Connective Tissue Disorders and Vasculitides Flashcards
(122 cards)
1
Q
IgA Vasculitis is common in what age group?
A
kids
2
Q
what are the symptoms associated with IgA V?
A
tetrad: 1) palpable purpura (with no thrombocytopenia) 2) arthritis/arthralgia 3)abdominal pain 4) renal disease
3
Q
how do you diagnose IgAV?
A
biopsy, which will show IgA deposits
4
Q
how do you treat IgAV?
A
supportive and glucocorticoids
5
Q
how does a patient with anti-GBM present/ have history of?
A
hematuria or hemoptysis
6
Q
if anti-GBM is not treated, what is the most likely cause of death?
A
pulmonary hemorrhage
7
Q
how do you diagnose anti-GBM?
A
biopsy (renal usually), which shows deposition of anti-basement membrane autoantibodies
8
Q
how do you treat anti-GBM?
A
plasmapheresis (removes abs), glucocorticoids, cyclophosphamide, dialysis sometimes
9
Q
what are the hallmarks of granulomatosis with polyangiitis?
A
granulomatous inflammation, necrotizing vasculitis, segmental glomerulonephritis, nasal involvement (saddle nose)
10
Q
how do you diagnose granulomatosis polyangiitis?
A
history/physical, serology: ANCA, biopsy: vessel changes with granulomas
11
Q
how do you treat granulomatosis polyangiitis?
A
no smoking and high dose glucocorticoids
12
Q
what are the hallmarks of EGPA?
A
asthma+ eosinophilia–> vasculitis with granulomas
13
Q
what are the phases of EGPA?
A
- prodromal phase 2. eosinophilia-tissue infiltration phase 3. vasculitis phase
14
Q
how do you diagnosed EGPA?
A
CBC with high eosinophilia count serology: ANCA: pANCA, lung biopsy shows granulomas and vascular changes, eosinophils in tissues
15
Q
how do you treat EGPA?
A
no smoking and glucocorticoids
16
Q
where is behcet syndrome more commonly seen?
A
turkey, asia, and middle east
17
Q
how does behcet syndrome present when it affects the large vessels?
A
aneurysms
18
Q
how does behcet syndrome present when it affects the veins?
A
DVTs
19
Q
how does behcet syndrome present when it affects the small vessels?
A
triad: 1. recurrent mouth ulcers 2. genital ulcers 3. eye inflammation
20
Q
what is pathergy and what disease is it associated with?
A
pustules at the site of sterile needle pricks; behcet syndrome
21
Q
how do you diagnose behcet syndrome?
A
history and physical exam/ serology: HLA-B51
22
Q
how do you treat behcet syndrome?
A
low dose glucocorticoids
23
Q
how do you diagnose buerger disease?
A
angiography “corkscrew appearance”
24
Q
how do you treat Buerger disease?
A
STOP SMOKING- glucocorticoids and anticoagulation do not work
25
what is polyarteritis nodosa associated with?
HBV
26
how does polyarteritis nodosa affect the skin?
livedo reticularis, subcutaneous nodules, ulcers, and digital gangrene
27
how does polyarteritis nodosa affect the peripheral nerves?
80% have vasculitis neuropathy--> mononeuritis multiplex (foot drop)
28
how does polyarteritis nodosa affect the lungs?
it doesn't--> they are spared
29
how do you diagnose polyarteritis nodosa?
biopsy (fibrinoid necrosis and no granulomas) or angiogram--> micro-aneurysm
30
how do you treat polyarteritis nodosa?
glucocorticoids and treat HBV
31
how does kowasaki syndrome present?
less than 5 years old M>F; Japan; fever, lymphadenopathy, rash strawberry tongue
32
if kowasaki syndrome goes untreated, how do you die?
from coronary involvement --> aneurysm or MI
33
how do you treat kowasaki syndrome?
IVIG within 10 days of symptoms and high does of ASA
34
who is at most risk of takayasu arteritis?
less than 40 year old females, most common in asians
35
how can takayasu arteritis be described?
pulseless disease- obliterate UE peripheral pulses
36
how does takayasu affect the body?
pulmonary involvement, renal artery stenosis leading to HTN, retinopathy (copper wiring infarctions can be seen), and aortic complications: dilations, regurgitation, aneurysm, rupture
37
how do you diagnose takayasu arteritis?
