Rheum - MedEd - other connective tissue diseases Flashcards
Scleroderma - pathophys
Collagen deposition - collagen makes everything stiff
Presentations of scleroderma - 2
1) Limited cutaneous systemic sclerosis (LCSS) similar to CREST
2) Diffuse cutaneous systemic sclerosis (DCSS) similar to scleroderma
What does CREST stand for and what does it mean?
- Calcinosis - HTN
- Raynaud’s - fingers get cold, will vasoconstrict and get white
- Esophageal dysmotility - unrelenting GERD
- Sclerodactyly - fingers tense, skin between replaced by smooth muscle. Also in general, they don’t have wrinkles/tight face and hands. Loss of skin folds
- Telangiectasia - GI bleeds/anemia
What is the difference between limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis
DCSS same as LCSS/CREST
with addition of visceral involvement - lungs, heart, kidney
What happens to the lungs, heart, kidneys in DCSS
Lungs - interstitial lung disease
Heart - constrictive pericarditis
Kidneys - scleroderma renal crisis
Treatment of scleroderma
No treatment Can only control symptoms Treatment of sclerodactyly - penicillamine Treatment of GERD - PPI Treatment of Raynaud's - CCB Treatment of calcinosis - CCB No treatment of telangiectasia
How to treat scleroderma - renal crisis? (Cr going up)
Give ACEi
Do not give steroids - can precipitate renal crisis
Serology of CREST
Anti-Centromere
Remember C in Centromere and C in CREST
CREST can cause what in lungs?
Pulmonary artery hypertension
No damage to lungs
DCSS - what serology?
Anti-Scl70 = topoisomerase
ILD in DCSS can lead to
Pulmonary artery hypertension
So difference between pulmonary artery hypertension in CREST and DCSS is…
CREST - no lung changes
DCSS - ILD, so there are lung changes
Nephrogenic systemic sclerosis
Same physical symptoms as CREST and DCSS
But happens in context of gadolinium exposure (MRI) and CKD
Sjogren’s - pathophys
Lymphoplasmacytic exocrine glands - on biopsy
3 symptoms of sjogren’s
1) Dry eyes - keratoconjunctivitis sicca
2) Dry mouth -xerostomia
3) Partotid swelling
Diagnosis of sjogren’s
ANA and RF - usually positive
Ro, La - positive in sjogren’s (need to know this!)
Schirmer test - can they produce their own tears
Treatment of sjogren’s
Artificial tears and artificial saliva (i.e. with food)
Live normal lifespan and no issues
But need to check for other rheum diseases - i.e. lupus
Idiopathic inflammatory myopathy = myositis
There are 3 types
1) Inclusion myositis - T cell
2) Polymyositis - T cell
3) Dermatomyositis - complex deposition
- Inclusion myositis is different; while polymyositis and dermatomyositis are similar
Presentation of myositis
Proximal muscle weakness - distal muscles are usually okay
Painless
Subacute
Can be associated with malignancy - should do age appropriate cancer screen
Heliotrope rash around eyes - pathogneumonic
Groton’s papules - around large joints - pathogneumonic
Shaw rash - exposure to sun causes photosensitivity rash
Diagnosis of myositis
CK (elevated)
But is this myositis or neuropathy? get EMG!
Best test: biopsy
Antibodies: Anti - Mi, Anti - Jo (not as helpful as above)
Treatment of myositis
Steroids
