SLE-related illnesses Flashcards
3 SLE related illnesses
Polymyositis/Dermatomyositis
Sjögren’s syndrome
Sarcoidosis
what are polymyositis and dermatomyositis?
idiopathic inflammatory myopathies:
-muscle inflammation leading to weakness
polymyositis vs dermatomyositis
PM: just muscle (proximal first)
DM: muscle AND skin , rashes
Amyopathic dermatomyositis (ADM)
Rash only, no muscle involvement (newly described and uncommon)
polymyositis and dermatomyositis: women or men more?
women
clinical presentation of muscle weakness from PM/DM?
Usually painless
Can’t climb stairs or lift arms to do hair
Trouble swallowing
4 characteristic skin manifestions of DM
- Heliotrope rash (purple around eyes)
- Malar rash (looks like SLE)
- Shawl sign – photsensitization of skin (scarf around neck rash)
- Gottron’s papules – over PIP and MCP
*diffuse redness on face + Possible Raynaud phenomenon
Labs for DM/PM:
+ ANA
CK (creatine kinase- muscle breakdown) - HIGH
adolase (converts glucose to energy)- HIGH
what do you need to dx DM/PM?
Muscle Bx: shows inflammation
Txt for DM/PM
Steroids high dose
Methotrexate- longterm
Azathioprine - longterm
polymyositis (PM) vs polymyalgia rheumatica (PMR)
PM: can’t get up b/c weakness
PMR: can’t get up b/c of pain
Sjögren’s disease (A.K.A. Sicca)
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)
3 presenting symptoms of SICCA
- dry eye (xerophthalmia)
- dry mouth (xerostomia)
- parotid gland enlargement (trying to produce more alive = hypertrophy)
txt options for SICCA
mostly symptomatic, avoiding anticholinergic drugs
- artificial tears
- restasis aka cyclosporine (immunesuppresant)
- silicone sporine ducts to keeps tears in eye and not draining into nose
primary or secondary SICCA more common ?
what does secondary come from?
uncommon: primary
Common: secondary to other connective tissue disorder
RA, SLE, polymyositis, scleroderma
who gets SICCA?
women, 60yo, w/ some connective tissue disorder
labs for SICCA
- antibodies (anti- SSA, anti- SSB)
- ESR high
what is Schirmer’s test?
for SICCA : paper in bottom of eye for 5 minutes <5mm of wetness then test is positive
what is there a high risk for with SICCA
lymphoma
who gets sarcoidosis?
Most in North American black women
Northern European whites
pathophys of sarcoidosis?
not really autoimmune…
Chronic granulomatous inflammation, most often in lung but can be anywhere
clinical presentation of sarcoidosis
cough, SOB and chest pain
Malaise and fever (systemic inflamm. response)
what is lofgren’s syndrome?
Arthritis, erythema nodosum (EN), hilar adenopathy
a specific kind of sarcoidosis
what is erythema nodosum (EN)?
EN = painful red nodular rash usually on shins
Dx of sarcoidosis? which do you need for the final dx?
ACE- high
ESR- high
hilar adenopathy and possible diffuse reticular infiltrates (xray)
NEED!: Bx (transbronchial or fine needle)
Noncaseating Granuloma is pathognomonic
txt of sarcoidosis
Oral prednisone high enough to control symptoms
referral for sarcoidosis
usually to pulmonary
may need lung transplant if its bad enough
In general, autoimmune disease that is CHRONIC and PROGRESSIVE is alleviated by _____ and _____, but these are not first-line. First line is what?
steroids and NSAIDs
1st line: DMARDS (started early)
which kind of autoimmune diseases can be txted with steroids?
Short-term, nonprogressive autoimmune