MRI or CT angiography showing long smooth tapered stenosis; biopsy shows granuloma with some giant cells
38
how do you treat takayasu arteritis?
glucocorticoids
39
giant cell arteritis frequently coexists with what?
polymyalgia rheumatica (PMR)
40
who is more at risk for getting GCA and PMR?
females, 40-50, whites>blacks
41
what might the clinical history/symptoms be in a person with GCA?
headache, jaw claudication, visual abnormalities
42
how do you diagnose GCA?
increased ESR, serology: HLA-DR4, temporal artery biopsy is a gold standard
43
what does a temporal artery biopsy show in a patient with GCA?
segmental granulomatous vasculitis with multinucleated giant cells
44
how do you treat GCA?
start glucocorticoids (before biopsy)
45
what happens if GCA is not treated?
blindeness
46
how does polymyalgia rheumatica (PMR) present?
stiffness, soreness, and muscle pain; feelings of weakness as a result of pain but not the objective weakness
47
how do you diagnose PMR?
everything is normal except ESR and CRP
48
how do you treat PMR?
glucocorticoids
49
how do you diagnose raynaud's phenomenon?
Nailfold capillaroscopy: will be normal in primary. secondary: distorted with widened and irregular loops, dilated lumen and areas of vascular dropout
50
what type of hypersensitivity is SLE?
type III
51
what are the hematologic effects of SLE?
anemia, thrombocytopenia, venous or arterial thrombosis
52
what are the cardiopulmonary effects of SLE?
pericarditis, pleuritis, myocarditis, libman-sacks endocarditis, pts also at risk of MI due to accelerated atherosclerosis
53
what are the neurological effects of SLE?
retinopathy (cotton wool spots)
54
what serology testing is used for diagnosing SLE?
dsDNA, smith, and anti-Ro and LA
55
which serology test correlates with disease activity of SLE?
dsDNA
56
when might complement levels suggest SLE?
if C3 and C4 are decreased
57
how do you treat SLE?
NSAIDs, avoid sun exposure, glucocorticoids, hydroxychloroquine
58
what should be considered in the management of SLE?
you should minimize other conventional risk factors for atherosclerosis--> avoid or quit smoking
59
in patients with long term corticosteroid use, what should you monitor for?
avascular necrosis of bone and osteoporosis (DEXA)
60
what are the complications associated with SLE early on?
infections and kidney/CNS disease
61
what are the complications associated with SLE later on?
accelerated atherosclerosis and thromboembolic events
62
what are the three primary antibodies involved in antiphospholipid antibody syndrome?
anti-cardiolipin antibodies (aCL), lupus anticoagulant (LA) and beta 2 glycoprotein 1 (anti-B2 GP1)
63
what is the treatment for anti-phospholipid antibody syndrome?
indefinite systemic anticoagulation
64
what effect does DIL have on the body?
it promotes demethylation of DNA
65
how do you diagnose DIL?
(+) ANA and (+) anti-histone antibodies
66
who is affected by neonatal lupus?
it affects children born of mothers with anti-Ro (SSA) antibodies
67
how does neonatal lupus present?
rashes, thrombocytopenia, hemolytic anemia, arthritis; congenital heart block
68
how do you diagnose neonatal lupus?
anti-Ro in mother
69
how do you treat neonatal lupus?
delivery if distress occurs in fetus, dexamethasone tx of mother when in utero heart block is detected; hydroxychlorquine treatment of anti-ro mother to prevent other fetuses from getting it
70
how does discoid lupus present?
well-defined inflammatory plaques that evolve into atrophic disfiguring scars- most commonly occurs on the head
71
how do you diagnose discoid lupus?
clinical exam and biopsy
72
what does discoid lupus resemble?
tinea infection, psoriasis, and morphea
73
what is the hallmark of scleroderma?
thickening and hardening of the skin; almost all patients have secondary raynaud phenomenon
74
what is the first symptom typically for scleroderma and how does this differ with race?
white patients: raynaud's> pigmentation changes; black patients: pigmentation changes> raynaud's
75
how would you define localized scleroderma?
inflammatory condition that causes hard and thickened localized patches of skin on different areas of the body; no involvement of internal organs (NOT systemic, but may affect joints or muscles in these areas)
76
how does localized scleroderma present?
discreet patches of discolored skin induration; patches= morphea; NO raynaud's; typically asymptomatic, but some individuals may experience itching or pain
77
how do you diagnose localized scleroderma?
it is histologically indistinguishable from other forms of scleroderma
78
how do you treat localized scleroderma?
quite resistant to therapy; can try phototherapy UVA or methotrexate + steroids
79
how would you define limited (cutaneous) scleroderma?
inflammatory condition that causes hard and thickened tissues in the body (some internal organs)
80
how does limited scleroderma present?
