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M2F > CT diseases 1 > Flashcards

CT diseases 1 Flashcards

1
Q

+ANA

A
  • autoimmune diseases
  • not specific for individual autoimmune disorders
  • titer of <1:40 is normal/negative- higher the ratio= positive/clinically significant!!
  • most are F>M
  • cANCA= PR3-ANCA
  • pANCA= MPO-ANCA
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2
Q

ANA patterns

A
  • homogeneous
  • speckled
  • centromere
  • nucleolar
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3
Q

ANA- homogenous staining pattern

A
  • entire nucleus is diffusely stained
  • ab against chromatin, histone proteins, DNA
  • drug-induced SLE
  • Sjogren’s syndrome
  • SLE
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4
Q

ANA- speckled staining pattern

A
  • fine or coarse speckles are seen thruout the nucleus
  • many diff ab’s- against UI RNP, Sm, La antigens
  • mixed CT dz
  • diffuse systemic sclerosis
  • sjogren’s syndrome
  • SLE
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5
Q

ANA- centromere pattern

A
  • 30-60 uniform speckles thruout the nucleus of resting cells
  • in mitotic cells, the speckles localize to the chromosomes at the metaphase plate
  • limited systemic sclerosis (CREST)
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6
Q

ANA- nucleolar pattern

A
  • homogenous or speckled staining of nucleolus
  • ab against RNA- fibrillarin, RNA polymerase I and III, Th, Pm-Scl, RNA helicase
  • Diffuse systemic sclerosis
  • SLE
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7
Q

SLE- multisystem involvement

A
  • pleural effusions
  • butterfly rash
  • heart problems
  • lupus nephritis
  • arthritis
  • raynaud’s
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8
Q

SLE- is what

A
  • multisystem
  • infl autoimmune disorder
  • autoab’s to nuclear ag’s (immune complexes- type III hypersensitivity)
  • remissions and relapses
  • heterogenous (varies from pt to pt)
  • F>M
  • Af Ams and hispanics > whites
  • socioeconomic factors (poor, no insurance)
  • genetic and environmental factors- UV light, viruses (EBV)
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9
Q

SLE serology

A
  • +ANA
    • anti-ds DNA- correlate w dz activity
    • Sm- does not correlate w dz activity
  • C3/4 dec- inc consumption during dz activity
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10
Q

Anti-phospholipid Ab Syndrome (APS)

A
  • 1/3 of SLE pts (secondary APS)
  • primary APS
  • 3 types of ab’s
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11
Q

APS- tx

A

anticoagulant

  • warfarin- achieve INR of 2-3
  • pregnancy= LMW heparin (enoxaparin) + aspirin
  • warfarin contraindicated in pregnancy
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12
Q

3 types of antiphospholipid ab’s

A
  • Type 1- biologic false + tests for syphilis
  • Type 2- lupus anticoagulant
  • Type 3- anti-cardiolipin ab’s
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13
Q

Lupus anticoagulant

A
  • risk factor for venous and arterial thrombosis and miscarriage
  • prolongation of aPTT
  • confirmed by abnormal Russel viper venom time (RVVT)- corrects w addition of phospholipid but not normal plasma
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14
Q

Anti-cardiolipin ab’s

A

-directed at a serum cofactor- Beta2GPI

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15
Q

DDX for thombosis

A
  • APS
  • prot C and S def
  • antithrombin def
  • factor V leiden
  • HIT
  • sepsis
  • systemic vasculitis
  • DIC
  • TTP
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16
Q

Cotton wool spots- DDx

A
  • HTN
  • diabetes
  • ischemia
  • embolic
  • infectious
  • toxic
  • radiation-induced
  • neoplastic
  • traumatic
  • immune-mediated- SLE/APS
  • idiopathic
17
Q

Lupus-like syndrome/ Drug induced

A
  • promote demethylation of DNA
  • no renal or neurologic sx’s
  • +ANA
    • anti-histone ab’s
  • sulfa abx- SLE flare!!!
18
Q

Lupus- pregnancy and neonatal

A
  • gestational HTN (preeclampsia), fetal growth restriction, fetal distress- fetal loss or premature delivery
  • neonatal lupus- affects children born of mothers w Anti-Ro (SSA) or LA (SSB) abs (1-2%)- transient- rashes, thrombocytopenia, hemolytic anemia, arthritis- permanent complete heart block
19
Q

SLE- tx

A
  • avoid sun exposure, wear sunscreen
  • NSAIDs
  • corticosteroids (topical or systemic)
  • hydroxychloroquine
20
Q

