Connective Tissue Disorders And Vasculitis - Dr. McGowen Flashcards
(108 cards)
1
Q
SLE prevalence and what is it
A
multisystem disorder
= F
= black and latino
= autoimmune to nuclear Ags (TYPE 3 hypersensitivity)
2
Q
SLE SX you can see
A
- fever
- malar rash, photosensitivity
- arthritis inflammatory
- Pericarditis (pain laying supine, ST elevation diffuse, crushing sharp CP)
- Libman sacks endocarditits
- thrombosis
3
Q
SLE DX how
A
- dsDNA ABs
- Sm (Smith) ABs
- low C3 / C4
4
Q
SLE TX
A
- avoid sun
- NSAIDs for pain
- Glucocorticoids
- Hydroxychloroquine
5
Q
SLE management for risks and prevention ** 6
A
- SLE increases atheroscleorosis : minimize risk
- no smoking, obesity, htn, hypercholestemia, exercise
- flu vaccine
- pneumonococcal vaccine every 5 years
- higher malignancy risk so screen
- glucocorticoid use = minitor for avascular necrosis in bones
6
Q
Early stage SLE reason for death
A
- infections (opportunistic infections from immunosuppressive TX)
- Kidney + CNS disease *
7
Q
later stage SLE reason for death
A
- Accelerated atherosclerosis from chronic inflammation (%x MI risk)
8
Q
Anti-Phospholipid AB syndrome (APS) : secondary vs primary
A
primary : APS ABs only
secondary : APS and SLE (1/3 patients with SLE have APS
9
Q
Anti-Phospholipid AB syndrome (APS) :
AB serology
A
- Anti- Cardiolipin (aCL)
- Lupus anticoagulant (LA)
- Beta 2 glycoprotein 1 (anti-B2 GPI)
= all 3 testes 2 times at least 12 weeks after
10
Q
Anti-Phospholipid AB syndrome (APS) :
what are risks and what happens
A
- venous and arterial thrombosis
- miscarriage
= prolong PTT clotting time not reversed by plasma (plt free)
11
Q
aCL features
A
+ in APS
= can cause false + syphilis test in non-treponemal test
12
Q
APS tx
A
systemic anticoagulation
13
Q
cotton wool spots are seen in
A
SLE
APS
DM
systemis HTN
14
Q
Lupus - Like syndrome / Drug Induced Lupus (DIL)
happens how and drugs
A
= drug induce demethylation of DNA
- procainamide
- Hyrdalazine
- Isoniazid
- Sulfasalazine
- Hydrochlorothiazide
15
Q
Lupus - Like syndrome / Drug Induced Lupus (DIL) DX and TX
A
- ANTI- histone ABs
2. stop medication
16
Q
Neonatal lupus happens how
A
cutaneous lesions look like SLE, only this is NOT SLE
= mother can have Sjögrens, SLE, or normal
= mother has Anti- Ro (SSA) ABs**
17
Q
Neonatal lupus risks
A
- ***congenital heart block = life threatening
- hemolytic anemia, thrombocytopenia, rashes, arthritis
18
Q
Neonatal lupus DX
A
+ Anti-Ro mother
19
Q
Neonatal lupus TX
A
- Delivery when fetus is in distress
- Dexamethasone when fetus has 1st or 2nd degree heart block can prevent progression
- Hydroxychloroquine : prevents in future child only
20
Q
Discoid Lupus is what
A
part of or not part of SLE
=skin well-defined inflammation plaques —-> atrophic, disfiguring scars (usually on head)
21
Q
Discoid Lupus DX and Tx
A
- Biopsy
2. photo-protection + topical anti-inflammatory / anti-malaria drugs
22
Q
Discoid Lupus can look like what 3 things
A
- ring worm (tinea)
- Psoriasis
- Morphea (local scleroderma)
23
Q
Scleroderma (SSc) 3 types
A
- local
- Limited (CREST)
- Diffuse
24
Q
hallmark of SSc
A
thickening and hardening of skin
= multisystemic
= Raynaud Phenomenon (pigment changes seen first)
25
Localized scleroderma is what
localized patches of skin on different parts of body (joints, muscles can be involved)
26
Localized scleroderma SX details
MORPHEA (patches)
= discreet area of discolored skin
