Connective Tissue Disease Flashcards
(46 cards)
1
Q
In SLE, who are more commonly affected, men or women?
A
Women (8:1)
2
Q
What is the peak age of onset in SLE?
A
Mid-thirties
3
Q
What drugs can cause SLE?
A
Hydrazine and Isoniazid
4
Q
What is the Mnemonic for remembering how SLE manifests?
A
SOAP BRAIN MD
- Arthritis
- ANA Positive
- DS DNA Positive
- Seizures
- Pleuritis Percarditis
- Malar Rash
- Discoid Rash
5
Q
If a patient is photosensitive, what should you always do?
A
Test for ANA to exclude lupus
6
Q
What are some cutaneous signs of lupus?
A
- Butterfly rash - photosensitivity over cheeks and nose.
- Urticarial lesions that are not itchy that last more than 24 hours.
- Vasculitis - if so, always screen for SLE.
- Nail fold telangectasia and splinter haemorrhages.
- Non-scarring hair loss - 50% of cases.
7
Q
What investigations should be done if SLE is suspected?
A
- Urine - test for blood and protein
- FBC - thrombocytopenia
- ESR - raised in active disease
- U&Es - renal involvement
- LFTs - hepatitis
- Lupus serology - ANA, dsDNA & ENA
- Complement - low C3 or C4 suggests active disease.
8
Q
With serological markers, what is quite indicative of Lupus?
A
Both Positive dsDNA and positive ANA.
9
Q
What is the diagnostic criteria of SLE?
A
The presence of any 4 of the following:
- Malar rash
- Discoid rash
- Abnormal photosensitivity
- Oral ulceration
- Non-erosive arthritis
- Serositis (pleurisy or pericarditis)
- Renal disease (proteinuria or renal casts)
- Neurological disease (epilepsy or psychosis)
- Haematological disorder (anaemia, leucopenia, lymphopenia or thrombocytopenia)
- Abnormal serology; ANA, dsDNA
10
Q
What is the treatment for SLE?
A
- Immunosupression is the main treatment - this depends on the overall degree of involvement.
- Oral steroids
These all usually are initiated and monitored by a specialist.
11
Q
What are the 2 main types of lupus?
A
- Subacute Cutaneous Lupus
- Discoid Lupus
12
Q
What % of patients with either form of cutaneous lupus go on to get systemic symptoms?
A
5%
13
Q
How does subacute cutaneous lupus present?
A
- Rash on sunexposed areas.
- Often annular
- Some scaling
- Lasts weeks or months.
14
Q
What does the rash of discoid lupus look like?
What is the cardinal sign?
A
it is red and scaly.
The cardinal sign is atrophy and scarring.
15
Q
Can lupus cause scarring?
A
yes
16
Q
What are the investigations for cutaneous lupus?
A
-
Biopsy - remember to look for Immunofluorescence
- On IMF - they will see the LUPUS BAND, IgG and C3 in the dermoepidermal junction.
-
Autoantibodies
- Subacute cutaneous lupus - Usually ENA positive.
- Discoid lupus - negative for autoantibodies
- Screen with ANA, dsDNA & Complement. If positive, these patients are more likely to develop Systemic Lupus.
17
Q
What are some complications of cutaneous lupus?
A
- Discoid lupus - can progress to SCC.
- SCLE - if Ro antibody positive - this antibody can cross the placenta causing neonatal heart block and SCLE in the neonate.
18
Q
What is the treatment of cutaneous lupus?
A
- Avoid UV exposure
- Oral prednisolone (10-40mg OD) - short term
-
Hydroxycholorquine Oral - 200-400mg daily over summer months.
- This with sunblock helps to reduce lesions in 80% of patients.
19
Q
What are the most common drugs to cause lupus?
A
- Hydralazine
- Procainamide
- Quinidine
- Isoniazid
- Diltiazem
- Sulphasalazine
- Minocycline
20
Q
How long does drug induced lupus last when the drug is stopped?
A
The symptoms usually clear within weeks.
21
Q
What are the symptoms of drug induced lupus?
A
- Muscle and joint pain
- Swelling
- Flu-like symptoms such as fatigue and fever
- Serositis
- Skin rash
- Raynaud’s phenomenon
- Positive ANA test results
22
Q
What antibodies are present in drug induced lupus?
A
- Positive ANA
- Negative Anti-ds DNA
- Anti-histone antibodies. (In 75% but not specific)
23
Q
What is this?
