MSK - CTD / autoimmune Flashcards
1
Q
What is systemic sclerosis?
A
An autoimmune connective tissue disease
Features:
- 4 x more common in women
- Scleroderma of skin (skin fibrosis):
- Hardening / scarring
- Red
- Scaly
- Internal organ fibrosis:
- Lungs - many patients have impaired lung function on pulmonary function tests (may not feel SoB) + some can develop pulmonary HTN –> leads to right-sided heart failure
- Heart - pulmonary HTN –> right-sided heart failure
- GI tract - reflux oesophagitis, oseophageal strictures, malabsorption and many others.
- Kidneys - renal involvement = poor prognosis, scleroderma renal crisis (symptoms: malignant HTN, hyperteninaemia, azotemia and microangiopathic haemolytic anaemia)
- Microvascular abnormalities e.g. Raynaud’s
2
Q
There are 3 patterns of systemic sclerosis - what are they?
A
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
- Scleroderma (without internal organ involvement)
3
Q
What are the features following patterns of systemic sclerosis:
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
- Scleroderma (without internal organ involvement)
A
-
Limited cutaneous systemic sclerosis
- Scleroderma affects face, hands and feet predominantly
- Associated with ACA antibodies (anti-centromere antibodies) 70-80%
- Raynaud’s may be 1st sign
- Used to be called CREST syndrome
-
Diffuse cutaneous systemic sclerosis
- Scleroderma affects trunk and proximal limbs predominately
- Associated with scl-70 antibodies (anti-topoisomerase I antibody) 40%
- Most common cause of death is due to resp issues: ILD and pulmonary HTN
- Poor prognosis
-
Scleroderma (without internal organ involvement)
- Tightening + fibrosing of skin
- Skin lesions can manifest as plaques (patch shape) or linear (resembles a longitudinal line)
4
Q
What is CREST syndrome?
A
The old name for limited cutaneous systemic sclerosis
CREST = 5 main features:
- C = calcinosis - soft tissue deposits of calcium
- R = Raynaud’s phenomenon - stess/cold induce vasoconstriction –> white-blue-red transition in skin colour
- E = esophageal dysmotility - dysphagia, needing liquid to swallow solids
- S = sclerodactyly - local thickening + tightening of skin - can occur so much as to cause fingers to curl + lose mobility
- T = telangiectasia (dilated capillaries) - face, palms, mucous membranes - no. tend to increase over time
5
Q
Which antibodies might be +ve on serology in systemic sclerosis?
A
- ANA (antinuclear antibody) > 90%
- ACA (anti-centromere antibody) - often in limited systemic sclerosis
- Anti-Scl-70 (anti-topoisomerase I antibody) - often in diffuse systemic sclerosis
- RhF (rhematoid factor) - +ve in ~ 30% of systemic sclerosis
6
Q
What monitoring do systemic sclerosis patients require?
A
- Annual echocardiogram - to monitor for pulmonary HTN / right-sided heart failure
- Spirometry (lung function tests) to monitor respiratory impact
- Regular clinic appointments if there is renal involvement
7
Q
How is systemic sclerosis managed?
A
- No cure
- Monitor BP + U+Es (renal function)
- Pharmacological:
- Immunosuppressants - with organ involvement or progressive skin disease e.g. IV cyclophosphamide
- ACE-I or ARBs –> ↓ risk of renal crisis
- Manage Raynaud’s if present:
- Keep warm
- Smoking cessation
- Nifedipine (Ca2+ channel blocker) 5-20 mg/8h PO - may help
- Sildenafil (viagra) - may help
- Rarely - prostagladin infusion over 3-5 days (need to be monitored due to cardiac side effects)
8
Q
Sclerodactyly is a feature of CREST syndrome (Limited cutaneous systemic sclerosis) but looks very similar to a complication of diabetes mellitus - what complication?
A
Diabetic Chieroarthropathy
Features:
- Thickened skin + limited joint mobility of hands / fingers
- Results in flexion contractures
- Seen in ~ 30% of longstanding diabetic patients
- Classically: patients can’t put palms of hands/fingers flat against each other ‘prayer sign’ - +ve when gap between palms
9
Q
What is the difference between primary and secondary Raynaud’s?
