hydroxychloroquine - fight SLE - stop anti-phospholipid Ab formation

Connective Tissue Disease - Darrow Flashcards by Matt Stanton

autoimmune diseases

linked to HLA antigens

class I - A, B, C
class II - D

class I - men
class II - female

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SLE

in black women
ANA, HLA-B8, DR2 and DR3 positive

seen with complement deficiency

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arthralgia, rash on face, lung field rub, proteinuria, anemia

SLE

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elevated ESR and normal CRP

in SLE

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pericarditis

in SLE

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diagnosis of SLE

requires 4 of 11 criteria

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criteria for diagnosis of SLE

B3 O1 R1 N1
with
D3ermA1titiS1

B3 - blood
1 - heme disorder - anemia, leukopenia, thrombocytopenia
2 - immunologic disorder - ds-DNA, Sm, APA, FPSTS
3 - ANA

O1
4 - oral ulcers

R1
5 - renal disorders - proteinuria or cellular casts

N1
6 - neurologic disorders - seizure or psychosis

D3
7 - malar rash
8 - discoid lupus
9 - photosensitivity

A1
10 - arthritis

S1
11 - serositis - pleuritis or pericarditis

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speckled and rim pattern

in SLE

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seizure of psychosis

in SLE

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smoking

makes SLE rash worse

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cough up blood

alveolar hemorrhage

-associated with SLE

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non-infectious endocarditis

libman sacks

-associated with SLE

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tx of SLE

hydroxychloroquine

fight SLE
stop anti-phospholipid Ab formation

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before making diagnosis of SLE in patient

exclude drug induced lupus

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splinter hemorrhages

with bacterial endocarditis

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drug induced lupus

slow acetylators - if inhibit DNA methylation T cells - become autoreactive

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sx with drug induced lupus

no renal disease
no CNS disease

+ anti-histone Abs

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drugs triggering autoimmunogenicity

hydralazine
isoniazid
procainamide
methimazole
prophylthiouracil
etanercept

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anti-histone Abs

drug induced lupus

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CI in pregnancy

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safe in pregnancy

azathioprine and HCQ

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SLE vs. preeclampsia

abnormal liver test and high uric acid - preeclampsia

dsDNA, low C3/C4 and normal uric acid and liver tests - SLE

both have HTN, proteinuria, and thrombocytopenia

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RO/SSA patient daughter born with

heart block

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ring like or snake like plaque with central clearing

SCSLE - subacute cutaneous lupus erythematosis
-characteristic rash

RO-SSA and heart block in offspring

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lupus anticoagulant

worst prognosis venous and arterial clotting antiphospholipid antibody syndrome

diagnosis of antiphospholipid antibody syndrome

1 clinical and 1 lab criteria - 3 months apart lab - anticardiolipid Ab - lupus anticoagulant - anti-beta2 glycoprotein 1 clinical - spontaneous abortion - vascular abortion

best correlation with thrombotic events in antiphospholipid Ab syndorme

lupus anticoagulant

russel viper venom test

confirmation of antiphospholipid Ab syndrome

tx with hydroxychloroquine

need to do retinal screening

small vessel vasculitis

cryoglobulinemia giraffe type rash - livedo reticularis

cause of livedo reticularis

cryoglobulinemia

tightening skin and fibrosis

scleroderma

progressive systemic sclerosis

scleroderma | -systemic

onion skinning of renal arteries

scleroderma

GI sx of scleroderma

stay below sternum

fibroblast metabolic defect with increased collagen

scleroderma autoimmunity can be caused by silica**

diagnosis of scleroderma

1 major or 2 of 3 minor major - thick, tight, induration of skin proximal to MCP minor - sclerodactyly - digital pitting - bibasilar pulmonary fibrosis

high probability of scleroderma

with 3 of 5 crest featuresq

first sign of scleroderma

raynauds

signs of scleroderma

``` raynauds nonpitting edema subQ calcifications finger tip ulcerations telangiectasia ```

tendon friction rub

scleroderma

raynauds

female | -precipitated with cold, stress, drugs

finger loss

anti-centromere

digital ulcer

anti-topoisomerase

<30yo, normal sed rate, no nail bed capillary changes

primary raynauds

visceral changes in scleroderma

linked to extent of skin thickening

what kills you in scleroderma

kidney failure with HTN -until ACE inhibitors now - lung issues kill you

anti-RNA polymerase III

bad Ab in scleroderma onion skinning - renal disease**

stop progression of scleroderma

ACE inhibitors

anit-SCL-70

scleroderma interstitial lung disease**

scleroderma risk fx

anti-centromere Ab and anti-B23

scleroderma pulmonary HTN

dysphagia cause

scleroderma for solids and liquids

limited scleroderma

CREST syndrome

induces fibrosing dermopathy

gadolinum prep for X-ray or CAT scan

trauma, following exercise, M protein

eosinophilic fasciitis

shulmans syndrome

eosinophilic fasciitis

plantar fascitia and polyarthritis syndrome

associated with malignancy** is a paraneoplastic syndrome fibroblast proliferation due to TGF-B ``` pain, stiff, swollen DIP and PIP thick palmar fascia thick plantar fascia ANA negative no RP ```

polymyositis and dermatomyositis

proximal muscle weakness

inclusion body myositis

distal muscle weakness

dermatomyositis

CD4 - B cells

polymyositis and inclusion body myositis

CD8 T cells

very high CPK

dermatomyositis

heliotrope rash, shawl sign, gottrons papules, nailfold capillary loop

dermatomyositis

scleroderma dysphagia

low in esophagus - smooth m

dermatomyositis dysphagia

high in esophagus - skeletal m

specific diagnosis of dermatomyositis

perivascular infiltrate with cap necrosis and fiber destruction

shawl sign

in dermatomyositis association with malignancy**

anti-Jo1 dermatomyositis

lung involvement**

anti-p155/140

associated with cancer also shawl sign

anti-U1 RNP

mixed connective tissue disease | -aka overlap syndrome

pt on ventilator, sepsis forms, high blucose, flaccid distal and proximal muscle groups, absent DTRs

critical illness myopathy neuropathy

sjogrens

T cell CD4 - infiltrate gland anti-Ro and anti-La produced by B cells

cryoglobulins

small vessel vasculitis in sjogrens

Ab to type II collagen

relapsing polychondritis polyarthritis across wrist and MCPs, also eye and ear involvement

IgG4 releated disease

relapsing polychondritis

obliterative phlebitis

inflammation of well of vein that lumen obstructed in relapsing polychondritis IgG4 related disease

large joint arthritis, night sweats, anorexia, diarrhea, weight loss, lymphadenopathy, hepatosplenomegaly, hyperpigmentation

whipples disease

stages of whipples

prodrome - migratory arthritis steady state - diarrhea and weight loss

PAS positive macrophages

whipples disease

hyperpigmentation

niacin decrease aka pellagra

Connective Tissue Disease - Darrow Flashcards

(81 cards)