indolent course, raynaud's can be one of the first symptoms; CREST syndrome; pulmonary involvement
81
what does CREST stand for?
calcinosis cutis, raynaud's, esophageal dysmotility, sclerodactyly, telangiectasia
82
what is the pulmonary involvement seen in limited scleroderma?
progressive pulmonary artery hypertension (PAH)--> presents as SOB
83
how do you diagnose limited scleroderma?
typically made in advanced disease; serology: (+) ANA and (+) anti-centromere
84
how do you treat limited scleroderma?
no therapy to date--> manage organ system involvement; glucocorticoids show no efficacy in slowing progression and high doses may be associated with development of renal crisis
85
what can be used in a patient with limited scleroderma and pulmonary hypertension?
phosphodiesterase type 5 inhibitor--> may improve cardiopulmonary hemodynamics
86
how would you define diffuse scleroderma?
inflammatory condition that causes hard and thickened tissues in the body, INCLUDING internal organs
87
how does diffuse scleroderma present?
systemic, interstitial lung disease, renal crisis
88
how does interstitial lung disease present?
SOB, dry, course (velcro-like) crackles on auscultation
89
what is a heralding feature of renal disease?
blood pressure elevation
90
how do you diagnose diffuse scleroderma?
(+) anti-scl 70 aka anti (DNA) topoisomerase I; (+) anti-RNA polymerase III; (+) ANA
91
how do you diagnose ILD?
chest x-ray or CT scan of chest: shows ground glass appearance; pulmonary function tests
92
what is a main difference between scleroderma and sjogren?
in scleroderma, the glands are obliterated by fibrosis; in sjogren, there is destruction due to inflammatory process of immune system
93
how do you diagnose pulmonary artery hypertension?
2D echocardiogram or right heart catheterization, which will shoe elevated pulmonary artery pressure
94
those with scleroderma are at an increased risk of developing what?
bronchoaveolar carcinoma
95
what are 3 signs of CKD?
proteinuria, elevation of creatinine, or HTN
96
what defines renal crisis?
malignant hypertension, hemolytic anemia, and progressive renal insufficiency
97
what endocrine disorder is common in patients with scleroderma?
hypothyroidism
98
what MSK disease is common in patients with scleroderma?
carpal tunnel syndrome
99
how would you define sjogren syndrome?
autoimmune, inflammatory destruction of exocrine glands
100
how does sjogren syndrome present?
sicca symptoms- dryness and parotid/salivary gland enlargement
101
sjogren syndrome has a strong association with what?
B cell non-Hodgkin lymphoma (MALT lymphoma)
102
how do you diagnose sjogren syndrome?
serology: (+) ANA, polyclonal hypergammaglobulinemia, (+) Anti SSA/Ro, (+) Anti SSB/LA (never present without Ro); labial salivary gland biopsy
103
what are the three inflammatory myopathies?
dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM)
104
how does dermatomyositis present?
ages 7-15 and 30-60; weakness without sensory symptoms with characteristic skin lesions
105
what are the characteristics skin lesions associated with dermatomyositis?
grotton's patches/papules, heliotrope rash, periungual erythema, v-neck erythema
106
patients with dermatomyositis are at increased risk of what?
occult malignancy
107
what should you do when you make the diagnosis of dermatomyositis?
start looking for occult malignancy
108
how doe labs appear in patients with dermatomyositis?
elevated CK and aldolase
109
what serology is used for the diagnosis of dermatomyositis?
anti Jo-1
110
biopsy in a patient with dermatomyositis shows what?
perimysial and perivascular inflammation, perifascicular atrophy
111
how do you treat dermatomyositis?
glucocorticoids
112
what is the medical term for shawl sign?
poikiloderma
113
how does polymyositis present?
30-50+ years, subacute, proximal muscle weakness, no skin changes
114
what do the labs look like in a patient with polymyositis?
elevated CK and aldolase
115
what serology is used to diagnose polymyositis?
anti-jo1
116
what does biopsy show in a patient with polymyositis?
endomysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another diagnosis
117
how do you treat polymyositis?
glucocorticoids
118
how does inclusion body myositis present?
>40-50, M>F, white> black; weakness: finger flexion or quadriceps weakness
119
what do the labs look like in a patient with inclusion body myositis?
mild elevation or normal CK
120
what is the serology used for inclusion body myositis?
anti-cN1A autoantibodies
121
what does biopsy show in a patient with inclusion body myositis (IBM)?
endomysial inflammation, rimmed vacuoles, invasion of non-necrotic muscle fibers
122
how do you treat inclusion body myositis?
treatment is supportive
