Rheumatology Flashcards
Rheumatoid arthritis - state the following:
- Pathophysiology
- Typical patient
- Presentation
- Investigations
- Management
Pathophysiology:
- Chronic autoimmune inflammatory condition
- Autoantibodies attack the synovial membranes of joints, bursa and tendon sheaths, leading to chronic inflammation (synovitis)
Typical patient:
- Middle age onset
- (3x) F > M
- Family history of RA
Presentation:
- Joint pain / swelling / stiffness, particularly of the small/distal joints
- Symmetrical and affects many joints
- Onset can be sudden or more gradual
- Pain is worse in the morning and worse on rest
- May have some signs in the hands
Systemic symptoms:
- Fatigue
- Weight loss
- Flu-like illness
- Myalgia and weakness
Investigations:
Diagnosis is clinical but want back up of:
- Specific bloods for rheumatoid factor (RF) and then anti-CCP if RF negative
- Routine bloods e.g. FBC, ESR, CRP
- X-rays hands and feet
- Ultrasound of joints to confirm synovitis if unsure
Management:
1. One early DMARD (disease modifying anti-rheumatic drugs) e.g. Methotrexate, Leflunomide or Sulfasalazine
2. Two DMARDs
3. Methotrexate + biologic (e.g. TNF inhibitor Infliximab)
4. Methotrexate + Rituximab (Anti-CD20)
- Use of steroids to settle first presentation and for flares
- Surgery can be used to improve joint complications of RA but not used as much now due to early interventions
List some signs in the hands of someone with established rheumatoid arthritis
- Ulnar deviation of fingers (at MCP joints)
- Bouchard nodes (flexed PIP and extended DIP)
- Swan neck deformity (extended PIP and flexed DIP)
- Z shaped deformity of thumb
+ rheumatoid nodules on elbows
List some extra-articular manifestations of rheumatoid arthritis (3 CAPS)
(3)CAPS
3 C’s:
- Carpal tunnel syndrome
- Cardiovascular disease (increased risk)
- Cord compression (atlanto-axial subluxation)
3 A’s:
- Anaemia of chronic disease (normochromic and normocytic)
- Amyloidosis
- Arteritis
3 P’s:
- Pulmonary disease e.g. fibrosis, bronchiolitis obliterans, bronchiectasis
- Pleural disease
- Pericarditis
3 S’s:
- Sjogren’s syndrome (secondary)
- Scleritis / episcleritis
- Splenomegaly (Felty’s syndrome if with neutropaenia)
List the x-ray changes seen in rheumatoid arthritis
LESS!
Loss of joint space
Erosions
Soft tissue swelling
See-through bones (osteopenia)
Outline the DAS28 score for rheumatoid factor and what it’s used for
DAS 28 score - disease activity score of 28 joints
Points given for:
- Swollen joints
- Tender joints
- ESR / CRP level
Useful for monitoring disease activity and response to treatment
List some side effects of Methotrexate
- GI upset e.g. N&V and diarrhoea
- Pulmonary fibrosis
- Mouth ulcers and mucositis
- Bone marrow suppression / leukopenia
- Thrombocytopenia
- Teratogenic
- Hepatotoxic
List some side effects of NSAIDs
- Indigestion
- Peptic ulcer disease
- VTE e.g. DVT/PE
- Peripheral oedema
- Slight increased risk of stroke and heart attack
List some side effects of corticosteroids e.g. Prednisolone
- Immunosuppression
- Diabetes mellitus
- Osteoporosis
- Suppression of HPA axis
- Teratogenic
- Increased BP
- Cataracts and glaucoma
- Oedema
Cushing’s syndrome
- Thin skin, easy bruising
- Obesity distributed centrally
- Increased hair growth (hirsutism)
- Striae
List some side effects of Sulfasalazine
- Myelosuppression
- Nausea
- Rash
- Oral ulcers
- Infertility / decreased sperm count
List some side effects of Biologic therapy (e.g. Adalimumab)
- Immunosuppression
- Reactivation of TB
- Allergic reaction / reaction at infusion site
Systemic lupus erythematosus (SLE) - state the following:
