connective tissue

Question 1

what are the 5 cardinal signs of systemic sclerosis?

Answer 1

calcinosis
raynauds
esophageal dismotolity
sclerodactyly
telangiestasia

Question 2

What diagnostic test indicate systemic sclerosis?

Answer 2

-anti-scl 70 positive in 70% of people with diffuse
(same as anti DNA topoisomerase)
-anticentromere antibody associated with 30% of limited form CREST syndrome
-ANA 95%
-RF in 25%
-elevated ESR

Question 3

How does systemic sclerosis present?

Answer 3

puffy taut thick skin without wrinkles
thicken tendon sheath-loss ROM, crepitus
esophageal dysmotility
calcinosis
initially present as Raynauds
sclerodactyly
telangiestasia
malignant HTN-renal fail
pulmonary fibrosis

Question 4

How do patients with systemic sclerosis die?

Answer 4

pulmonary fibrosis or renal fail

Question 5

Who is most likely to get scleroderma or systemic sclerosis?

Answer 5

75% are women

50-60 yo

Question 6

Where does the limited form of scleroderma present?

Answer 6

forearm, face, fingers, skin

Question 7

what is the course of diffuse scleroderma?

Answer 7

rapid
widespread to skin
early involve of visceral organs(renal, GI, lungs, heart)

Question 8

What does an x-ray of systemic sclerosis diffuse form show?

Answer 8

destructive changes in the joint
soft tisuse calcifications
periarticular resorption of bone

Question 9

who is fibromyalgia most common in?

Answer 9

women 20-50years

Question 10

what are the diagnostic requirements of Fibromyalgia?

Answer 10

muscle pain of at least 3 months duration
significant pain or tenderness in 11 of 19 tender points
normal neuro exam

Question 11

What are some symptoms patients complain of in fibromyalgia?

Answer 11

1. general muscle pain
2. pain on exertion
3. fatigue
4. aching or burn or stiff (paresthesia)
5. anxiety and depression
6. stress aggrevates
7. poor sleep

Question 12

what is the treatment for fibromyalgia?

Answer 12

treat depression (SSRI, TCA)
cyclobenzaprine
NSAID
maintain good circulation
improve sleep quality
continue exercise

Question 13

what type of treatment should be avoid in treating fibromyalgia?

Answer 13

glucocorticoids

opiates

Question 14

Who does polymyalgia rheumitca present in?

Answer 14

most commonly women over 50

Question 15

What is the clinical presentation of Polymyalgia rheumatic?

Answer 15

pain and stiffness in shoulders and hips and neck
weight loss
fever
fatigue
DOESNT cause muscle weakness
later stages can present with muscle atrophy

Question 16

what condition is associated with PMR?

Answer 16

giant cell arteritis (temporal)

Question 17

What are some diagnostic tests for PMR?

Answer 17

RF (-)
muscle biopsy (-)
normal CK
elevated ESR
can have normochromic monocytic anemia

Question 18

Who does sarcoidosis most commonly present in?

Answer 18

blacks 20-40

women more than men

Question 19

what is the etiology of sarcoidosis?

Answer 19

immune dysregulation

genetic background or possible environmental trigger

Question 20

What does the histology of sarcoidosis show?

Answer 20

noncaseating granulima of epitheliod macrophages
(process begins with acculumation of T cells and macros)
noncaseating granulomas contain Schaumann and asteriod bodies

Question 21

Where do noncaseating granulomas appear in sarcoidosis?

Answer 21

lung is most common

then skin and eye

Question 22

What is the treatment for PMR?

Answer 22

corticosteriods low dose

Question 23

What is the treatment for sarcoidosis?

Answer 23

steriods

70% recovery with minimal residual

Question 24

What is the clinical presenation of sarcoidosis?

Answer 24

1. asymptomatic except for bilateral hilar adenopathy or reticular opacities
2. hemoptysis
3. cough, chest pain
4. shortness of breath

Question 25

What is the onset for sarcoidosis?

Answer 25

insidious 40-70% | can be subacute a few weeks duration

Question 26

What are exam findings for sarcoidosis?

Answer 26

bilateral hilar adenopathy splenomegaly hepatomegaly cutaneous lesions in eye (retinal opacities)

Question 27

what are some diagnostic tests for sarcoidosis?

Answer 27

CXR bilateral hilar adenopathy elevated serum calcium (1 alpha hydroxylase mediated vitamin D activation by epitheliod macros) elevated serum ACE levels biopsy adenopathy

Question 28

What is the course of sarcoidosis?

Answer 28

progressive unrelenting or fluctuating

Question 29

What are some complications of sarcoidosis?

Answer 29

1. bell's palsy 2. intersitital fibrosis(restrictive lung disease) 3. erythema nodosum 4. uvetitus

Question 30

what is systemic primary amyloidosis associated with?

Answer 30

multiple myeloma (anaplastic small cell cancer of bone marrow)

Question 31

What is systemic secondary amyloidosis?

Answer 31

AA- chronic disease of reactive nature

Question 32

Where are some places for local deposits of amyloid?

Answer 32

``` endocrine atrial amyloid angiopathy cerebral -alziehmers prion disease ```

Question 33

how does amyloidosis present?

Answer 33

1. general ROS symptoms - weak - weight loss - dizzy 2. later organ symptoms

Question 34

What is the presentation of SLE?

Answer 34

``` malar butterfly rash photsensitivity fever pleuritic pain joint pain ```

Question 35

who most commonly presents with SLE

Answer 35

black females under 20

Question 36

what is the presentation of SLE?

Answer 36

1. acute, chronic or insidious onset 2. joint pain 3. fever 4. buttery fly rash and photosensitivity, oral ulcers 5. renal, cardiac and neuro symptoms 6. can remit and relapse 7. weight loss

Question 37

What is the etiology of SLE?

Answer 37

1. fail self tolerance HLADR/HLADQ 2. drug induced: d-peni, isonazid, hydrazine, procaninamide 3. infectious agent (epstein barr titer positive) trigger hypersensitivity

Question 38

What organ systems are affected in SLE?

Answer 38

mucosal membranes (skin, lung, renal, cardiac, joint)

Question 39

what are some diagnostic tests for SLE?

Answer 39

anti-ds Anti-smith ANA anti-phospholid (false negative with syphililis)

Question 40

How is SLE diagnosed?

Answer 40

1. ANA, ds-DNA, Anti-sm, SSA and SSB antibodies 2. 4 or more -malar rash -photosensivity -discoid rash -oral ulcers -serositis(pleuritis)(pericarditis) -renal (nephritis common cause of death) proteinuria -neuro (seizure or psychosis) -hemolytic anemia leukopenia

Question 41

how does erythema nodosum present?

Answer 41

can be with sarcoidosis or IBD 1. tender poorly defined nodules 2. nodules on extensor surface of lower extremity 3. erythematous nodules 4. edematous

Question 42

What is erythema nodosum associated with?

Answer 42

beta hemolytic strep infections TB drugs-sulfonamides, oral contraception comorbid: sarcoidosis and IBD

Question 43

What is the most common cause of death in SLE?

Answer 43

renal fail-nephritis

Question 44

How is erythema nodosum diagnosed

Answer 44

biopsy the nodules | inflammed neutrophil infiltrate of subcutaneous fat and fascia

Question 45

What is the etiology of scleroderma?

Answer 45

``` systemic automimmune B cell Th1 and T cell mediated -T cell fibrogenesis of small vessels -endothelial cell damage (renal, pulmonary, GI, skin, cardiac) -vasocontrict and ischemia ```