Systemic connective tissue disease

Question 1

Describe the epidemiology of lupus

Answer 1

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

Question 2

List the 11 criteria for diagnosing lupus

Answer 2

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

Question 3

List risk factors for lupus

Answer 3

genetics
estrogen
EBV in childhood
smoking
silica dust
dogs
UV light

Question 4

Describe the pathologic findings in lupus

Answer 4

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

Question 5

Describe renal histopathology in lupus

Answer 5

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

Question 6

Describe clinical manifestations of lupus

Answer 6

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

Question 7

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

Answer 7

hydroxychloroquine

Question 8

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

Answer 8

high dose prednisone, cyclophosphamide or mycophenolate

Question 9

Describe the epidemiology of Sjogren’s syndrome

Answer 9

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

Question 10

What are major clinical manifestations of Sjogren’s syndrome?

Answer 10

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

Question 11

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

Answer 11

lymphoproliferative, lymphoma

Question 12

Describe tests that can help diagnose Sjogren’s

Answer 12

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

Question 13

Describe the clinical manifestations of diffuse systemic sclerosis

Answer 13

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

Question 14

List manifestations of limited systemic sclerosis

Answer 14

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

Question 15

What are the clinical manifestations of dermatomyositis/ polymyositis?

Answer 15

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

Question 16

Describe the types of rash typically seen in dermatomyositis

Answer 16

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