= red/purple oval --> thickens --> central hypopigmentation + erythematous border around
= asymptomatic , itching, pain
27
Localized scleroderma TX
resistant to tx, immunosuppressives, PT of joints
28
Limited (cutaneous) Scleroderma is what and SX
= hardening and thickening inflammatory condition involving internal organs also
1. C : Calcinous cutis (calcification)
2. R : Raynoud's (digital ischemia)
3. E : Esophageal dysmotilty
4. S : Sclerodactyly
5. T : Telangiectasia
= progressive Pulmonary Artery HTN**** SOB
29
Limited (cutaneous) Scleroderma DX
1. + Anti - centromere *
2. Right heart catheterization for PAH (gold standard)*
3. esophagus barium swallow , hand xray
30
Limited (cutaneous) Scleroderma TX
manage each organ system
1. warm clothing
2. CCB (raynauds)
3. ACE inhibitors (control htn)
4. glucocorticoids (slow SSc****) can cause renal crisis
5. Phosphodiesterase Type 5 inhibitor : Pulm htn**
31
Diffuse (Cutaneous) SSc is what and sx 4
= inflammatory condition thickening of tissues includes internal organs
1. extr. + trunk
2. Interstital Lung Disease ****, dry velcro-like crackles, SOB
3. Renal crisis
4. Raynauds phenomena later stages
32
Diffuse (Cutaneous) SSc DX
1. + Anti- scl 70 (Anti-DNA topoisomerase 1)
2. Anti-RNA polymerase 3
3. ILD : interstitial lung d, CXRay, CT
33
ILD
```
interstitial lung disease
= dry velcro like crackles
= SOB
= CT : ground glass or honeycomb looking
= Pulmonary Function test
```
34
Diffuse (Cutaneous) SSc TX
1. ILD : glucocorticoids, O2 + Pulmonary rehab = renal crisis can happen
2. manage each organ system like in limited SSc
35
Systemic Sclerosis : SKIN
= hyper or hypo pigment
| = fibrosis of skin glands (dry itchy)
36
Systemic Sclerosis : Pulmonary
1. ILD : DIFFUSE SSc, Dx with PFT or CT, Auscultation velcro crackles
2. PAH : pul artery htn, LIMITED SSc, dx right heart catheterization
3. high risk bronchoalveolar carcinoma
37
Systemic Sclerosis : Renal
chronic kidney disease , renal crisis (Diffuse, when using glucocorticoids)
38
Systemic Sclerosis :Endocrine
hypothyroid from thyroid fibrosis
39
Systemic Sclerosis : MSK
Carpal tunnel : fibrosis on tendon sheaths
40
Systemic Sclerosis : Cardiac
Myocardial fibrosis, Cardiomyopathy, arrhythmia, pericarditis, pericardial effusion
41
Systemic Sclerosis : GI
Xerostomia, Esophagus = GERD and dysphagia
42
Sjögren Syndrome prevalence and what
autoimmune to exocrine glands
| postmenopause, F
43
Sjögren Syndrome SX
1. sicca sx : dry eyes, mouth , oral infaction, cavitites, parotid and other salivary gland enlargement
2. Keratoconjunctivits sicca : X tears
44
Sjögren Syndrome risk
B-cell non-Hodgkin lymphoma (MALToma)
45
Sjögren Syndrome DX
1. + Anti SSA/Ro
2. Anti SSB/La
3. Polyclonal Hypergammaglobuulinemia
4. Labial salivary gland (LIP) biopsy, if unilateral gland enlargement bx it
46
Sjögren Syndrome TX
tx problems, artificial tears, infection, drink water more
47
Inflammatory myopathies 3 types
1. Dermatomyositis (DM)
2. Polymyositis (PM)
3. Inclusion Body Myositis (IMB)
48
Inflammatory myopathies most common sx
bilateral proximal muscle weakness
| = hard time standing from chair or bathtub or going up stairs
49
Inflammatory myopathies dx typical
high CK and adolase
| ESR and CRP is normal
50
Dermatomyositis what happens, SX
1. weakness with no sensory sx (early proximal, later distal)
2. skin lesions : Gottron's Patches / papules, Heliotrope rash, V-neck erythmia
51
Dermatomyositis risk
occult
malignancy
