Connective Tissue Disorders and Vasculitides Flashcards
IgA Vasculitis is common in what age group?
kids
what are the symptoms associated with IgA V?
tetrad: 1) palpable purpura (with no thrombocytopenia) 2) arthritis/arthralgia 3)abdominal pain 4) renal disease
how do you diagnose IgAV?
biopsy, which will show IgA deposits
how do you treat IgAV?
supportive and glucocorticoids
how does a patient with anti-GBM present/ have history of?
hematuria or hemoptysis
if anti-GBM is not treated, what is the most likely cause of death?
pulmonary hemorrhage
how do you diagnose anti-GBM?
biopsy (renal usually), which shows deposition of anti-basement membrane autoantibodies
how do you treat anti-GBM?
plasmapheresis (removes abs), glucocorticoids, cyclophosphamide, dialysis sometimes
what are the hallmarks of granulomatosis with polyangiitis?
granulomatous inflammation, necrotizing vasculitis, segmental glomerulonephritis, nasal involvement (saddle nose)
how do you diagnose granulomatosis polyangiitis?
history/physical, serology: ANCA, biopsy: vessel changes with granulomas
how do you treat granulomatosis polyangiitis?
no smoking and high dose glucocorticoids
what are the hallmarks of EGPA?
asthma+ eosinophilia–> vasculitis with granulomas
what are the phases of EGPA?
- prodromal phase 2. eosinophilia-tissue infiltration phase 3. vasculitis phase
how do you diagnosed EGPA?
CBC with high eosinophilia count serology: ANCA: pANCA, lung biopsy shows granulomas and vascular changes, eosinophils in tissues
how do you treat EGPA?
no smoking and glucocorticoids
where is behcet syndrome more commonly seen?
turkey, asia, and middle east
how does behcet syndrome present when it affects the large vessels?
aneurysms
how does behcet syndrome present when it affects the veins?
DVTs
how does behcet syndrome present when it affects the small vessels?
triad: 1. recurrent mouth ulcers 2. genital ulcers 3. eye inflammation
what is pathergy and what disease is it associated with?
pustules at the site of sterile needle pricks; behcet syndrome
how do you diagnose behcet syndrome?
history and physical exam/ serology: HLA-B51
how do you treat behcet syndrome?
low dose glucocorticoids
how do you diagnose buerger disease?
angiography “corkscrew appearance”
how do you treat Buerger disease?
STOP SMOKING- glucocorticoids and anticoagulation do not work
what is polyarteritis nodosa associated with?
HBV
how does polyarteritis nodosa affect the skin?
livedo reticularis, subcutaneous nodules, ulcers, and digital gangrene
how does polyarteritis nodosa affect the peripheral nerves?
80% have vasculitis neuropathy–> mononeuritis multiplex (foot drop)
how does polyarteritis nodosa affect the lungs?
it doesn’t–> they are spared
how do you diagnose polyarteritis nodosa?
biopsy (fibrinoid necrosis and no granulomas) or angiogram–> micro-aneurysm
how do you treat polyarteritis nodosa?
glucocorticoids and treat HBV
how does kowasaki syndrome present?
less than 5 years old M>F; Japan; fever, lymphadenopathy, rash strawberry tongue
if kowasaki syndrome goes untreated, how do you die?
from coronary involvement –> aneurysm or MI
how do you treat kowasaki syndrome?
IVIG within 10 days of symptoms and high does of ASA
who is at most risk of takayasu arteritis?
less than 40 year old females, most common in asians
how can takayasu arteritis be described?
pulseless disease- obliterate UE peripheral pulses
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
how do you diagnose takayasu arteritis?
MRI or CT angiography showing long smooth tapered stenosis; biopsy shows granuloma with some giant cells
how do you treat takayasu arteritis?
glucocorticoids
giant cell arteritis frequently coexists with what?
polymyalgia rheumatica (PMR)
who is more at risk for getting GCA and PMR?
females, 40-50, whites>blacks
what might the clinical history/symptoms be in a person with GCA?
headache, jaw claudication, visual abnormalities
how do you diagnose GCA?
increased ESR, serology: HLA-DR4, temporal artery biopsy is a gold standard
what does a temporal artery biopsy show in a patient with GCA?
segmental granulomatous vasculitis with multinucleated giant cells
how do you treat GCA?
start glucocorticoids (before biopsy)
what happens if GCA is not treated?
blindeness
how does polymyalgia rheumatica (PMR) present?
stiffness, soreness, and muscle pain; feelings of weakness as a result of pain but not the objective weakness
how do you diagnose PMR?
everything is normal except ESR and CRP
how do you treat PMR?
glucocorticoids
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
what type of hypersensitivity is SLE?
type III
what are the hematologic effects of SLE?
anemia, thrombocytopenia, venous or arterial thrombosis
what are the cardiopulmonary effects of SLE?
pericarditis, pleuritis, myocarditis, libman-sacks endocarditis, pts also at risk of MI due to accelerated atherosclerosis
what are the neurological effects of SLE?
retinopathy (cotton wool spots)
what serology testing is used for diagnosing SLE?
dsDNA, smith, and anti-Ro and LA
which serology test correlates with disease activity of SLE?
dsDNA
when might complement levels suggest SLE?
if C3 and C4 are decreased
how do you treat SLE?
