one joint, confirmed by a physician
P: Photosensitivity
B: Blood abnormalities-hemolytic anemia, leucopenia, lymphopenia or thrombocytopenia
R: Renal disease including proteinuria, hematuria and cellular casts
A: A positive antinuclear antibody (ANA) is noted in ~95% of lupus patients
I: Immunologic DNA Sm Antiphospholipid
N: Neurologic
M: Malar rash
D: Discoid rash


List risk factors for lupus

EBV in childhood
silica dust
UV light


Describe the pathologic findings in lupus

inflammation, bland vasculopathy and vasculitis, and immune complex deposits.


Describe renal histopathology in lupus

increase in mesangial cells and mesangial matrix, inflammation, cellular proliferation, basement membrane abnormalities, and immune complex deposits


Describe clinical manifestations of lupus

skin: photosensitivity, rash, keratotic scaling, alopecia
Mucosal: PAINLESS oral ulcers
MSK: non-erosive arthritis, reversible subluxation
pulmonary: pleurisy, PEs, interstitial lung dz, pulmonary HTN
Cardiac: pericarditis, Libman-Sacks endocarditis, cardiomyopathy
Renal: nephritis
Nervous: delerium, affective disorders, cognitive disordes, seizures
Hematologic: anemia of chronic disease, Coombs+ hemolysis


_______ is a drug that can decrease the frequency of lupus flares and is associated with improved survival



What drugs are used for severe life threatening kidney, brain, heart, or lung involvement of lupus?

high dose prednisone, cyclophosphamide or mycophenolate


Describe the epidemiology of Sjogren's syndrome

more common in women, mean age of onset is 60 yrs


What are major clinical manifestations of Sjogren's syndrome?

keratoconjunctiitis sicca, sicca complex, parotid swelling, arthralgia, dental caries, corneal ulceration


_______ disorders are more common in Sjogren's than the general population

lymphoproliferative, lymphoma


Describe tests that can help diagnose Sjogren's

ANA in 50%, often have speckled pattern
Rheumatoid factor in ~90%
SSA found in ~70%
SSB found in ~50%


Describe the clinical manifestations of diffuse systemic sclerosis

- skin tightening over the face, hands, forearms, trunk
- Raynaud's
- ANA+
- pulmonary fibrosis
- telangiectasias
- esophageal dysmotility, stricture
- pulmonary HTN
- scleroderma renal crisis


List manifestations of limited systemic sclerosis

- distal scleroderma
- sclerodactyly, subcutaneous calcium deposits
- Raynaud's
- esophageal dysmotility
- telangiectasias
- interstitial lung disease, pulmonary HTN


What are the clinical manifestations of dermatomyositis/ polymyositis?

myopathy, rashes (dermatomyositis), lung disease, esophageal disease, myocarditis, Raynaud's


Describe the types of rash typically seen in dermatomyositis

Gottrons: scaly eruption over MCP and IP joints
Heliotrope rash
Shawl sign
abnormal capillary loops in the nailbeds
mechanics hands