= when dx do age appropriate screening
52
Dermatomyositis DX
1. high CK (creatine Kinase)
2. high aldolase
3. Anti Jo-1
4. Biopsy : perimysial and perivascular inflammation = perifascicular atrophy
53
Dermatomyositis tx
glucocorticoids
54
Gottron's patches and papules
raised violet lesions bumps over dorsal hand of the finger joints
55
Heliotrope rash
purple red edema over upper eyelids
56
V-neck erythema
Poikiloderma : "Shawl sign" red over neck and shoulders upper chest and back
57
Polymyositis what happens sx
no skin changes,
| proximal muscle weakness
58
Polymyositis dx
1. high CK and adolase
2. Anti Jo-1
3. Biopsy : endomysial inflammation + non-necrotic muscle fibers, no other disease sxs (IBM or Muscular dystrophy)
59
Polymyositis TX
glucocorticoids
60
Inclusion Body myositis (IBM) what happens sx and prevalence
```
more in M (only one)
and white (others were black)
```
weakness in finger flexion and quadriceps
61
Inclusion Body myositis (IBM) DX
1. mild elevated CK
2. Anti -cN1A (dont have to know)
3. Biopsy : endomysial inflammation + rimmed vacuoles*, invasion of non-necrotic muscle fibers
62
Inclusion Body myositis (IBM) TX
resistant to tx
| support with pain killers and Physical therapy
63
IGA vasculitis other name and DX
Henoch- Schönlein purpura (HSP)
| = IgA deposits on biopsy
64
Henoch- Schönlein purpura (HSP) TETRAD
Henoch- Schönlein purpura (HSP)
1. Palpable purpura (not thrombocytopenia)
2. Arthritis, arthralgia
3. ABD pain
4. Glomerulonephrits, renal disease
65
Henoch- Schönlein purpura (HSP) TX and prevalence
kids more common, in small vessels
| = support + glucocorticoids
66
Anti-Glomerular Basement Membrane other name and what happens
GOODPASTURE SYNDROME
1. Hematuria (glomerular capillaries)
2. Hemoptosis (pulmonary capillaries can cause hemorrhage)
67
GOODPASTURE SYNDROME DX
renal bx : Anti-BM ABs
| in small vessels
68
GOODPASTURE SYNDROME TX
1. plasmapheresis (remove ABs)
| 2. Glucocorticoids
69
Granulomatosis with polyangitis (GPA) other name and prevalence
WEGENER'S GRANULOMATOSIS
| = MALE*, over 40yo
70
WEGENER'S GRANULOMATOSIS what happens
1. Granulomatous inflammation
2. necrotizing vasculitits
3. segmental glomerularnephritis
4. 90% NASAL involvment (saddle nose bleeding crusting)
5. lung cavitary lesions = alveolar hemorrhage
6. venous thrombotic events
71
WEGENER'S GRANULOMATOSIS dx
1. ANCA +
| 2. biopsy is granulomas in small vessels
72
WEGENER'S GRANULOMATOSIS TX
stop smoking, glucocorticoids high dose
73
Eosinophilic Granulomatosis with Polyangitis (EGPA) other name and TX
Churg- Strauss Syndrome
| = no smoking, glucocorticoids
74
Churg- Strauss Syndrome is what
resp tract
| = asthma + eosinophilia ----> vasculitis with granulomas
75
Churg- Strauss Syndrome 3 phases
1. prodromal : arthma, allergy, months to years
2. eosinophilia - tissue infiltration : high E in blood, go to lungs and GI and others
3. Vasculitis : systemic necrotizing heart, lungs, nerves, skin + Palpable purpura
76
Churg- Strauss Syndrome DX
1. high E count
2. ANCA+ usually (MPO-ANCA+ = pANCA+)
3. bx lung : granulomas and vascular changes, E in tissue
77
Churg- Strauss Syndrome vessels
small and medium
78
Behcet Syndrome prevalence and DX
1. Turkey, Asia, Mid East
| 2. HLA -B51
79
Behcet Syndrome what is it and triad
```
=Large vessel aneurysms
=DVT
1. recurrent mouth ulcers
2. genital ulcers
3. eye inflammation uveitis
= can look like MS (neuro) or Chrons (GI)
= pustules at site of needle injection
