Connective Tissue Disorders - Scleroderma/Sjogren Flashcards
What are the 3 types of Scleroderma?
- Localized
- Limited (CREST)
- Diffuse
What are the 3 types of Scleroderma?
- Localized
- Limited (CREST)
- Diffuse
What is the hallmark of Scleroderma?
Thickening and hardening of skin
What is the hallmark of Scleroderma?
Thickening and hardening of skin
Are there approved therapies for Scleroderma?
NO - treat symptoms
What do most patients have with Scleroderma?
Secondary Raynaud’s Phenomenon
Localized Scleroderma - skin and internal organ involvement?
Patches of thickened and hardened skin
- NO organ involvement
Discreet areas of discolored skin induration
(morphea = patches) is seen with?
Localized Scleroderma
Is Raynaud’s phenomenon present with Localized Scleroderma?
NO
Symptoms of Localized Scleroderma?
Usually Asymptomatic or itching/pain of localized areas of discolored skin induration patches (morphea)
A biopsy of the skin with Localized Scleroderma looks the same as?
Other forms of Scleroderma
Limited Scleroderma - skin and organ involvement?
Thickening and hardening of skin of fingers/toes, face/neck and distal extremities
- SOME organ involvement
Limited Scleroderma CREST symptoms?
CREST
- Calcinosis Cutis
- Raynaud’s Phenomenon
- Esophageal Dysmotility
- Sclerodactyly
- Telangiectasia
- progressive pulmonary A. HTN = dyspnea
Limited Scleroderma CREST symptoms?
CREST
- Calcinosis Cutis
- Raynaud’s Phenomenon
- Esophageal Dysmotility
- Sclerodactyly
- Telangiectasia
- Pulmonary A. HTN = Dyspnea
What pulmonary change is seen with Limited Scleroderma?
Pulmonary A. HTN = Dyspnea
Pulmonary A. HTN with Limited Scleroderma puts the patient at an increased risk for what malignancy?
Bronchoalveolar Carcinoma
How do you diagnose Pulmonary A. HTN with Limited Scleroderma?
Right Heart Catheterization
What antibody is positive with Limited Scleroderma?
(+) Anti-Centromere
(+) Anti-Centromere antibodies
Limited Scleroderma
What are some ways to treat the symptoms of Limited and Diffuse Scleroderma?
- Calcium, ACE and PDE5 (-)
- Glucocorticoids
- Anti-reflux meds
Diffuse Scleroderma - skin and organ involvement?
Thickening and hardening of skin and INCLUDES internal organs!
What antibodies are (+) with Diffuse Scleroderma?
(+) Anti-scl 70 (anti - DNA Topoisomerase 1)
(+) Anti-RNA Polermase 3
What antibodies are (+) with Diffuse Scleroderma?
(+) Anti-scl 70 (Anti-DNA Topoisomerase 1)
(+) Anti-RNA Polymerase 3
What lung manifestation will be seen with Diffuse Scleroderma?
Interstitial Lung Disease = dyspnea and velcro crackles