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
2
Q
ANA patterns
A
- homogeneous
- speckled
- centromere
- nucleolar
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
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
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)
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
7
Q
SLE- multisystem involvement
A
- pleural effusions
- butterfly rash
- heart problems
- lupus nephritis
- arthritis
- raynaud’s
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)
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
10
Q
Anti-phospholipid Ab Syndrome (APS)
A
- 1/3 of SLE pts (secondary APS)
- primary APS
- 3 types of ab’s
11
Q
APS- tx
A
anticoagulant
- warfarin- achieve INR of 2-3
- pregnancy= LMW heparin (enoxaparin) + aspirin
- warfarin contraindicated in pregnancy
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
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
14
Q
Anti-cardiolipin ab’s
A
-directed at a serum cofactor- Beta2GPI
15
Q
DDX for thombosis
A
- APS
- prot C and S def
- antithrombin def
- factor V leiden
- HIT
- sepsis
- systemic vasculitis
- DIC
- TTP
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
Scleroderma- diffuse
- systemic
- proximal extremities and trunk
- early and progressive organ involvement- kidney, cardiac, interstitial lung dz
- worst prognosis
26
Scleroderma- limited
- 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
Scleroderma- localized
- 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
Diffuse Scleroderma- serology
- + ANA
- + Anti-Scl 70 (Anti-topoisomerase I)
- anti-RNA polymerase III
29
Limited Scleroderma- serology
- + ANA
| - + Anti-centromere
30
scleroderma- tx
- no tx alters dz coarse
| - manage organ system involvement
31
Limited Cutaneous Systemic Sclerosis
- 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
Diffuse Cutaneous Systemic Sclerosis
- 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
systemic sclerosis- skin
- hyper/hypo pigmented
- dry/itchy skin (gland obliterated by fibrosis)
- masklike facies/wrinkles
- microstomia
- telangiectasia
- atrophic skin- ulcerations
- calcium deposits
- raynaud phenomenon
34
SS- GI
- 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
SS- pulmonology
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
SS- renal
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
SS- cardiac
- 50% of pts
- myocardial fibrosis
- cardiomyopathy
- pericarditis
- myocarditis
- pericardial effusion
- arrhythmia
38
SS- MSK/ other
- carpal tunnel syndrome
- tendon friction rubs
- fibrosis and adhesion of tendon sheaths
- hypothyroid from thyroid fibrosis
39
Morphea
see picture