- Pathophysiology
- Typical patient
- Presentation
- Investigations
- Management
Pathophysiology:
- Systemic autoimmune condition targeting the connective tissue
- Characterised by ANA antibodies (anti-nuclear) against own cell nuclei in proteins
- Often has a relapsing-remitting course
Typical patient:
- Women
- Young-middle age
- Asian
Presentation:
- Usually young-middle aged adults
- F > M and Asians
- Photosensitive malar rash
- Systemic features: fatigue, weight loss, fever
- Hair loss
- Joint pain and inflammatory arthritis
- Myalgia
- Mouth ulcers
- Lymphadenopathy
- Raynaud’s
- SOB
- Chest pain
Investigations:
Criteria used based on clinical features and presence of anti-nuclear antibodies
- Specific bloods for ANA and anti-dsDNA antibodies
- Routine bloods e.g. FBC, ESR, CRP
- Further bloods e.g. C3, C4, immunoglobulins
- Urinalysis and protein:creatitine ratio to test for kidney involvement
- Renal biopsy if lupus nephritis suspected
Management:
First line:
- Hydroxychloroquine
- NSAIDs
- Prednisolone (steroids)
- Skin protection e.g. suncream
If not responding/severe:
- Immunosuppressants e.g. Methotrexate, Mycophenolate mofetil
- Biologics e.g. Rituximab
List some complications of SLE
Cardiovascular disease (HTN and coronary artery disease) = LEADING CAUSE OF DEATH
- Infection (disease + immunosuppressants)
- Lupus nephritis (inflammation in kidneys)
- Anaemia of chronic disease (bone marrow suppression)
- Pericarditis
- Pleuritis
- Interstitial lung disease (fibrosis)
- Neuropsychiatric (optic neuritis, transverse myelitis or psychosis)
- Recurrent miscarriages
- Antiphospholipid syndrome, leading to VTE
Gout - state the following:
- Pathophysiology
- Typical patient
- Presentation
- Investigations
- Management
Pathophysiology:
- Crystal arthropathy which occurs as a result of chronic hyperuraemia
- Sodium urate crystals are deposited in the joint causing joint to become painful, swollen and hot
Typical patient:
- Male
- Obese
- Family history
- Existing cardiovascular or renal disease
Presentation:
- Single painful, swollen and hot joint
- Acute, severe onset
Key differential should be septic arthritis
Investigations:
- Diagnosed clinically or by joint aspiration
- X-ray of affected joint
KEY = exclude septic arthritis
Management:
Acute attack
- NSAIDs
- Colchicine
- Steroids
Prophylaxis/prevention
- Allopurinol (only once acute attack has resolved)
- Lifestyle changes e.g. lose weight, hydration, limit alcohol and limit purine-based foods
List some risk factors for gout (modifiable and non-modifiable)
Modifiable
- Alcohol intake / high purine diet
- Obesity
- Diuretics
Non-modifiable
- Male
- Family history
- Existing cardiovascular or renal disease
List some typical joints that are affected in gout
- Base of the big tole (MTP)
- Base of thumb (MCP)
- Bigger joints e.g. knee and ankle
List 3 things seen in a joint aspiration from a gouty joint
- Monosodium urate crystals (needle-shaped)
- Negative birefringent of polarised light
- Absence of bacteria
List 2 things that x-ray of a gout joint will show
- Punched out erosions (with overhanging edges and sclerotic margins)
- Lytic bone lesions
Joint space is generally maintained
Outline how pseudogout differs from gout
Pseudogout involves calcium pyrophosphate (rather than monosodium urate) crystals being deposited into joints
Osteoporosis - state the following:
- Pathophysiology
- Typical patient
- Presentation
- Investigations
- Management
Pathophysiology:
- Reduced density of bones (low bone mass) leading to reduced bone strength
- Increased risk of fractures