A
Neonatal Lupus
24
Q
What is this?
A
Lupus Profundus
- (Lupus that affects the deep paniniculus)
- Usually affects the face.
25
What is this?
Lupus Tumidus
* **Cherry pink dermal inflammatory plaques** and **patches**.
* Stimulated by sunshine.
* Disappears in the winter.
26
In **dermatomyositis**, there are two peak ages. What are they?
1. **Childhood type** - before the age of 10 years - benign.
2. **Adult** (age 40-60 years) - associated with paraneoplasms.
27
If someone developed dermatomyositis in adulthood,
what else should you do?
Seek an underlying malignancy
28
What rash is seen around the eyes/on the face in dermatomyositis?
Heliotropic rash
Purplish rash around the eyes, forehead, temples and cheeks.
Oedema can also occur around the eyes and chest.
29
What is this in dermatomyositis?
Gottron's Papules
A purplish-red rash on the dorsum of the hands.
30
What is dermatomyositis sensitive to?
The Sun
The rash gets worse with the sun.
31
What is **another feature** of **dermatomyositis** apart from the cutaneous symptoms?
**Musle Weakness**
* Usually proximal muscles
* If severe, patient can be laryngeal weakness and difficulty breathing.
32
What investigations should be done in Dermatomyositis?
* Blood tests
* **Autoantibodies**
* Do DsDNA and Lupus Anticoagulant (To Exclude Lupus
* ANA - Positive in 80%
* Anti-Ro & Anti-Jo
* **FBC**, **U&Es** and **LFTs**
* **CRP** & **CK** should be raised.
* **Biopsy** with **H&E** - is usually **unremarkable**.
* **EMG** - to confirm myopathy.
* **Muscle Biopsy**
* **MRI** - may show proximal muscle oedema.
33
What are 2 important associated conditions in dermatomyositis?
1. **Malignancy** in **50%** of **adult** patients with dermatomyositis
* **Breast** and **Ovarian** in women.
* **Prostatic**, **Lung** and **Colon** Cancer in Men.
2. **Systemic Lupus** or Mixed Connective Tissue Disease
34
What is the treatment for dermatomyositis?
* Oral Prednisolone and then switch to:
* Methotrexate, Azathioprine or cyclosporin for maintenance.
35
Once you treat an underlying malignancy in deramtomyositis, what happens?
The symptoms of dermatomyositis settle
36
What is the treatment for cutaneous disease in Dermatomyositis?
200-400mg of hydroxychloroquine daily.
37
Scleroderma can be a spectrum of conditions. How do you break it down?
Scleroderma
* **Vasculopathy** - Raynaud's phenomenon
* **Systemic**
* Limited systemic sclerosis
* Limited Cutaneous Systemic Sclerosis
* **Localised**
* Morphoea - localised and generalised
* Scleroderma
38
What is this?
Tell me a bit about it?
**Morphoea**
* **Localised plaques** of **collagen**
* It is due to a **build up of collagen by fibroblasts** underneath the skin.
* **Females** \> Males
* **Any age**
* **Woody** and **atrophic feeling** to the skin.
39
What is this?
**Linear Morphoea**
* More **common** in **childhood**
* Usually as **indurated lesions** in the **extremeties**.
* **Treat aggresively** otherwise it **spreads** to bones, muscle and soft tissue.
40
What is this?
**En Coup De Sabre Morphoea**
41
What is the treatment for Morphoea?
* **Initially**: **Superpotent** topical **steroids**
* **Narrow** band **UVB** if **extensive**
* **Linear Moprhoea** - **refer** to consultant dermatologist.
* They may beed immunosuppresive therapy - methotrexate etc.
* **Consider** **Borrelia Burgdorferi serology** (Lyme's disease) as Morphoea can occur after this.
42
What is CREST Syndrome?
It is a **limited form** of **systemic sclerosis** involving:
* **C**alcinosis
* **R**aynaudsd
* **E**osophageal Dysmotility
* **S**clerodactyly
* **T**elangiectasia
43
In CREST Syndrome, what is often the first sign to appear?
Raynauds Syndrome
| (Often by Years)
44
What autoantibody is positive in CREST?
Anticentromere antibody
45
Is linear morphoea symmetrical or unliateral?
Unilateral
46
What can happen in linear morphoea if untreated?
It can lead to **limb contractures**.
