Connective Tissue Diseases Flashcards
What diagnostic criteria is used to diagnose SLE?
SLICC Classificaiton Criteria for SLE, requiring at least 4 of the following (at least 1 from each)
Clinical
- Acute cutaneous lupus
- Chronic cutaneous lupus
- Oral/nasal ulcers
- Non-scarring alopecia
- Arthritis
- Serositis
- Renal
- Neurologic
- Haemolytic anaemia
- Leukopenia
- Thrombocytopenia
Immunological
- ANA
- Anti-DNA
- Anti-Sm
- Anti-pospholipid
- Low complement
- Direct Coombs’ test
SLE presentation is extremely variable and affects many different systems. List some of the more classical presentations
Constitutional
- fever
- fatigue
- weight loss
MSK
- arthralgia
- myalgia
- inflammatory arthritis
- increased risk of AVN
Mucocutaneous
- Butterfly rash
- Discoid lupus/subacute cutaneous lupus
- Photosensitivity
- Reynaud’s
- Alopecia
Renal
- nephritis
Resp
- PE
- Pleural effusions
- ILD
Etc. etc. etc.
What type of hypersensitivity reaction is SLE believed to be?
Type III (with potential type II involvement)
Inability to clear immune complexes (defect in apoptosis), which are then deposited in the basement membranes of the skin and kidneys
SLE - immunology investigations
Anti-dsDNA - specific to SLE and varies with severity of disease
ANA - positive in 95% of SLE, but not specific
Anti-Sm - specific but low sensitivity
C3 and C4 are low when the disease is active
How is SLE treated?
Manage symptoms
Skin disease/arthralgia - hydroxychloroquine, topical steroids and NSAIDs
Inflammatory arthritis/organ involvement - immunosuppression with azathioprine or mycophenolate mofetil
Steroids can be used if needed, but only short term
What symptoms does Sjogren’s classically cause?
Sicca symptoms - dry mouth, dry eyes, dry mucus membranes, vaginal dryness
Also arthralgia and fatigue
How is Sjogren’s tested for? (2)
Schirmer’s Test for ocular dryness
Presence of Anti-Ro and Anti-La antibodies
What are the two classifications of Systemic Sclerosis, and which antibody is associated with each?
Limited - anti-centromere antibody
Diffuse - anti-Scl-70 antibody
What are the key differences between Diffuse and Limited Systemic Sclerosis, with regards to presentation and organ involvement?
Limited - skin involvement is confined to face, hands, forearms and feet. Organ involvement tends to occur later, if at all
Diffuse - skin changes develop more rapidly and can involve the trunk. Early organ involvement is seen
What did Systemic Sclerosis used to be known as?
What symptoms does it classically present with?
Used to be called CREST
C - calcinosis
R - Raynaud’s
E - oesophageal dysmotility
S - scleryldactyly
T - telangiectasia
What are the symptoms of Mixed Connective Tissue Disease?
Raynauds
Arthalgia/Arthritis
Myositis
Sclerodactyly
Pulmonary hypertension
ILD
Which antibody is associated with Mixed Connective Tissue Disease?
Anti-RNP
Anti-Ro and Anti-La
Sjogren’s
Anti-RNP
Mixed CTD
Anti-centromere
Limited Systemic Sclerosis