Typical patient
- Post-menopausal women
- White / south asian
Presentation:
- Generally not symptomatic
- Often discovered upon an unusual fracture e.g. low energy fracture
Investigations:
- FRAX assessment to assess risk
- DEXA scan to confirm (dual energy x-ray absorptiometry)
Management:
Based on results from a FRAX prediction or DEXA scan
Conservative
- Weight bearing exercise
- Limit alcohol intake
- Stop smoking
- Increase dietary calcium
Medical
- Vitamin D and calcium supplements (AdCal D3)
- Oral bisphosphonates (IV if not tolerated)
- Then Denosumab 2nd line
List the risk factors for developing osteoporosis (modifiable and non-modifiable)
Modifiable:
- Low mobility (non-weight bearing)
- Low calcium diet / low sun exposure (vitamin D deficiency)
- Smoking
- Excess alcohol intake
- Low BMI/weight
- Corticosteroids
Non-modifiable:
- Female
- Post-menopausal
- Family history of osteoporosis
- Increased age
- White or south asian
- Premature menopause
Outline how a DEXA scan is used to diagnose osteoporosis
DEXA scan is gold standard for diagnosis of osteoporosis
- Assesses the bone mineral density using x-rays
- Measures how much radiation is absorbed by the bones, which indicates how dense the bones are
- Reading taken at the hip is key
- T score used (can also use Z score)
T score < - 2.5 = osteoporosis
T score > -1 = normal
T score between those two is osteopenia
Briefly outline how the FRAX score is calculated and it’s use in osteoporosis
FRAX score gives a risk of fragility fracture in the next 10 years (assess risk of osteoporosis)
Performed on individuals at risk of osteoporosis
- Women over 65
- Men over 75
- Anyone at increased risk e.g. low BMI, long-term steroids
Calculated using information e.g. age, BMI, co-morbidities, smoking, alcohol and family history
Can also enter results of a DEXA scan for a more accurate result
Overall gives a 10 year risk of having either:
- Major osteoporotic fracture
- Hip fracture
Osteomalacia - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management
Pathophysiology:
- Condition of ineffective bone mineralisation (soft bones)
- Caused by insufficient vitamin D
Presentation:
- Rickets if happens in childhood
May not have any symptoms!
- Bone pain
- Muscle pain / weakness
- Fatigue
- Fractures
Investigations:
- Serum vitamin D (25-hydroxyvitamin D)
- X-ray / DEXA scan showing osteopenia
Blood tests may also reveal
- Hypocalcaemia
- Hypophosphataemia
- Secondary hyperparathyroidism
Management:
- Vitamin D3 supplements (initial treatment followed by a life-long maintenance dose)
List some risk factors for developing osteomalacia
- Darker skin tone
- Reduced UV radiation exposure (lack of sunlight)
- Malabsorption disorders e.g. IBD, coeliac disease
Polymyalgia rheumatica (PMR) - state the following:
- Pathophysiology
- Typical patient
- Presentation
- How diagnosis is made
- Management
Pathophysiology:
- Inflammatory condition leading to pain and stiffness in: shoulders, pelvic girdle and neck
- Closely associated with GCA
Typical patient:
- Over 50
- Female
- Caucasian
- Association with GCA
Presentation:
- Pain and stiffness in: shoulders, pelvic girdle and neck
- Worse on movement
- Interferes with sleep
- Stiffness for > 45 mins in morning
- Systemic features e.g. weight loss, fatigue
- Upper arm tenderness
- Carpal tunnel syndrome
- Peripheral oedema
How diagnosis is made:
- Clinical diagnosis based on symptoms plus response to steroids
- May need to exclude other conditions
- Inflammatory markers usually raised also (ESR, CRP and plasma viscosity)
Management:
- Steroids e.g. Prednisolone (then assess response)
