Sjogren's Syndrome Flashcards
Acquired chronic connective tissue disease; more common in WOMEN; characterized by Autoimmunity, Vascular damage and Tissue Fibrosis
Systemic Sclerosis
(Limited/Diffuse) Scleroderma was previously known as CREST syndrome; involvement in hands, arms and face but not internal organs
Limited
(Limited/Diffuse) Scleroderma is rapidly progressive and involves the torso, arms and internal organs
Diffuse
3 Subtypes of Systemic Sclerosis
Limited Cutaneous
Diffuse Cutaneous
Sine Scleroderma
Features of LIMITED Cutaneous Sclerosis
Calcinosis Raynaud's Esophageal Dymotility Sclerodactyly Telangiectasis
(formerly known as “CREST” syndrome)
Features of DIFFUSE Cutaneous Sclerosis
RAPID involvement Tendon Friction Rubs Interstitial Lung Disease Renal Crisis Anti-Scl-70 (Topoisomerase) antibody Anti-RNA polymerase
What differentiates Limited/Diffuse Cutaneous Sclerosis from Sine Scleroderma
Sine Scleroderma means “no skin thickening”
many other features are present though
Most common organ systems involved with Systemic Sclerosis
Skin
Gastrointestinal
Pulmonary
Vasospastic reaction to cold temperatures or emotional stress; SHARPLY demarcated color changes of skin; can be associated with autoimmune conditions, especially Sclerosis
Raynaud’s Phenomenon
Pattern of Color changes seen in Raynaud’s Phenomenon
White (ischemia)–> Blue (Cyanosis)–> Red (Re-perfusion)
Conditions that can cause Secondary Raynaud’s Phenomenon
Systemic Sclerosis*
SLE
Sjogren Syndrome
Dermatomyositis
Treatment for Raynaud’s Phenomenon
Keep hands warm Ca+ channel blockers Nitroglycerin (topical) PDE inhibitors Prostacyclines
Leading mortality for Systemic Sclerosis patients
Lung Disease (Interstitial Lung Disease and/or PAH)
renal is second most
Interstitial Lung Disease due to Systemic Sclerosis is associated with which antibody?
Scl-70 (topoisomerase 1)
Anti-Scl 70 (Anti-Topoisomerase 1) is associated with ____________ Cutaneous Systemic Sclerosis
Diffuse (seen with ILD)
Pulmonary Artery Hypertension due to Systemic Sclerosis is associated with which antibody?
Anti-Centromere antibody
Anti-Centromere Antibody is associated with _______________ Cutaneous Systemic Sclerosis
Limited (seen with PAH)
How to tell the difference between IF staining for Anti-Centromere vs. Anti-Scl 70 (Topoisomerase) Antibody
Anti-Centromere: dots in nuclei (left)
Anti-Scl 70: all of cytoplasm (right)
Renal Crisis due to Systemic Sclerosis is associated with which antibody?
RNA-Polymerase I and III
Signs of Scleroderma Renal Crisis
HTN (high renin) MAHA Thrombocytopenia Proteinuria Renal insufficiency/failure
Treatment for Scleroderma Renal Crisis
ACEi (inhibits the high renin state)
When should steroids be used for Systemic Sclerosis
Active ILD (Mycophenolate or Cyclophosphamide)
GI effects of Scleroderma
Persistent GERD Dysphagia (dec. peristalsis) Strictures Gastric Antral Vascular Ectasia (melena or iron def. anemia) Gastroparesis Bloating/Nausea Constipation
Lack of gastric motility or emptying of the the stomach/intestines; can be caused by Scleroderma; associated with a Succussion Splash (fluid sloshing around due to dec. emptying)
Gastroparesis
Why do we use Carbon Monoxide to assess for Diffusion Capacity (DLCO)
Has a higher affinity for hemoglobin, so any difficulty in diffusion (dec surface area or inc. thickness) will be apparent
Treatment for Sclerotic ILD
Cyclophosphamide
Mycophenolate
Rituximab
Stem-cell transplant
MOA for Cyclophosphamide
Alkylating agent that interferes DNA replication—> dec. B and T cells
MOA for Mycophenolate
Inhibition of de-novo purine synthesis–> dec. lymphocyte proliferation
MOA for Rituximab
Anti-CD20 monoclonal antibody against B-cells
Numerical definition for Pulmonary Hypertension
> 25 mmHg at rest
Treatments for Pulmonary HTN
Endothelin receptor antagonists
PDE5 inhibitors
Prostacyclins
