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
Symptoms of Interstitial Lung Disease seen with Diffuse Scleroderma?
Dyspnea and velcro crackles
Renal crisis can also occur with Diffuse Scleroderma. What are the manifestations of that?
HTN
Anemia
Proteinuria
What will the CXR look like with Interstitial Lung Disease with Diffuse Scleroderma?
Ground glass/honey comb appearance
How do you diagnose Interstitial Lung Disease with Diffuse Scleroderma?
Pulmonary Function Tests
List 3 other manifestations of Scleroderma?
GERD
Hypothyroid
Carpal Tunnel
Autoimmune destruction of exocrine (salivary) glands
Sjogren Syndrome
What are the “sicca” symptoms with Sjogren syndrome?
- Keratoconjunctivitis sicca = dry eye with foreign body sensation and decreased tear production
- Dry mouth and vagina
- Bilateral parotid gland enlargment
What are the “sicca” symptoms of Sjogren Syndrome?
- Keratoconjuncitivits Sicca = dry eye with foreign body sensation and decreased tear production
- Dry mouth and vagina
- Bilateral parotid gland enlargement
Those with Sjogren Syndrome have an increased incidence of?
Oral (candida) infections
Dental Caries
MALT Lymphoma (non B cell NHL)
Serology of Sjogren Syndrome?
Polyclonal Hypergammaglobulinemia
+) Anti-RO (SSA) and Anti-LA (SSB
What is essential for diagnosis of Sjogren Syndrome?
Labial (lip) salivary gland biopsy
Is there treatment for Sjogren Syndrome?
No - treat symptoms
Polyclonal Hypergammaglobulinemia
+) Anti-RO (SSA) and Anti-LA (SSB
Sjogren Syndrome
What can Glucocorticoids induce?
Renal crisis – bad for patients with renal issues already
