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54 terms

KAPNotes: Rheum

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Define scleroderma:
Chronic multi-system dz characterized clinically by thickening of the skin dz and accumulation of connective tissue and involvement visceral organs and replace of smooth muscle
All patients with sclecroderma have
Raynaud's and skin thickening
The pathophysiology of Raynaud's?
diminished blood flow and vascular damage
GI features associate w scleroderma
esophageal dysmotility, achalasia, bacterial overgrowth, dilation of large intestines, hypomotility of small intestine, malabsorption
Pulmonary features assoc. w scleroderma
pulmonary fibrosis, cor pulmonale, restrictive lung dz
Leading cz of death in scleroderma
pulmonary fibrosis, cor pulmonale, restrictive lung dz
Renal features assoc w scleroderma
renal crisis w malignant HTN
Previously the m/c cz of death in scleroderma
ARF
Why is acute renal failure no longer the cz of death in scleroderma
can be tx'd w ACEI
Heart assoc. features assoc w scleroderma
fibrosis
Limited form of scleroderma
CREST syndrome
CREST syndrome is a dz w skin limited to?
distal extremities and face
What does CREST mean?
C-Calcinosis, R-Raynaud's, E-Esophageal dysmotility, S-Sclerodactyly, T-Telangiectasia
Ab assoc w scleroderma?
scl-70
New name for CREST syndrome?
limited scl or limited cutaneous systemic sclerosis
Calcinosis is a condition in which
calcium deposits occur in tissues (usually fingers) PIPs, knees, elbows
Most involved
knees and elbows
Limited scleroderma usually does not have dz
above the elbow or knee
compared to scleroderma limited dz progress vs diffuse
slowly
Raynaud's phenomenon
episodes of pallor or cyanosis in response to cold or emotional stimuli
Define: hyperemia
rebound blood flow
Primary Raynaud phenomenon is called?
Raynaud's dz
Underlying dz in Raynaud's is called
primary raynaud's
Raynaud's w/o underlying dz is referred to as
secondary raynaud phenomenon
How do you distinguish between primary and secondary Raynaud's phenomenon?
nailfold capillaryoscopy (place oil drop on fingernail base look under)
How do you treat Raynaud's phenomenon severe
Penicillinamine, CCBs, ACEIs
How do you tx HTN ?
HTN
Define: Sjogren
chronic autoimmune disorder charcterize by lymphocytic infiltration of exocrine glands w dry eyes and mouth
Is Sjogren a primary or secondary dz?
either.
Sjogren's may be secondary to?
RA, primary biliary cirrhosis, SLE
Progressive Sjogren's involves?
major organs and progresses into lymphoproliferative dz (malignant lymphoma)
Define keratoconjunctivitis?
dry eyes and mouth
Other sxs assoc w Sjogren's aside from sicca syndrome?
parotid enlargement, dental caries
Dx test for Sjogren's?
Schirmer's test (tear production), Bengal stain (corneal stains), ANA +, anti-la (SSB), anti-Ro (SSA)
Salivary gland bx will show?
lymphocytic infiltration
Tx for dz?
No definitive tx
Define: spondyloarthopathy
group of disorders that share clinical features w HLA-B27
List the the seronegative spondyloarthropathies
ankylosing spondylitis, reactive arthritis, psoriatic arthritis, enteropathic arthropathy
Define: acute spondylitis
disorder primarily affects axial skeleton and peripheral joints
Gender predominance assoc w
male greater than female (3-4 Xs)
Cz?
unknown
90% of pts have
HLA-B27
Ankylosing spondylitis presents w?
chronic lower back pain, young female, morning stiffness x 1 in, improved w exercise
Rarely
c-spine (late)
Common extraartiular dz w ankylosing spondylitis
anterior uveitis, tertiary heart block, aortic insufficiency progresses to CHF
Clinical exam of ankylosing spondylitis reveals?
limited spine mobility, obliterans of the lumbar lordosis, minimal trauma
XR in AS?
sacroilitis
What do spondyloarthropathies have in common?
seronegative, involve lower back, extracurricular, HLA-B27
Rarely involved?
c-spine
Earliest XR evidence of AS
sacroilitis
Late XR evidence of AS
fused sacroiliac
Tx for AS
NSAID, physiotherapy, exercise
Tx for Reactive arthritis
NSAIDS, physical therapy
A severe form of reactive arthritis
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