A
- Primary = Raynaud’s disease
- Secondary = Raynaud’s phenomenon (occurs secondary to underlying pathology)
10
Q
What is Raynaud’s disease/phenomenon?
A
Raynaud’s = peripheral digital ischaemia due to paroxysmal vasospasm precipitated by cold or emotion
Features:
- Fingers / toes ache
- Colour change: pale (ischaemia) –> blue (deoxygenation / cyanosis) –> red (reactive hyperaemia)
- Raynaud’s disease F:M = 9:1
11
Q
How is Raynaud’s managed?
A
- Nifedipine (Ca2+ channel blocker) 1st line: initially 5mg TDS PO –> then adjust according to response to 20mg TDS PO
- Sildenafil (viagra)
- IV prostacyclin (epoprostenol) infusions over 3-5 days - needs close monitoring due to cardiac side effects, but can provide relief for weeks/months
- Keep warm (hand warmers)
- Smoking cessation
- Digital sympathectomy for severe disease (cutting of sympathetic nerve or removal of a ganglion)
12
Q
What conditions can cause Raynayd’s phenomenon?
A
Secondary causes:
- Connective tissue disorders: systemic sclerosis/scleroderma (most common), SLE
- Rheumatoid arthritis
- Leukaemia
- Type I cryoglobulinaemia - pathological cold sensitive antibodies (cryoglobulins) in blood become insoluable at low temps
- Cold aglutinins - rare autoimmune condition which causes agglutination of RBCs at low temps
- Using vibrating tools
- Drugs: COCP
13
Q
Systemic sclerosis / scleroderma is the most common cause of Raynaud’s phenomenon. It is a connective tissue disease - what patient factors/symptoms mioght indicate an underlying connective tissue disease?
A
- Raynaud’s onset after 40 yrs
- Unilateral symptoms
- Rashes
- Presence of autoantibodies (autoimmune condition)
- Features of RA, SLE e.g. arthritis, recurrent miscarriages
- Hands: digitial ulcers, calcinosis
14
Q
What is antiphospholipid syndrome (APLS)?
A
APLS is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies
- Can be primary or secondary to other conditions e.g. SLE
15
Q
What are the common features of APLS?
A
Features:
- Venous + arterial thromboses
- Recurrent miscarriage, stillbirth, preterm delivery
- Thrombocytopenia (↓ platelets)
-
Livedo Reticularis - mottled reticulated (net-like) vascular pattern on skin (often legs)
- Caused by swelling of venules due to obstruction of capillaries by small blood clots
- Paradoxical rise in aPTT (activated partial thromboplastin time) - measures integrity of intrinsic clotting system + common clotting pathway
- This is due to an ex-vivo reaction of the lupus anticoagulant (immunoglobulin which is prothrombotic) autoantibodies with phospholipids involved in the coagulation cascasde
- Pre-eclampsia
- Pulmonary HTN
16
Q
What conditions can APLS occur secondary to?
A
- SLE (main one)
- Other autoimmune disorders
- Lymphoproliferative disorders e.g. lymphomas, leukemias
- Phenothiazines (rare) - used to treated schizophrenia / psychotic disorders
17
Q
How is ATLS managed for the following evnets/scenarios?
- Initial venous thromboembolic events
- Recurrent venous thromboembolic events
- Arterial thrombosis
A
- Initial venous thromboembolic events:
- Warfarin - target INR of 2-3 for 6 months
- Recurrent venous thromboembolic events:
- Lifelong warfarin - target INR 2-3 OR 3-4 (if thromboembolic event occured whilst taking warfarin)
- Arterial thrombosis:
- Lifelong warfarin - target INR of 2-3
N.B. Heparin is used instead of warfarin pre-pregnancy or during pregnancy as warfarin = cross the placenta and is teratogenic
18
Q
What are the diagnostic criteria for antiphospholipid syndrome (updated Sapporo APLS classification or Sydney classification)?
