Systemic Connective Tissue Diseases

Question 1

Who is most often effected by lupus?

Answer 1

women 9:1, more common an more severe in African Americans
peak onset of disease 15-45yo
some genetic predisposition
those taking oral contraceptive and estrogen therapy (men with lupus are 14x likely to have XXY Klinefelter’s Syndrome)

Question 2

List the ACR criteria for SLE. (SOAP BRAIN MD).

Answer 2

Serositis (pleurisy or pericarditis)
Oral or nasal ulcers
Arthritis (non-erosive)
Photosensitivity (skin rash or hives)
Blood: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
Renal: proteinuria and cellular casts, linear IgG deposits
ANA (IMPORTANT)
Immunologic (DNA, Smith, Antiphopholipid)
Neurologic (seizure, psychosis)
Malay rash (spares nasolabial folds)
Discoid rash

Question 3

When suspecting lupus what tests should you order? What are useful as secondary tests?

Answer 3

ANA- antinuclear antibody (95% lupus patients are positive but not specific)
complete blood count with differential
creatinine and UA with microscopy to check kidney function

additionally: complement levels C3 and C4
ENA panel: Sm, RNP, SSA, SSB, DNA* and antiphopholipid antibodies

DNA antibodies are very specific but not very sensitive

Question 4

Describe the standard treatment of SLE.

Answer 4

refer to rheumatologist to confirm and follow dx.
hydroxycholoroquine is given to all patients (associated with longer lifespan)

patients should be monitored for organ involvement and addressing CAD, infection risk, and drug side effects

Question 5

What things are known to trigger SLE flares?

Answer 5

sun, stress, sulfa drugs, surgery, infection, pregnancy

Question 6

What causes Sjogren’s syndrome? What are the resulting symptoms?

Answer 6

lymphocytic infiltrate of the exocrine glands as a primary problem or secondary to other autoimmune disease (RA, SLE)

leads to ocular, oral dryness (tooth decay), lypmhadenoapathy, arthralgia or arthritis (rarely lung, cardiac neurologic and renal involvement or increased risk for lymphoproliferative disorders)

typically effects women after the age of 60yo

Question 7

List conditions that can be accompanied by secondary sjorgren’s syndrome.

Answer 7

RA
SLE
thyroiditis
autoimmune hepatitis
primary billiary cirrhosis
cryoglobulinemia
vasculitis

Question 8

Which tests results are most diagnostic of Sjogren’s?

Answer 8

high ESR 80%
high gamma globulins 75%
RF 75%
SSA Ro 70%
SSB La 50%
speckled ANA 50%

Question 9

Who is typically effected by Scleroderma?

Answer 9

females 9:1 between 40 and 60yo

Question 10

Scleroderma is associated with what environmental exposures?

Answer 10

silica, vinyl chloride, toxic oil, L-tryptophan

Question 11

What are the principle symptoms of limited scleroderma?

Answer 11

CREST
Calcinosis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyl
Telangectasia (mat-like)

*20% pulmonary HTN

Question 12

What are the principle symptoms of diffuse scleroderma?

Answer 12

** proximal scleroderma (skin tightening over face, hands, forearms, trunk)

sclerodactyly, digital pitting or finger pad atrophy
biblasilar pulmonary fibrosis

Question 13

Describe the clinical features and laboratory findings of dermatomyositis and polymyositis?

Answer 13

clinical features: proximal muscle weakness, rashes (erythematous scaly eruption on extensor surfaces), shawl sign, heliotrope rash, mechanic’s hand, lung disease, esophageal weakness, capillary loop dilation of nail bed

lab: high serum CK, ANA+ in 80% +/- other antibodies, EMG, muscle biopsy