NSAIDs, avoid sun exposure, glucocorticoids, hydroxychloroquine
what should be considered in the management of SLE?
you should minimize other conventional risk factors for atherosclerosis–> avoid or quit smoking
in patients with long term corticosteroid use, what should you monitor for?
avascular necrosis of bone and osteoporosis (DEXA)
what are the complications associated with SLE early on?
infections and kidney/CNS disease
what are the complications associated with SLE later on?
accelerated atherosclerosis and thromboembolic events
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)
what is the treatment for anti-phospholipid antibody syndrome?
indefinite systemic anticoagulation
what effect does DIL have on the body?
it promotes demethylation of DNA
how do you diagnose DIL?
(+) ANA and (+) anti-histone antibodies
who is affected by neonatal lupus?
it affects children born of mothers with anti-Ro (SSA) antibodies
how does neonatal lupus present?
rashes, thrombocytopenia, hemolytic anemia, arthritis; congenital heart block
how do you diagnose neonatal lupus?
anti-Ro in mother
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
how does discoid lupus present?
well-defined inflammatory plaques that evolve into atrophic disfiguring scars- most commonly occurs on the head
how do you diagnose discoid lupus?
clinical exam and biopsy
what does discoid lupus resemble?
tinea infection, psoriasis, and morphea
what is the hallmark of scleroderma?
thickening and hardening of the skin; almost all patients have secondary raynaud phenomenon
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
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)
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
how do you diagnose localized scleroderma?
it is histologically indistinguishable from other forms of scleroderma
how do you treat localized scleroderma?
quite resistant to therapy; can try phototherapy UVA or methotrexate + steroids
how would you define limited (cutaneous) scleroderma?
inflammatory condition that causes hard and thickened tissues in the body (some internal organs)
how does limited scleroderma present?
indolent course, raynaud’s can be one of the first symptoms; CREST syndrome; pulmonary involvement
what does CREST stand for?
calcinosis cutis, raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia
what is the pulmonary involvement seen in limited scleroderma?
progressive pulmonary artery hypertension (PAH)–> presents as SOB
how do you diagnose limited scleroderma?
typically made in advanced disease; serology: (+) ANA and (+) anti-centromere
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
what can be used in a patient with limited scleroderma and pulmonary hypertension?
phosphodiesterase type 5 inhibitor–> may improve cardiopulmonary hemodynamics
how would you define diffuse scleroderma?
inflammatory condition that causes hard and thickened tissues in the body, INCLUDING internal organs
how does diffuse scleroderma present?
systemic, interstitial lung disease, renal crisis
how does interstitial lung disease present?
SOB, dry, course (velcro-like) crackles on auscultation
what is a heralding feature of renal disease?
blood pressure elevation
how do you diagnose diffuse scleroderma?
(+) anti-scl 70 aka anti (DNA) topoisomerase I; (+) anti-RNA polymerase III; (+) ANA
how do you diagnose ILD?
chest x-ray or CT scan of chest: shows ground glass appearance; pulmonary function tests
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
how do you diagnose pulmonary artery hypertension?
2D echocardiogram or right heart catheterization, which will shoe elevated pulmonary artery pressure
those with scleroderma are at an increased risk of developing what?
bronchoaveolar carcinoma
what are 3 signs of CKD?
proteinuria, elevation of creatinine, or HTN
what defines renal crisis?
malignant hypertension, hemolytic anemia, and progressive renal insufficiency
what endocrine disorder is common in patients with scleroderma?
hypothyroidism
what MSK disease is common in patients with scleroderma?
carpal tunnel syndrome
how would you define sjogren syndrome?
autoimmune, inflammatory destruction of exocrine glands
how does sjogren syndrome present?
sicca symptoms- dryness and parotid/salivary gland enlargement
sjogren syndrome has a strong association with what?
B cell non-Hodgkin lymphoma (MALT lymphoma)
how do you diagnose sjogren syndrome?
serology: (+) ANA, polyclonal hypergammaglobulinemia, (+) Anti SSA/Ro, (+) Anti SSB/LA (never present without Ro); labial salivary gland biopsy
what are the three inflammatory myopathies?
dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM)
how does dermatomyositis present?
ages 7-15 and 30-60; weakness without sensory symptoms with characteristic skin lesions
what are the characteristics skin lesions associated with dermatomyositis?
grotton’s patches/papules, heliotrope rash, periungual erythema, v-neck erythema
patients with dermatomyositis are at increased risk of what?
occult malignancy
what should you do when you make the diagnosis of dermatomyositis?
start looking for occult malignancy
how doe labs appear in patients with dermatomyositis?
elevated CK and aldolase
what serology is used for the diagnosis of dermatomyositis?
anti Jo-1
biopsy in a patient with dermatomyositis shows what?
perimysial and perivascular inflammation, perifascicular atrophy
how do you treat dermatomyositis?
glucocorticoids
what is the medical term for shawl sign?
poikiloderma
how does polymyositis present?
30-50+ years, subacute, proximal muscle weakness, no skin changes
what do the labs look like in a patient with polymyositis?
elevated CK and aldolase
what serology is used to diagnose polymyositis?
anti-jo1
what does biopsy show in a patient with polymyositis?
endomysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another diagnosis
how do you treat polymyositis?
glucocorticoids
how does inclusion body myositis present?
> 40-50, M>F, white> black; weakness: finger flexion or quadriceps weakness
what do the labs look like in a patient with inclusion body myositis?
mild elevation or normal CK
what is the serology used for inclusion body myositis?
anti-cN1A autoantibodies
what does biopsy show in a patient with inclusion body myositis (IBM)?
endomysial inflammation, rimmed vacuoles, invasion of non-necrotic muscle fibers
how do you treat inclusion body myositis?
treatment is supportive
