sampling of ctd 2011

8/3/2019 Sampling of CTD 2011

http://slidepdf.com/reader/full/sampling-of-ctd-2011 1/28

Sampling of Connective Tissue Diseases

Anne Minenko, MD

Assistant Professor of Medicine Division of

Rheumatic and Autoimmune Diseases



Scleroderma

Chronic, multisystem, autoimmune disease

4-5 x more common in women than in men

ANA +ve in > 95%

Sounds like lupus so far?

So how is scleroderma different from lupus?



Scleroderma

Common presenting symptoms/

features of scleroderma include:

Raynaud¶s

Gastroesophageal reflux/ dysmotility

Sounds like garden variety cold hands,

heartburn.

So how is scleroderma different from what

many of us complain of?



Raynaud¶s: exaggerated vasoconstriction

upon exposure to stress or to cold

Cold: pallor/ cyanosis

Rewarming: reactive hyperemia



Normal nailbeds: tidy, ³hairpin blood

vessels´

Abnormal nailbeds: swollen blood

vessels mixed with dropped out

blood vessels

Using ophthalmoscope set atgreen/black 40 and KY jelly on the

nailbed can see this:1



https://reader009.{domain}/reader009/html5/0506/5aee50ea05231/5aee50e

http://jama.ama-assn.org/cgi/reprint/301/6/686.pdf

Raynaud¶s in CTD: risk for infarcts

Raynaud¶s in CTD: risk for pulmonary

hypertension, right heart failure

-Late complication-About 25% of patients

-Shortness of breath

-Swollen legs, loud P2, hepatomegaly

-Frequent PFTests, EKG, ECHO

Raynaud¶s in CTD: risk for renal ³crisis´-early complication

-about 10%

-sudden onset severe hypertension

-new headache ± seizures

-frequent BP checks, creatinine

measures



Scleroderma

Common presenting symptoms/ features of

scleroderma include:

Raynaud¶sGastroesophageal

reflux

Scleroderma

Telangiectasias)

2



Scleroderma


4-5 x more common in women than in men

± Onset around age 50

ANA +ve in > 95%

http://www.accessmedicine.com/popup.aspx?aID=2725968

sensitive insensitive, but specific

3



Scleroderma

CREST (90%+ 5 year survival) Systemic Sclerosis(70% 5 year survival)

Calcinosis

Raynauds Raynauds

Esophageal dysmotility Esophageal dysmotility

Sclerodactyly Diffuse Scleroderma

Telangiectasias

Renal Crisis

Pulmonary hypertension Interstitial lung disease

(+/- pulmonary hypertension)

+ve ANA +ve ANA

Centromere Ab +ve Scl-70 Ab +ve



Scleroderma Lupus

ANA +ve

-Anticentromere Ab-SCL ± 70

-high creatinine but bland urine

ANA +ve

-ds DNA-complement consuming

-high creatinine but urine ³active´

with proteinuria



Polymyositis/ dermatomyositis


2x more common in women than in men

ANA +ve in > 50%


So how is PM/DM different from lupus?





features of PM/DM include:

Raynaud¶s

Swallowing problems

Shortness of breath

Skin changes

In some ways sounds like scleroderma?

So how is PM/DM different?




vessels´



blood vessels


nailbed can see this:




Common presenting symptoms/ features of PM/DM include:

Raynaud¶s

Swallowing problemsShortness of breath

Skin changes

- Gottron¶s

- Heliotrope- Shawl sign

1

https://reader009.{domain}/reader009/html5/0506/5aee50ea05231/5aee50f3bf536.jpg





features of PM/DM include:

Raynaud¶s

Swallowing problems

Shortness of breath

My ositis

- Proximal muscle weakness- High CK

- Abnormal E M G

- Uniquel y abnormal muscle

biopsies

2

http://www.neuropathologyweb.org/chapter13/images13/13-16DMl.jpg

http://www.s-ibm.org/images/dermatomyositis_01.jpg

CD8 Tcell infiltration

between muscle fascicles

B cell infiltration

³vasocentric´





2x more common in women than in men

± 2 peaks a) age 10 ± 15, b) 45 ± 60 years of age

ANA +ve in > 50%sensitive

insensitive, but specific

AutoAntibody Prevalence Associated features

Antinuclear Antibody 50% - Antisynthetase

antibody20% Raynaud¶s, interstitial lung

disease

Mi-2 antibody < 20% dermatomyositis

3



Inflammatory myopathy

Polymyositis Dermatomyositis (associatedwith malignancy?)

Raynauds Raynauds

Esophageal dysmotility Esophageal dysmotility

Myositis: high CK, proximalmuscle weakness,

CD8 Tcell infiltration

between fascicles

Myositis: high CK, proximalmuscle weakness

Vasocentric B

cell infiltration

+ve ANA +ve ANA

Antisynthetase antibody

- Interstitial lung disease

Mi-2 antibody

- Typical rashes



Inflammatory

myopathies

Lupus

ANA +ve-Antisynthetase antibody (PM)

Interstitial lung disease

Raynaud¶s

-Mi-2 antibody (DM)

Unique rashes

-high CK and myositis andproximal muscle weakness are

primary feature in PM/DM

ANA +ve-ds DNA

-high CK

-Interstitial lung disease

-Raynaud¶s

- Complement consuming



Inflammatory

myopathies

High CK

ANA +ve-Antisynthetase antibody (PM)

Interstitial lung disease

Raynaud¶s

-Mi-2 antibody (DM)

Unique rashes

-high CK and myositis andproximal muscle weakness are

primary feature in PM/DM

-- Drugs eg. Statins, colchicine«

-Infection eg. HIV, pyomyositis«.

-Toxins eg. Alcohol

-Trauma eg. Crush injury,

electrocution«.-Etc«

«and these (more common

causes of high CK) can be

distinguished from PM/ DM byhistory, EMG, biopsy



Sjogren¶s syndrome


14x + more common in women than in men

ANA +ve in > 80%


So how is Sjogren¶s different from lupus?



Sjogren¶s syndrome

Common presenting

symptoms/ features of

Sjogren¶s include:

Raynaud¶s

Swallowing problems

In some ways sounds like

scleroderma?

So how is sjogren¶s different?




vessels´



blood vessels


nailbed can see this:



Sjogren¶s syndrome

Common presenting

symptoms/ features of

Sjogren¶s include:

Raynaud¶s

Swallowing problems

Skin changes

- Palpable purpura

1



Sjogren¶s syndrome

Common presentingsymptoms/ features of Sjogren¶s include:

Raynaud¶sSwallowing problems due to«.

Dr y ness

- Xerostomia

- xerophthalmia

2



Sjogren¶s syndrome

Common presentingsymptoms/ features of Sjogren¶s include:

Raynaud¶sSwallowing problems

Dr y ness

- Xerostomia

- xerophthalmia

2



Sjogren¶s syndrome


14x + more common in women than in men

± Onset around 4th ± 6th decade of life

ANA +ve in > 80%

AutoAntibody Prevalence Associated features

Antinuclear Antibody 80%Ro (SSA)

30 -60%

Extraglandular features like

cytopenias, neuropathy, purpuraLa (SSB)

RF 50%

3

sensitiveinsensitive, but specific



RA Sjogren¶s Lupus

RF+ve

xerostomia

xerophthalmia

symmetricalsmall joint

polyarthritis

primary feature

ANA +ve

Ro and La

cytopenias

neuropathy

RF+velow c3c4

xerostomia

xerophthalmia

primary feature

RTA

ANA +ve

ds-DNA

low c3c4xerostomia

xerophthalmia

IC renal disease

cytopenias

myositis

sampling of ctd 2011

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