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27 Cards in this Set

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116. Tight skin + heartburn + Raynaud's=?

Scleroderma.

117. Presentation of scleroderma (systemic sclerosis) [presents with three main symptoms]?

1. Skin (100%): look for a woman with tight, fibrous thickening of the skin that gives a tight face and tight, immobile fingers.
2. Raynaud's phenomenon (100%)
3. Joint Pain.

118. What are tight, immobile fingers known as?

Sclerodactyly

119. Raynaud's phenomenon?

a. This is a three–phase vascular hyperreactivity, with the skin of the fingers becoming white, then blue, then red.
b. It can be quite painful
c. Digital Ulceration may occur from infarction of the skin!
d. There might also be abnormal giant capillaries in the nail folds!



After rewarming the hands, the blood flow will rebound (hyperemia) and the skin will appear reddened or blushed.

How to differentiat between primary Raynaud phenomenon (Raynaud disease) and secondary Raynaud phenomenon (e.g., scleroderma)?

Nailfold capillaroscopy test (placing a drop of oil on the patient’s nailfold at the base of the fingernail).


Examination of this area under a microscope is then conducted to look for any capillary changes.


Enlarged, dilated, or absent nailfold capillaries are noted among patients with scleroderma and other autoimmune diseases.

120. Characteristics of joint pain with scleroderma (2)?

Mild and symmetrical.

121. 4 other organs affected by diffuse scleroderma?

1. Lungs
2. G.I.
3. Heart
4. Renal

122. lung complications Of Diffuse Scleroderma?

Fibrosis and pulmonary hypertension (These are the leading cause of death)
123. G.I. Complications of diffuse scleroderma?
1. Wide–mouthed clonic diverticula
2. Esophageal Dysmotility, this leads to reflux and Barrett's esophagus
3. there is primary biliary or cirrhosis in 15% of patients!
124. Heart complications of scleroderma?
Restrictive cardiomyopathy

125. renal complications of scleroderma?

May lead to malignant hypertension “scleroderma renal crisis”

How to prevent “scleroderma renal crisis” in pt. with scleroderma?

The ACE inhibitors.


The treatment has to be initiated before the onset of renal failure.

126. Diagnostic testing for scleroderma?

a. There is no single diagnostic test.
b. ANA is present in 95% of cases, but is nonspecific.
c. Anti–topoisomerase (anti–Scl 70) is only present in 30% of patients.

127. Treatment of systemic scleroderma?

a. No treatment has been proven effective in stopping scleroderma.
b. Penicillamine is not effective in delaying progression of this disease.
c. One can use the following therapies for treatment of each individual organ system

Treatment for the skin manifestations in pt. with scleroderma?

d-penicillamine

128. important: how is interstitial lung disease in scleroderma treated?

Cyclophosphamide.

129. treatment of renal involvement and hypertension in scleroderma?

Use ace inhibitors

130. Treatment of Pulmonary Hypertension in scleroderma (3 classes of agents)?

1. Bosentan (endothelin antagonist)
2. Prostacyclin Analogs (epoprostenol, treprostinil, iloprost)
3. Sildenafil

131. Treatment of Raynaud's , with scleroderma?

Use calcium channel blockers.


(especially nifedipine)

132. Treatment of GERD in scleroderma?

Regular use of PPIs.
133. Treatment of lung fibrosis in scleroderma?
Cyclophosphamide.

134. What is CREST syndrome composed of (limited scleroderma)?

1. Calcinosis of the fingers
2. Raynaud's
3. esophageal dysmotility
4. Sclerodactyly
5. Telangiectasia

135. What does CREST Syndrome NOT present with?

1. Joint pain
2. heart involvement
3. lung involvement (except for pulmonary hypertension)
4. kidney involvement

136. Note: CREST DOES present with primary hypertension.

Note: CREST DOES present with primary hypertension.

137. Diagnostic testing for CREST Syndrome?
a. Anti–centromere antibodies!!! CREST is characterized by anticentromere antibodies.
b. Crest syndrome does not involve anti–SCl70

138. Presentation of eosinophilic fasciitis?

a. Thickened skin that looks like scleroderma.
b. However, the following are not present:
1. hand involvement
2. Raynaud's
3. heart, lung or kidney involvement
c. there is marked eosinophilia, and the appearance of an “orange peel” (peau d'orange)

139. treatment of use and affiliate fasciitis?

Corticosteroids