SLE-related illnesses

Question 1

3 SLE related illnesses

Answer 1

Polymyositis/Dermatomyositis
Sjögren’s syndrome
Sarcoidosis

Question 2

what are polymyositis and dermatomyositis?

Answer 2

idiopathic inflammatory myopathies:

-muscle inflammation leading to weakness

Question 3

polymyositis vs dermatomyositis

Answer 3

PM: just muscle (proximal first)
DM: muscle AND skin , rashes

Question 4

Amyopathic dermatomyositis (ADM)

Answer 4

Rash only, no muscle involvement (newly described and uncommon)

Question 5

polymyositis and dermatomyositis: women or men more?

Answer 5

women

Question 6

clinical presentation of muscle weakness from PM/DM?

Answer 6

Usually painless
Can’t climb stairs or lift arms to do hair
Trouble swallowing

Question 7

4 characteristic skin manifestions of DM

Answer 7

1. Heliotrope rash (purple around eyes)
2. Malar rash (looks like SLE)
3. Shawl sign – photsensitization of skin (scarf around neck rash)
4. Gottron’s papules – over PIP and MCP

*diffuse redness on face + Possible Raynaud phenomenon

Question 8

Labs for DM/PM:

Answer 8

+ ANA
CK (creatine kinase- muscle breakdown) - HIGH
adolase (converts glucose to energy)- HIGH

Question 9

what do you need to dx DM/PM?

Answer 9

Muscle Bx: shows inflammation

Question 10

Txt for DM/PM

Answer 10

Steroids high dose
Methotrexate- longterm
Azathioprine - longterm

Question 11

polymyositis (PM) vs polymyalgia rheumatica (PMR)

Answer 11

PM: can’t get up b/c weakness
PMR: can’t get up b/c of pain

Question 12

Sjögren’s disease (A.K.A. Sicca)

Answer 12

Autoantibodies directed against exocrine glands,
Most affected are lacrimal and salivary glands, with inflammatory destruction of glands
(can be primary to secondary to other autoimmune d/o)

Question 13

3 presenting symptoms of SICCA

Answer 13

• dry eye (xerophthalmia)
• dry mouth (xerostomia)
• parotid gland enlargement (trying to produce more alive = hypertrophy)

Question 14

txt options for SICCA

Answer 14

mostly symptomatic, avoiding anticholinergic drugs

• artificial tears
• restasis aka cyclosporine (immunesuppresant)
• silicone sporine ducts to keeps tears in eye and not draining into nose

Question 15

primary or secondary SICCA more common ?

what does secondary come from?

Answer 15

uncommon: primary
Common: secondary to other connective tissue disorder
RA, SLE, polymyositis, scleroderma

Question 16

who gets SICCA?

Answer 16

women, 60yo, w/ some connective tissue disorder

Question 17

labs for SICCA

Answer 17

• antibodies (anti- SSA, anti- SSB)

- ESR high

Question 18

what is Schirmer’s test?

Answer 18

for SICCA : paper in bottom of eye for 5 minutes <5mm of wetness then test is positive

Question 19

what is there a high risk for with SICCA

Answer 19

lymphoma

Question 20

who gets sarcoidosis?

Answer 20

Most in North American black women

Northern European whites

Question 21

pathophys of sarcoidosis?

Answer 21

not really autoimmune…

Chronic granulomatous inflammation, most often in lung but can be anywhere

Question 22

clinical presentation of sarcoidosis

Answer 22

cough, SOB and chest pain

Malaise and fever (systemic inflamm. response)

Question 23

what is lofgren’s syndrome?

Answer 23

Arthritis, erythema nodosum (EN), hilar adenopathy

a specific kind of sarcoidosis

Question 24

what is erythema nodosum (EN)?

Answer 24

EN = painful red nodular rash usually on shins

Question 25

Dx of sarcoidosis? which do you need for the final dx?

Answer 25

ACE- high
ESR- high
hilar adenopathy and possible diffuse reticular infiltrates (xray)

NEED!: Bx (transbronchial or fine needle)
Noncaseating Granuloma is pathognomonic

Question 26

txt of sarcoidosis

Answer 26

Oral prednisone high enough to control symptoms

Question 27

referral for sarcoidosis

Answer 27

usually to pulmonary

may need lung transplant if its bad enough

Question 28

In general, autoimmune disease that is CHRONIC and PROGRESSIVE is alleviated by _____ and _____, but these are not first-line. First line is what?

Answer 28

steroids and NSAIDs

1st line: DMARDS (started early)

Question 29

which kind of autoimmune diseases can be txted with steroids?

Answer 29

Short-term, nonprogressive autoimmune