Connective Tissues CIS (McGowan) Flashcards
1
Q
what does a + ANA test tell you?
A
- loosely associated with underlying autoimmune disease
- titer of <1:40 is normal/ negative
- titer of 1:160 or above means there is probably an underlying autoimmune disease
2
Q
what are the clinical aspects of localized scleroderma?
A
- benign skin conditions
- affect children
- discreet areas of discolored skin induration
- NO Raynauds
- NOT systemic
- Histologically indistinguishable from SSc
- Patches= Morphea
- Coalesced patches= generalized morephea
3
Q
what are the clinical aspects of Sjogren’s syndrome?
A
- F>M
- Mid 50’s/ postmenopausal
-
Sicca sx (immune mediated dysfunction of lacrimal and salivary glands):
- dry eyes, dry mouth (xerostomia), vagina dryness, tracheo-bronchial dryness
- increased incidence of oral infection (candida)
- dental caries
- parotid or other major salivary gland enlargement
4
Q
What is the term used to describe the foreign body sensation in the eye due to inadequate tear production in SJogren’s syndrome?
A
Keratoconjunctivitis sicca
5
Q
Which disease is Sjogren’s syndrome most strongly associated with?
A
B cell non-hodgkin lymphoma
6
Q
Which disease is Libman-Sacks endocarditis (a type of non-infective endocardidtis) associated with?
A
- SLE
- complication can be stroke
7
Q
which autoimmune disease increases risk for esophageal adenocarcinoma?
A
Diffuse scleroderma
Diffuse scleroderma–> GERD–> Barretts esophagus–>esophageal adenocarcinoma
treat with omeprazole
8
Q
Diffuse sceleroderma can increase risk of getting esophageal adenocarcinoma and ____
A
Gastric Antral Vascular Ectasia (GAVE)
9
Q
which autoimmune disease increases risk for pulmonary artery hypertension? is also a cause of mortality in these patients
A
limited scleroderma
10
Q
which autoimmune disease increases risk for interstitial lung disease? is a cause of mortality in thses patients
A
diffuse scleroderma
11
Q
what are the clinical sx of polymyalgia rheumatica? (PMR)
A
- W>M
- >40-50
- white>
- frequently coexists with giant cell arteritis
- constitutional sx:
- fever, malaise, weight loss
- normal WBC count
- proximal severe, symmetrical morning and daylong stiffness, soreness, and pain in shoulder, neck, and pelvic girdles
- associated with GCA
- elevated ESR
- muscle enzymes and EMG normal
- no inflammation on muscle bx
12
Q
which condition only occurs in smokers?
A
Thromboangiitis Obliterans AKA Buerger Disease
treatment= stop smoking
13
Q
What is Primary Raynauds?
A
- physiologic hyperresponse to:
- emotions
- cold temperatures
- no underlying pathology
14
Q
How is Secondary Raynauds different from Primary Raynauds?
A
- Secondary Raynauds occurs as part of an underlying condition.
- Primary Raynauds has no underlying pathology associated with it.
15
Q
what condition is associated with Hep B infection?
A
Polyarteritis Nodosa
16
Q
Name some conditions that increase your risk for DVT
A
- Factor V Leiden
- Antiphospholipid antibody syndrome
- SLE
- Becet’s
17
Q
What are the clinical sx of Becet’s disease?
A
- Oral ulcers
- genital ulcers
- uveitis
- DVT
- arterial clotting
18
Q
Which drugs are associated with drug-induced lupus?
A
- hydralazine
- isoniazid
- minocycline
- TNF inhibitors
- Quinidine
- Chlorpromazine
- Methyldopa
- Procainamide
19
Q
ds-DNA Ab is mostly seen in ___
A
SLE
20
Q
Sm Ab is mostly seen in
A
SLE
21
Q
Histone Ab are seen in
A
Drug-induced SLE
22
Q
centromere Ab are seen in ___
A
limited scleroderma (CREST)
23
Q
Scl 70 Ab (Topoisomerase I Ab) is seen in
A
diffuse scleroderma
24
Q
clinical signs of SLE
A
- multisystem
- unknown eitiology
- inflammatory autoimmune disorder
- T and B cell hyperactivity
- Autoantibodies to nuclear antigens
- Immune complexes (type III hypersensitivity)
- spontaneous remissions and relapses
- F>M
- african americans>
- those without insurance
- UV light
- EBV
- Cotton wool spots
- malar rash (butterfly rash)
25
What is a VDRL and in what autoimmune disorders is it positive in?
