OnlineMedEd: Rheumatology - "Other Connective Tissue" Flashcards
Scleroderma results from _____________.
collagen deposition (everything gets stiffer)
Review CREST syndrome (limited cutaneous systemic scleroderma –LCSS).
- Calcinosis (HTN?)
- Raynaud’s
- Esophageal dysmotility (unrelenting GERD)
- Sclerodactyly (tense fingers)
- Telangiectasia (GI bleed, IDA)
What are the diffuse manifestations of scleroderma (diffuse cutaneous systemic scleroderma –DCSS)?
- Intrinsic lung disease
- Constrictive pericarditis
- Sclerodermal renal crisis
How can you treat CREST?
There are no DMARDs, but you can manage symptoms:
• Sclerodactyly –penicillamine
•Esophageal dysmotility –PPIs, Nissen
•Raynaud’s –CCBs
True or false: steroids can be used in sclerodermal crises.
False
Steroids can precipitate a renal crisis in scleroderma.
How do you treat sclerodermal renal crises?
ACE inhibitors
It is unusual to give an ACEi in the setting of a rising creatinine, but this works.
What antibody is seen in diffuse scleroderma?
Anti-scl70 (aka anti-topoisomerase)
Sjögren’s is caused by _________________.
lymphoplasmocytic destruction of the exocrine glands
Review the signs and symptoms of Sjögren’s.
- Keratoconjunctivitis sicca (dry eyes)
- Xerostomia (dry mouth)
- Parotid swelling
Describe the diagnosis of Sjögren’s.
1st: ANA, RF positive
2nd: Ro, La antibodies
3rd: Schirmer test positive
What is the usual cause of death in Sjögren’s?
Trick question: nothing
Those with Sjögren’s are generally only symptomatically managed and do not have complications.
There are three autoimmune myositis disorders: ______________.
dermatomyositis, polymyositis, and inclusion-body myositis
How do you diagnose inflammatory myopathies?
- CK
- EMG
- Biopsy
- Anti-Jo and anti-Jo antibodies