A
-
One of the following clinical findings:
- Vascular thrombosis:
- ≥ 1 episodes of arterial, venous or small-vessel thrombosis of any organ, without vessel wall inflammation (often DVT or PE for veins and TIA/Stroke for arteries)
-
Pregnancy morbidity:
- ≥ 1 foetal deaths of normal fetuses at > 10w gestation
- ≥ 1 premature births of normal fetuses before 34th week of gestation (prematurity must be due to eclampsia, pre-eclampsia or placental insufficiency)
- ≥ 3 consecutive spontaneous abortions before 10th week gestation (hormonal, chromosomal or maternal abnormalities must be ruled out)
- Vascular thrombosis:
-
One of the following lab findings:
- Detection of lupus anticoagulant in plasma on 2 or more occasions, > 3 months apart
- Detection of IgG or IgM subtype of anti-cardiolipin antibodies in serum or plasma on 2 or more occasions, > 3 months apart
- Detection of Anti-beta2 glycoprotein-1 antibody of IgG and/or IgM isotype in serum or plasma on 2 or more occasions, > 3 months apart
19
Q
What is sarcoidosis and what are its features?
A
It is a multisystem disorder of unkown aetiology - characterised by:
Features:
- Non-caseating granulomas
- Caseating = necrosis in which tissue has soft cheese-like appearance
- More comon in young adults + African descent
- Acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
- Insidious: SoB, non-productive cough, malaise, weight-loss
- Skin: lupus pernio (chronic indurated red/purplish lesion on nose, ears, cheeks, lips, forhead)
- Hypercalcaemia - macrophages in the granulomas cause increased conversion of Vit D to active form i.e. calcitriol –> ↑ [Ca2+]
20
Q
Describe lupus pernio?
A
Chronic red/purplish indurated lesion on
skin: nose, cheeks, ears, lips, forhead - seen in sarcoidosis
Lupus pernio = misnomer as it has nothing to do with lupus or pernio
21
Q
What is Lofgren’s syndrome?
A
It is an acute form of sarcoidosis characterised by:
Features (first 3 = triad –> if present then diagnose):
- Bilateral hilar lymphadenopathy (BHL)
- Erythema nodosum
- Polyarthralgia / arthritis - often acute and involving lower limbs
- Fever
- More common in Scandanavian population
N.B. It usually carries an excellent prognosis
22
Q
When a patient presents with a rash as a symptom, what questions should you ask?
A
- How long have you had the rash?
- Is it always there?
- What does it look like?
- Do you have a photo of it? (if it’s not there now)
- What areas are affected by the rash?
- Is it itchy? painful?
- Does anything make it worse?
- New medication, chemicals (allergy), sunlight (photosensitivity)
23
Q
What is the most likely explanation for peripheral oedema and heavy proteinuria in combination?
A
Nephrotic Syndrome
Can be caused by:
- Systemic disorders affecting kidneys e.g. amyloidosis, lupus, diabetes
- OR
- Primary renal disorders e.g. membranous nephropathy, focal segmental glomerulosclerosis
24
Q
What is nephrotic syndrome?
A
- Heavy Proteinuria ( >3.5g in 24hrs, ++++ Protein Dipstick, urine looks frothy)
- Hypoalbuminaemia (Albumin lost in urine due to gaps in podocytes of glomerulus)
- Oedema (Albumin lost in urine –> ↓ intravascular oncotic pressure –> fluid moves into into surrounding tissues)
- Hyperlipidemia (Liver compensates for hypoalbuminaemia by ↑ production, but side effect is ↑ lipid production)
Also you will see:
- Lipiduria
- Loss of antithrombin-III, proteins C and S
25
What are the defining characteristics of nephritic syndrome?
1. **Haematuria** (+++ Blood dipstick - microscopic or macroscopic/cola-urine)
* Possible 'red cell casts' = microscopic cylindrical structure, present in urine, produced in nephrons in diseased states
* Haematuria occurs due to gaps in podocytes of glomerulus
2. **Proteinuria** (++ Protein dipstick = small amount, usually \< 2g/24hrs)
3. **Hypertension** (usually mild - caused by salt + water retention)
4. **Low Urine Volume** (i.e. oliguria, \< 300 ml/day) - due to ↓ renal function
You also see:
* Oedema - periorbital, leg or sacral
* Uraemia - pathological features associated with azotemia (↑ blood [urea] )
26
Describe the appearance of malar rash / butterfly rash.
What condition is it often seen in?
It is a **red**/**purpleish**, mildly **scaly**, rash across both **cheeks** and the **bridge of the nose** (nasolabial folds are spared)
It is associated with **SLE**
27
What is the epidemiology of SLE?
