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Flashcards in Rhumatology Deck (17):
1

common lab findings in polymyosits and dermatomyositis:

increased CK
+ANA
+anti-Jo-1 antibodies
+anti-SRP antibodies
+anti-Mi-2 antibodies

2

what disorder is characterized by progressive symmetric proximal muscle weakness associated with endomysial inflammation with CD8+ T cells

-most commonly involves the sholders

polymyositis

3

what differentiates dermatomyositis from polymyositis?

Dermatomyositis has
>malar rash
>Gotton papules (erythematous bumps on hands)
- "mechanics hands"
>heliotrope rash (periorbital erythematous rash)
>shawl and face rash

>perimesial inflammation with CD4+ T cell vs CD8+ T cells in polymyositis

4

What is the triad of scleroderma?


autoimmunity
non-inflammatory vasculopathy
collagen deposition with fibrosis

-commonly presents with:
puffy, tight skin without wrinkles
pitting of fingertips (not finger nails)
-slcerosis of renal, pulmonary, cardiovascular, GI systems

75% of cases occur in females

2 major types:

5

What markers indicate diffuse vs limited scleroderma?

Diffuse scleroderma - anti-Scl-70 (anti-DNA topoisomerase I) antibodies

Presents with wide spread skin involvement, rapid progression, early visceral involvement

Limited scleroderma - anti-centromere antibody

Presents with limited skin involvement confined to the fingers and face - can have CREST syndrome

6

What is CREST syndrome? What disease is it associated with?

Calcinosis
Raynaud phenomena
Esophageal dysmotility
Sclerodactyly
Tangiectasia

Associated with limited varient of scleraderma (+anti-centromere antibody)

7

What is the leading cause of death from Scleroderma?

pulmonary fibrosis (esophogyeal dysmotility is most common clinical symptom)

8

Pt presents with widespread non-caseating granulomas, elevated serum ACE, and elevated CD4+/CD8+ T cell ratio in bronchiolar lavage fluid
-asymptomatic except for enlarged lymph nodes
CXR/CT shows bilateral hilar and mediastinal adenopathy with coarse reticular opacities

Disease progresses to restrictive lung disease with interstitial fibrosis
erythemia nodosum,
Bell palsy,
Hypercalcemia

Sarcoidosis

most common:

african american woman w/

lymphadenopathy
+
pulmonary fibrosis

Treatment = steroids

9

What lab findings are expected in PT with sarcoidosis?

high serum ACE
elevated CD4/CD8 ratio on bronchiolar lavage fluid

10

55 YO female presents with pain and stiffness in the shoulders and hips, with fever, malaise and weight loss but no muscular weakness. Symptoms rapidly improve with low dose coritcosteroids.

Labs show increased ESR, elevated CRP, but normal CK.

What is the diagnosis?
What disease is associated with this condition?

polymylagia rhumatica

temporal (giant cell) arteritis

11

What antibody is sensitive for drug induced lupis caused by hydralazine or procainamide?

anti-histone antibody

12

what does the presence of anti-dsDNA antibodies in pt with SLE indicate?

specific for poor prognosis renal disease

diffuse proliferative glomerulonephritis - most common, most severe
-presents with nephritic (+/- nephrotic) syndrome showing wire-looping of glomerular capilaries, SUBENDOTHELIAL deposits of IgG containing immune complexes + C3 deposition, granular immunofleorescence

less common presentation is with secondary membranous glomerlulonephritis/nephropathy (nephrotic disease - NOT anti-PLA2R+)
-diffuse capillary and GBM thickening with granular appearance on IF
-EM shows spike and dome appearance with SUBEPITHELIAL depositis

13

What antibody is specific but not prognostic for SLE?

anti-Smith antibodies directed against snRNPs

14

What population is most affected by SLE?

females of reproductive age
african americans

15

Pt.s lab results are positive for:
lupis anti-coagulant,
anticardiolipin antibodies, and
anti-beta2 glycoprotein antibodies

what else is required for Dx?

Dx of antiphospholipid syndrome is based on clinical criteria:

-history of thrombosis (arterial or venous) or spontaneous abortion along with lab findings of auto-antibodies

16

presence of anti-cardiolipin antibodies and lupis anticoagulant can cause false positive ______ and prolonged ______

false positive VDRL/RPR and prolonged PTT

17

What disease is associated with anti-U1 RNP antibodies (speckled ANA)

mixed connective tissue disease
- features of SLE, systemic sclerosis (diffuse scleroderma) and/or polymyositits

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