* VDRL is a test for syphillis
* biologic false + VDRL tests seen in **SLE** and **Antiphospholipid antibody syndrome**
26
what is the best management/treatment plan for SLE?
* hydroxychloroquine
* is one of the mainstay first treatments for SLE
* anti-coagulant to treat DVT
* start with LMW heparin/unfractionated heparin first then
* bridge to warfarin/coumadin
* starting warfarin on its own is PROthrombotic
* Can ALSO:
* avoid sun exposure
* use NSAIDS
* use corticosteroids
27
Calcium channel blockers, such as Amlodipine, are used in the treatment of \_\_\_
Raynaud's
28
IVIG and high dose ASA is used to treat \_\_\_\_\_
Kawasaki Disease
29
post prandial intermittent abdominal pain
intestinal angina (associated with SLE)
not a cause of mortality
30
seizure disorder is associated with which autoimmune disease?
SLE
31
Mortality in SLE patients is due to:
* early years
* infections
* later years
* accelerated **atherosclerosis**
* thomboembolic events
32
what are some common cardiac manifestations of SLE?
* libman sacks endocarditis
* pleuritis
* **pericarditis**
* pain worse with positional changes
* diffuse elevated ST wave changes on EKG
* myocarditis
33
Which things are positive on SLE Serology?
* **ANA**
* **non-specific** for autoimmune disease
* **anti-ds DNA**
* correlates with **disease activity**
* **Sm (Smith)**
* does **NOT** correlate with disease activity
* **decreased C3 and C4**
* ****increased comsupmtion of compliment suggests disease activity
* returns normal when in remission
34
Elevated Creatine Kinase (CK) is diagnostic in which inflammatory myopathies?
* dermatomyositis
* polymyositis
35
Deposits of ___ are diagnostic in Heoch-Schonlein Purpura
IgA
36
List preventative measures to be taken for a patient with SLE
* avoid smoking
* minimize risk factors for atherosclerosis
* **influenza vaccination**
* pneumococcal vaccination
* preventative cancer screening
* check for avascular necrosis of the bone
37
what are the clinical features of Kawasaki disease?
* medium vessel
* \<5 yo, Asian
* **Mucocutaneous lumph node syndrome**
* **Strawberry tongue**
* DDx: toxic shock syndrome and Scarlet Fever
* **death from _coronary_ involvement (_aneurysm_ or MI- can occur years later)**
* **Tx: IVIG w/in 10 days of sxs and high dose ASA**
* **yes ASA in a ped patient**
38
what are the clinical sx of dermatomyositis?
* bimodal age: 7-15 yo and 30-60 yo
* weakness _w/o sensory sx_
* proximal muscles early--\> distal muscles late in disease course
* characteristic skin lesions of DM
* **_heliotrope rash_**
* **__**periorbital edema, purplish suffusion over eyelids
* **_"Shawl sign"_**
* erythema over neck/shoulders, upper chest and back
* increased risk of occult **malignancy**** (correlates to age and risk factors for each individual patient)**
* Dx: **biopsy-perimysial and perivascular inflammation**
* **_perifascicular atrophy_**
* **elevated _creatine kinase_ and _aldolase_**
* **anti Jo-1**
* **anti Mi2**
* **anti-MDA5**
* **anti-P155/P140**
39
which connective tissue diseases include proximal muscle weakness?
* dermatomyositis
* polymyositis
* polymyalgia rheumatica
40
What does CREST stand for and what is another name for it?
* C=calcinosis cutis (white nodules on fingwers)
* R=Raynauds (secondary)
* susceptible to digital ischemia (fingers turn blue)
* usually first sx
* E=esophageal dysmotility
* S=sclerodactyly
* T=telangiectasias
* also called "limited scleroderma"
41
what type of hypersensitivity reaction is SLE?
Type III
42
Patients are at risk for MALT lymphoma with which disease?
Sjogren's syndrome
43
how do you diagnose Sjogren's syndrome?
lip biopsy
44
how do you diagnose temporal arteritis/giant cell arteritis?
temporal artery biopsy
45
which disease is associated with temporal arteritis/giant cell arteritis?
polymyalgia rheumatica
46
most likely cause of shortness of air in patients with diffuse scleroderma?
* interstitial lung disease
* restrictive PFT
* mediastinal LAD and honeycombing on CXR
* dry crackels
47
what pulmonary artery pressure is indicative of pulmonary HTN?
