Connective Tissue Disorders Flashcards
What does SLE stand for?
Systemic Lupus Erythematosus
What is SLE?
Auto-Antibodies vs components of Cell Nucleus
What is pathophysiology of SLE?
Incomplete understanding Cell apoptosis ⇒ Nuclear Ag’s released Nuclear Self-Antigen’s presented to Immune cells B cell mediated Autoimmunity Healthy tissue damage by Complement activation Fc receptor engagement
What is the demographic of SLE?
- Px : 1 – 40 yo
- F:M = 9:1
- ↑ prevalence in African & Asian ancestry population
What is the management goal of SLE?
Remission or ↓ Disease activity & Prevention of flares
What is the treatment for SLE?
- Hydroxychloroquine
- Symptomatic Relief
- Cyclophosphamide & B cell MAb’s
What is the Risk Factor Modification in SLE?
Antiphospholipid Syndrome – Anticoagulants
Assess Infection + Cardiovascular risk
Planned pregnancy
What are the symptomatic relief treatments for SLE?
- Glucocorticoids – Symptomatic Relief (Caution LT use)
2. Immunomodulatory agents (e.g. methotrexate, azathioprine, mycophenolate) to help ↓ GC use
What are the two types of scleroderma?
- Diffuse Scleroderma
2. Limited Scleroderma
What is the aetiology of Sjogen’s syndrome?
Can be 1° or 2° to another CT disorder
What is the investigation for Sjogen’s syndrome?
Schirmer’s Test
What is Schirmer’s Test?
Add filter paper under the eye, measure wetness for 5 min
What is the pathophysiology of Sjogen’s syndrome?
Autoimmune Exocrinopathy:
-Lymphocytic infiltration of exocrine glands + sometimes extra glandular involvement
What are the symptoms for Sjogen’s syndrome?
- Glands:
- Dry eyes (xeropthalmia)
- Dry mouth (xerostoma)
- Parotid enlargement - Extra glandular involvement:
- Non erosive arthritis
- Raynaud’s phenomenon
What is medical term for dry eyes?
Xeropthalmia
What is the medical term for dry mouth?
Xerostoma
What is xeropthalmia?
Dry eyes
What is xerostoma?
Dry mouth
What is the aetiology for Autoimmune Muscle Disorders?
2 main types – Polymyositis, Dermatomyositis
= Proximal Muscle weakness ± Rash
What are the symptoms for Polymyositis?
- Proximal Muscle weakness
What are the symptoms for Dermatomyositis?
- Proximal Muscle Weakness
- Heliotrope Rash
- Gottron’s papules
- Subcutaneous calcinosis
- Mechanic Hands
What investigations are done for Autoimmune Muscle Disorders?
- ↑ Serum Creatinine Kinase
- Abnormal EMG
- Abnormal Muscle Biopsy
- Polymyositis = CD8
- Dermatomyositis = CD4
What are the associations with Autoimmune Muscle Disorders?
Malignancy (10-15%)
Pulmonary Fibrosis
What abnormal muscle biopsy is found in Polymyositis?
CD8
What abnormal muscle biopsy is found in Dermatomyositis?
CD4
What Autoimmune Muscle Disorder is CD8 found in an abnormal muscle biopsy?
Polymyositis
What Autoimmune Muscle Disorder is CD4 found in an abnormal muscle biopsy?
Dermatomyositis