* W \>\>\> M (9:1)
* More common in Afro-Caribbeans and Asian communities
* Onset usually 20-40yrs
28
What is the pathophysiology of SLE?
* Autoimmune disease
* Associated with HLA B8, DR2, DR3
* Immune dysregulation thought to cause immune complex deposition which can affect any organ e.g. skin, joints, kidneys and brain
29
What are the features of SLE?
**General**:
* Fatigue
* Low-grade fever
* Mouth ulcers
* Lymphadenopathy
**Skin**:
* Malar (butterfly) rash: spares nasolabial folds
* Discoid rash: scaly, erythematous, well demarcated rash in sun-exposed areas
* Lesions may progress to become pigmented and hyperkeratotic before becoming atrophic
* Photosensitivity
* Raynaud's phenomenon
* Livedo reticularis
* Non-scarring alopecia
MSK:
* arthralgia
* non-erosive arthritis
CVD:
* Pericarditis (most common cardiac manifestation)
* Myocarditis
* Venous or arterial thrombosis
Resp:
* Pleurisy/pleuritis (more common than pericarditis)
* Fibrosing alveolitis (IPF)
Renal:
* Proteinuria
* Glomerulonephritis (most common type = diffuse proliferative glomerulonephritis)
Neuro-psych:
* Anxiety and depression
* Psychosis
* Seizures
30
What investigations might you order in suspected SLE?
* **FBC** - looking for thrombocytopenia, leukopenia due to lymphopenia, anaemia
* ESR + CRP - SLE can have ↑ ESR but normal CRP due to immunoglobulins increasing ESR, if ↑ CRP then exclude infection
* **U+Es** and **BUN** - SLE with renal manifestations may have ↑ BUN and creatinine
* **Urinanalysis** e.g. MSU - assess renal involvement, haematuria, casts, proteinuria
* **24h urine collection** or **protein-creatinine ratio** - if urinalysis is abnormal, may show proteinuria
* **CXR** - pleural effusion, cardiomegaly, IPF
* **Joint X-ray** - in pts with arthalgia - should show inflammation, nonerosive arthritis
* **ECG** - exclude other causes of chest pain
* **Immunology**:
* 99% are **ANA** positive - ANA not specific to SLE as can be +ve in dRA, systemic sclerosis, Sjogren's etc.
* **anti-dsDNA**: highly specific (\> 99%), but less sensitive (70%)
* **anti-Smith**: most specific (\> 99%), sensitivity (30%)
* 20% are rheumatoid factor positive
* also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
* **Anti-phospholipid antibodies** - in anyone with history of VTE, miscarriages or prolonged aPTT
N.B. there are LOADS more tests as SLE affects many systems
31
Is a +ve ANA always diagnostic of connective tissue disease?
NO!!
* A +ve ANA is seen in a number of rheumatological and autoimmune conditions and also healthy people
* ~ 5% of the population will have a positive ANA test
32
For the following conditions which anti-nuclear antibodies (ANA) are associated with them?
* SLE
* Sjogren's
* Dermatomyositis (type of chronic muscle inflammation)
* SCLE (subacute cutaneous lupus erythematosus)
* Diffuse cutaneous systemic scleroderma
* CREST (limited cutaneous systemic sclerosis)
* MCTD (mixed conntective tissue disease)
* SLE = **dsRNA**, anti-**Smith**, anti-**nRNP**
* Sjogren's = **Ro** (**SSA**), **La** (**SSB**)
* Dermatomyositis = anti-**Jo-1**
* SCLE = **Ro** (**SSA**), **La** (**SSB**)
* Diffuse cutaneous systemic scleroderma = anti-**Scl-70**
* CREST = anti-**centromere**
* MCTD = anti-**nRNP**
33
What important considerations are there in a patient with SLE who wishes to become pregnant?
* Pregnancy planning is required as patient's SLE needs to be **stable**
* If on **immunosuppressants** --\> ensure compatible with pregnancy
* Check for presence of **Ro** (SSA), **La** (SSB) and **antiphospholipids** antibodies
* Ro (SSA) and La (SSB) +ve = risk of neonatal lupus as they can cross placenta --\> lupus rash, complete heart block and blood abnormalities e.g. cytopaenias (↓ in mature blood cells e.g. thrombocytopenia)
34
What renal complication can SLE cause?