SLE DDx

A
  • RA
  • systemic vasculitis
  • scleroderma
  • infl myopathies
  • viral hepatitis
  • sarcoidosis
  • acute drug rxns
21
Q

SLE- clinical

A
  • malar rash

- libman-sacks endocarditis

22
Q

Discoid lupus- DDx

A
  • tinea infection (ringworm)
  • psoriasis
  • morphea (limited scleroderma)
23
Q

Scleroderma (Systemic sclerosis)

A
  • 30-60 yo, W>M
  • progressive (inc severity in Af Ams)
  • hallmark- thickening and hardening of skin; fibrosis of skin and visceral organs
  • skin, lungs, GI, kidney, MSK, herat
  • secondary Raynaud phenomenon in all pts
  • obliteration of eccrine sweat and sebaceous glands- dry and itchy skin
  • tx- control sx’s and slow progression
24
Q

Scleroderma- types

A
  • diffuse
  • limited
  • localized
25
Q

Scleroderma- diffuse

A
  • systemic
  • proximal extremities and trunk
  • early and progressive organ involvement- kidney, cardiac, interstitial lung dz
  • worst prognosis
26
Q

Scleroderma- limited

A
  • fingers, toes face/neck, distal extremities
  • Raynaud’s commonly precedes other sxs
  • pulmonary HTN
  • CREST syndrome:
  • Calcinosis cutis
  • Raynaud’s (usually 1st sx)
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
  • indolent course, good prognosis
27
Q

Scleroderma- localized

A
  • benign skin conditions
  • affect children
  • discreet areas of discolored skin induration
  • NO raynaud’s
  • NOT systemic
  • histo indistinguishable from SSc
  • patches= morphea
  • coalesced patches= generalized morphea
28
Q

Diffuse Scleroderma- serology

A
    • ANA
    • Anti-Scl 70 (Anti-topoisomerase I)
  • anti-RNA polymerase III
29
Q

Limited Scleroderma- serology

A
    • ANA

- + Anti-centromere

30
Q

scleroderma- tx

A
  • no tx alters dz coarse

- manage organ system involvement

31
Q

Limited Cutaneous Systemic Sclerosis

A
  • long-standing Raynaud’s
  • indolent course
  • GERD, telangiectasia, cutaneous calcinosis
  • vascular manifestations are more pronounced (than diffuse)- digital ischemia and pulm a HTN
  • renal crisis is uncommon
32
Q

Diffuse Cutaneous Systemic Sclerosis

A
  • soft tissue swelling, erythema, pruritus
  • fatigue, stiffness, malaise
  • arthralgia, m weakness, carpal tunnel
  • Raynaud (later)
  • infl edematous phase- fibrotic phase: skin induration, hyperpigmentation, loss of body hair, impaired sweating; fibrotic joints- stiffness
  • Renal crisis- may see hemolytic anemia during crisis
33
Q

systemic sclerosis- skin

A
  • hyper/hypo pigmented
  • dry/itchy skin (gland obliterated by fibrosis)
  • masklike facies/wrinkles
  • microstomia
  • telangiectasia
  • atrophic skin- ulcerations
  • calcium deposits
  • raynaud phenomenon
34
Q

SS- GI

A
  • malnutrition- fat, B12, vit D def
  • xerostomia
  • GERD, dysphagia, barrett esophagus (inc risk of esophgeal adenoCA)
  • gastroparesis
  • GAVE (watermelon stomach)
  • chronic diarrhea from bacterial overgrowth
  • pseudo-obstruction
  • primary biliary cirrhosis (anti-mitochondrial ab)
35
Q

SS- pulmonology

A

primary cause of morbidity and mortality

  • aspiration pneumonia (GERD)
  • interstitial lung dz- DIFFUSE- chronic dry cough, dyspnea, velcro crackles- dx by PFT
  • pulm a HTN- LIMITED- mean pulm a P >25mmHg- right heart cath to confirm dx!!
  • inc risk of bronchoalveolar carcinoma
36
Q

SS- renal

A

CKD

  • renal crisis uncommon but life-threatening- abrupt onset of malignant HTN, hemolytic anemia, progressive renal insuff
  • more common in DIFFUSE SSc
  • tx- avoid high dose corticosteroids
37
Q

SS- cardiac

A
  • 50% of pts
  • myocardial fibrosis
  • cardiomyopathy
  • pericarditis
  • myocarditis
  • pericardial effusion
  • arrhythmia
38
Q

SS- MSK/ other

A
  • carpal tunnel syndrome
  • tendon friction rubs
  • fibrosis and adhesion of tendon sheaths
  • hypothyroid from thyroid fibrosis
39
Q

Morphea

A

see picture

M2F (15 decks)

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