\>40mmHg
48
CHF can be seen in \_\_\_\_\_
Polyarteritis Nodosa
49
What are the clinical aspects of inclusion body myositis?
* \>40-50 yo
* **M\>F**
* caucasian more common
* **finger flexion pr quadriceps weakness**
* **Ck is mild elevation or _normal_**
* **muscle biopsy:**
* **endomysial inflammation**
* **_rimmed vacuoles_**
* **invasion of non-necrotic muscle fibers**
* **_anti-cN1A autoantibodies_**
50
what is the treatment for inclusion body myositis?
refractory to treatment, so treatment is supportive (physical therapy)
51
what are the clinical sx of Takyasu arteritis?
* asians (indian in CIS)
* affects large vessels (aorta and branches)
* subclavian and innominate most common
* Age \<40, F\>M
* chronic relapsing and remitting course
* **long smooth tapered stenosis**
* **"pulseless disease"**
* **obliterate UE peripheral pulses**
* **50%pulmonary involvement**
* **retinopathy, _renal artery steonsis_****, aortic dilations, aortic regurgitation, aneurysm, _aortic rupture_**
52
how do you diagnose takyasu arteritis?
* MRI or CT angiography
* copper wiring on fundoscopy
53
what histology is diagnostic of takyasu arteritis?
granuloma with some giant cells
54
what is the treatment for takayasu arteritis?
glucocorticoids
55
what are the clinical aspects of Behcet syndrome?
* **silk route (Turkey, Asia, mid East)**
* **HLA-B51**
* **_Triad:_**
* **__recurrent mouth ulcers**
* **genital ulcers**
* **eye inflammation (uveitis)**
* **large vessel anerysms**
* **venous involvement--\> DVT**
* **_pathergy=_** pustules at site of sterile needle pricks
56
What are the clinical findings in _diffuse scleroderma_?
* thickening and hardening of the skin
* fibrosis of skin and visceral organs
* systemic
* diffuse involvement
* including proximal extremities and trunk
* early and progressive internal organ involvement
* especially kidney, cardiac, and interstitial lung disease
* worst prognosis
* **serology**
* **+ Anti Sc1 70**
* **aka anti topoisomeras 1**
* **anti-RNA polymerase III**
57
positive serology for scleroderma
*
58
\_\_\_\_\_\_\_\_is an abrupt onset of malignant hypertention, hemolytic anemia, and progressive renal insufficiency and is most common in \_\_\_\_\_
renal crisis; diffuse scleroderma
M\>F
59
what is polyarteritis nodosa?
* medium vessel vasculitis
* associated with **Hepatits B**
* **M**\>F
* constitutional: fever, malaise, weight loss
* lungs are spared
*
60
how do you diagnose polyarteritis nodosa?
* biopsy-**infiltration and destruction of blood vessels by inflammatory cells--\> fibrinoid necrosis, NO granulomas**
* **angiogram-micro-aneurysm**
* **ANCA negative (check HBsAg and HBeAg)**
61
patient presents with headache that will not go away with tylenol and her temporal arteries are tender to palpation (temporal/giant cell arteritis). What is the appropriate next step?
* START CORTICOSTEROIDS BEFORE GETTING BIOPSY
* no treatment puts them at risk for blindness
62
what are the clinical manifestations of polymyositis?
* 30-50 yo+
* subacute (over weeks to months) proximal muscle weakness
* _like dermatomyositis except NO skin changes_
* **elevated serum CK**
* **anti-Jo 1**
* **muscle biopsy**
* **_endomysial inflammation_ with invasion of non-necrotic muscle fibers without features suggestive of another dx (IBM or muscular dystropy)**
63
what are the clinical features of Eosinophillic Granulomatosis with Polyangiitis (Churg Strauss Syndrome)?
* ANCA+ [\<50% of patients]
* **MPO-ANCA**
* **granulomas with eosinophilia**
* **asthma+eosinophila--\> vasculitis**
64
what organ systems are affected by polyarteritis nodosa?
* cardiac
* GI
* skin
* renal
* vasculitis neuropathy (right foot drop)
65
what is the most common cause of mortality in kawaskai disease?
coronary artery aneurysm
66
what are the clinical manifestations of Granulomatosis With Polyangiitis: aka Wegners Granulomatosis?
* **C-ANCA/PR3-ANCA +**
* **respiratory tract incolvement**
* **upper and lower**
* **saddle nose**
* **cavitary lesions on CXR**
* **Kidney involvement**
* **granulomatous inflammation**
* **necrotizing vasculitis**
* **segmental glomerulonephritis**