How is this complication monitored?
**Lupus nephritis**
Monitor: **urinalysis** at regular check-up appointments to **rule out proteinuria**
35
What are the classes in the WHO classification of lupus nephritis?
* class I: normal kidney
* class II: mesangial glomerulonephritis
* class III: focal (and segmental) proliferative glomerulonephritis
* class IV: diffuse proliferative glomerulonephritis
* Most common + severe form
* class V: diffuse membranous glomerulonephritis
* class VI: sclerosing glomerulonephritis
36
How is SLE managed?
* Lifestyle: smoking cessation, sun protection, exercise
* Treat hypertension
* **Corticosteroids** - if clinical evidence of disease
* GI + bone protection if use long term e.g. PPI + bisphosphonates
* **Hydroxychloroquine** (DMARD) - joint symptoms, lupus nephritis, flares etc.
* **Immunosuppressants**:
* Cyclophosphamide
* Azathiopine
* Tacrolimus
37
What criteria are included for the diagnosis of SLE?
N.B. some common symptoms are not included and the criteria weren't designed for diagnostic purposes but are used this way.
1997 ACR Classification of SLE:
**4 or more** = diagnose SLE
1. **Malar rash**
* Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
2. **Discoid rash**
* Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions
3. **Photosensitivity**
* Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
4. **Oral ulcers**
* Oral or nasopharyngeal ulceration, usually painless, observed by physician
5. **Nonerosive Arthritis**
* Involving ≥ 2 peripheral joints, characterized by tenderness, swelling, or effusion
6. **Pleuritis or Pericarditis**
* Pleuritis – convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion
* OR
* Pericarditis – documented by electrocardigram or rub or evidence of pericardial effusion
7. **Renal Disorder**
* Persistent proteinuria \> 0.5 g/day or \> +++ on urine dip
* OR
* Cellular casts – may be red cell, hemoglobin, granular, tubular, or mixed
8. **Neurologic Disorder:**
* Seizures – in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance
* OR
* Psychosis – in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance
9. **Hematologic Disorder:**
* Hemolytic anemia – with reticulocytosis
* OR
* Leukopenia – \< 4,000/mm3 on ≥ 2 occasions
* OR
* Lyphopenia – \< 1,500/ mm3 on ≥ 2 occasions
* OR
* Thrombocytopenia – \< 100,000/ mm3
10. **Immunologic Disorder:**
* Anti-DNA
* OR
* Anti-Smith
* OR
* Positive finding of antiphospholipid antibodies
11. **Positive Antinuclear Antibody (ANA)**
* ****Very sensitive but not specific
38
What is the single most useful test to monitor for renal involvement and relapses after treatment in a 40 year old man diagnoses with SLE?
* Blood pressure
* dsDNA
* ESR
* Urinalysis
* U+Es
**Urinalysis**
* dsDNA and ESR are helpful as a markers of disease and going up with disease respectively, but aren't the best options
* U+Es - This is helpful but renal involvement may not always result in an abnormality of the U&Es
39
Which of the following patients have anti-phospholipid syndrome?
1. A 25 year old lady who presents with an unprovoked deep vein thrombosis
2. A 33 year old man who tests positive for Lupus anticoagulant and anti-cardiolipin antibodies twice at a 2 month interval
3. A 33 year old man who tests positive for Lupus anticoagulant and anti-cardiolipin antibodies twice at a 3 month interval, the first test is done after a pulmonary embolus
4. A 37 year old lady who suffers three miscarriages
5. A 54 year old lady with Factor V Leiden thrombophilia who has a pulmonary embolus
Answer (**3**) - Interval should be a minimum of 3 months. He meets the clinical and lab criteria
1. She could have but the diagnosis is based on clinical and lab criteria
2. Interval should be a minimum of 3 months, also laboratory criteria only
3. Interval should be a minimum of 3 months. He meets the clinical and lab criteria
4. Clinical criteria only
5. This is the most common inherited thrombophilia but is nothing to do with anti-phospholipid syndrome
40
What is Sjogren's syndrome?