```
80
Behcet Syndrome tx
low dose glucocorticoids
81
Thromboangiitis Oblitirans other name and prevalence
BUERGER Disease
| = MALES, under 45yo
82
BUERGER Disease what happens
only in people who smoke
| = loss of digits
83
BUERGER Disease DX and Tx
1. corkscrew appearence on agiography , recent tobacco use, under 45yo
2. Stop smoking
84
Polyarteritis nodosa prevalence and vessel type
medium BV
| = HBV, MALE
85
Polyarteritis nodosa SX
1. fever, livedo reticularis, ulcers, digital gangrene
2. 80% = vasculitis neuropathy (foot drop)
3. renin made HTN
4. caridiac problem can cause HTN
5. NO LUNG PROBLEMS
86
Polyarteritis nodosa DX
1. bx: fibrinoid necrosis , no granulomas
2. angiogram : micro-aneurysm
3. HBV
87
Polyarteritis nodosa tx
glucocorticoids, tx HBV
88
Kawasaki Disease other name and prevalence
MUCOCUTANEOUS LN SYNDROME
| - MALE, under 5yo, JAPAN
89
MUCOCUTANEOUS LN SYNDROME tetrad
1. fever
2. lymphadenopathy
3. rash
4. strawberry tongue
= red eyes, pealing skin around nails
90
MUCOCUTANEOUS LN SYNDROME DX
clinical features
91
MUCOCUTANEOUS LN SYNDROME risk
coronary artery aneurysm of MI
92
MUCOCUTANEOUS LN SYNDROME TX
IVIG within 10days
| high dose ASA (Asprin)
93
Takayasu Arteritis vessel typer and prevalence
= large
| = under 40yo, F, AORTA, asia
94
Takayasu Arteritis what happens
AORTA or branches involved
= limb pulseless (only collaterals made to prevent ischemia)
= Renal artery stenosis, HTN
= Retinopahty : copper wire infarctions)
= Aortic dilation, regurg, aneurysm, rupture
= lung probs
95
Takayasu Arteritis DX
1. MRI, CT angiogram = long smooth tapered stenosis (atherosclerosis looks irregular lesions)
2. BX : granuloma with giant cells
96
Takayasu Arteritis tx
glucocorticoids
97
Giant cell arteritis (GCA) associated with
Polymyalgia Rheumatica (PMR)
98
Giant cell arteritis (GCA) other name , vessel type
```
Temporal Arteritis (TA)
large BV
```
99
Giant cell arteritis (GCA) what happens
cranial arteries involved (Temporal A)
1. dull headache, tender temporal A upon palpation
2. jaw pain while chewing
3. PMR association
4. amaurosis fugax or diplopia : vision problems
100
Giant cell arteritis (GCA) DX
1. HIGH ESR (over 50mm/h)
2. HLA- DR4
3. Temporal A BX ****: segmented granulomatous vasculitis + multinucleated giant cells ****
101
Giant cell arteritis (GCA) TX
TREAT GLUCOCORTICOIDS BEFORE anything else including bx
| they can go blind if not treated
102
Polymyalgia Rheumatica (PMR) is what
1. stiffness, soreness and muscle pain = causing untrue muscle weakness sx (only muscles are normal in strength and everything, just from pain)
2. pain putting coat on, combing hair, shoulder, neck, pelvis
103
```
Polymyalgia Rheumatica (PMR)
DX
```
1. high ESR and CRP
2. no inflammation in muscles,
3. normal EMG (elecromyography)
104
```
Polymyalgia Rheumatica (PMR)
tx
```
glucocorticoids
105
Raynauds Phenomenon what happens
episodic from cold / stress = primary , from other disease cause = secondary + more ischemia
1. pallor (vasoconstriction) white
2. Cyanosis (ischemia) blue
3. erythema (reperfusion) red
106
Raynauds Phenomenon age
primary 15-30
| secondary over 30
107
Raynauds Phenomenon dx
Naifold capillaroscopy : normal in primary
| = distorted + widened and irregular loops , dilated lumen and vascular "dropout" areas in secondary
108
Raynauds Phenomenon tx
wear gloves, stay warm, lotion, dont smoke, limit sympathetic drugs
CCB
secondary : tx underlying cause