Sjogren's is an **autoimmune** disorder affecting **exocrine glands** resulting in **dry mucosal surfaces**
* Primary or secondary to RA and other connective tissue disorders e.g. SLE or systemic sclerosis (usually develops ~10yrs after initial onset of RA/CTD)
* F \>\>\>\> M (9:1)
* 20's - 30's (most common ages)
41
Sjogren's syndrome is associated with an increased risk of what?
Lymphoid malignancy (**Lymphoma**) 40-60 fold
42
What are the features of Sjogren's syndrome?
* **Dry eyes** (keratoconjunctivitis sicca)
* Sensation of sand/gravel, itch or burning, multiple episodes weekly for \> 3 months
* **Dry mouth**
* ****Can cause poor dentition e.g. dental caries, as saliva is protective - means dentists often notice Sjogren's 1st
* **Vaginal dryness**
* Arthralgia
* Fatigue, myalgia
* Raynaud's
* Sensory polyneuropathy - usually purely sensory loss but pts commonly have neuropathic pain
* Recurrent episodes of parotitis
* Renal tubular acidosis (usually subclinical)
43
What investigations might you order for suspected Sjogren's syndrome?
* Rheumatoid factor (**RF**) +ve (~ **100%** of pts)
* **ANA** +ve in **70%**
* anti-**Ro** (SSA) antibodies in **70%** of patients with PSS (primary)
* anti-**La** (SSB) antibodies in **30%** of patients with PSS (primary)
* **Schirmer's test** - filter paper (I-DEW strips) near conjunctival sac to measure tear formation ( 15ml in 5 mins is normal )
* Histology - focal lymphocytic infiltration
* Also:
* Hypergammaglobulinaemia
* low C4 (complement component 4)
44
How is Sjogren's syndrome managed?
* **Artificial tears** and **saliva**
* Humidifiers - reduce loss of sections due to evaporation
* **Pilocarpine** 5mg TDS PO - muscarinic cholinergic receptor agonist may help stimulate saliva production
* Sugar free sweet to encourage salivation
* Massage face in morning to encourage mucous gland activity
45
What condition can longstanding Sjogren's cause?
**AA amyloidosis**
* This is a type of amyloidosis, characterised by abnormal **deposition of fibres of insoluable protein** in the extracellular space of various tissues / organs
* Protein = serum amyloid A protein
* AA amyloidosis is a complication of several inflammatory diseases / infections e.g. RA, ankylosing spondylitis, Crohns, UC etc.
* Most common presentation = renal:
* Proteinuria
* Nephrotic syndrome
* Progressive CKD --\> ESKD --\> dialysis / transplant
46
What blood test is commonly used to test for TB?
**QuantiFERON**-TB Gold (QFT)
* Used to detect Mycobacterium tuberculosis - bacteria which causes TB
* It is unaffected by previous BCG vaccination
* It can't distinguish between ACTIVE and LATENT TB infection
* Once a pt has had TB, they will always test +ve on QuantiFERON test
47
What retinal side effect can long-term hydroxychloroquine have?
Chloroquine retinopathy - also called **Bull's eye maculopathy**
* It is a form of toxic retinopathy
* Earliest signs include bilateral paracentral visual field changes
* Associated with gradual decreasing visual acuity
* Can cause widespread funal atrophy and visual loss
48
What factors might suggest underlying connective tissue disease in a patient with Raynaud's?
* Onset \> 40 years
* Unilateral symptoms
* Rashes
* Presence of autoantibodies
* Features suggestive of RA or SLE e.g. arthritis or recurrent miscarriages
* Digital ulcers or calcinosis
49
What is the typical presentation for Raynaud's disease?
Young (e.g. 30 yrs) female with bilateral fingers going pale white in cold, then blue then red.
50
What are common features of Raynaud's phenomenon (secondary)?
* Digit pallor (must be present)
* Blue and/or red discolouration of digits (one must be present)
* Dilated, torturous capillary nail beds - looks like small red pen marks (mainly in secondary)
51
What are the risk factors for Raynaud's phenomenon (secondary)?
* Female
* FHx of Raynaud's
* Presence of connective tissue disease:
* RP occurs in \>90% with systemic sclerosis, \>80% with mixed connective tissue disease, 40-45% with SLE, 20% RA and 13-17% with Sjogren's
* Vibration injury
* Smoking
* Beurger's disease - inflammation + thrombosis of small + medium sized BVs, often in the legs, causing gangrene
* Colder climate
52