- Check after 1 week: if poor response, probably not PMR
- Check after 1 month: should expect 70% improvement, then start to wean off
Outline the mnemonic ‘don’t STOP’ for long-term steroid use
don’t stop steroids suddenly
Sick day rules (double dose)
Treatment card (steroid card)
Osteoporosis
PPI cover (gastric protection)
Ankylosing spondylitis - state the following:
- Pathophysiology
- Typical patient
- Presentation
- Investigation and special test
- Management
Pathophysiology:
- Inflammatory condition mainly affecting the spine
- Leads to progressive pain and stiffness in mainly the sacroiliac joints and the vertebral column joints
- Can progress to fusion of sacroiliac joints and spine
- Seronegative spondyloarthropathy: related to reactive arthritis and psoriatic arthritis (HLA-B27 gene)
Typical patient:
- Young male
- Late-teens or 20’s
Presentation:
- Gradual development > 3 months
- Lower back pain and stiffness
- Buttock/sacroiliac pain
- Worse with rest, better on movement
- Worse in morning and at night, can wake from sleep
- > 30 min early morning stiffness
Symptoms can fluctuate with flares
Investigation and special test:
- Blood tests e.g. CRP, ESR
- HLA B27 genetic test
- X-ray spine and sacrum
- MRI spine may show bone marrow oedema in early disease
- Special test: Schober’s test
Management:
Conservative
- Physiotherapy
- Avoid smoking
- Surgery if deformity
Medical
- NSAIDs to help with pain
- Steroids for flares
- MAB e.g. Adalimumab or Infliximab or Anti-TNF e.g. Etanercept
List some x-ray changes seen in ankylosing spondylitis (including the main one)
Main: bamboo spine (due to spine fusion)
- Squaring of vertebral bodies
- Subchondral sclerosis and erosions
- Syndesmophytes
- Ossification of ligaments, discs and joints
- Fusion of sacroiliac and facet/costovertebral joints
Osteoarthritis - state the following:
- Pathophysiology
- Typical patient
- Presentation
- How diagnosis is made
- Management
Pathophysiology:
- General wear and tear of joints (non-inflammatory)
- Imbalance of cartilage wearing down and chondrocyte repair
- Occurs in synovial joints
Typical patient:
- Female
- Increased age
- Obese
- Family history
Presentation:
- Joint pain, worse on movement
- Joint stiffness, worse throughout the day
- May have signs of OA in the hand
- History of risk factors
How diagnosis is made:
- Can be made without any investigations
- May do an x-ray to confirm
Management:
Conservative
- Patient education
- Weight loss
- Activity modification / physiotherapy
Medical
- Paracetamol / NSAIDs (topical or oral)
- Opiates e.g. Co-codamol or Morphine
- Intra-articular joint replacement
- Surgery joint replacement
Outline x-ray changes seen in osteoarthritis
LOSS!
Loss of joint space
Osteophytes
Subchondral sclerosis
Subchondral cysts
However, x-ray findings don’t always correlate with clinical symptoms
List some risk factors for osteoarthritis
- Increased age
- Female
- Obesity
- Previous trauma leading to articular damage
- Occupation
- Family history
List commonly affected joints in osteoarthritis
- Hips
- Knees
- Sacroiliac joints
- MCP (thumb)
- DIPs (hands)
- Wrist
- Cervical spine
List signs of osteoarthritis in the hands
- Squaring of base of thumb
- Heberden’s nodes (DIPs)
- Boucharde’s nodes (PIPs)
- Weak grip
- Reduced ROM
Fibromyalgia - state the following:
- Pathophysiology
- Typical patient
- Presentation
- How diagnosis is made
- Management
Pathophysiology:
- Disorder of central pain processing
- Can be triggered by a traumatic event or sleep deprivation
Presentation:
- Chronic widespread pain in all 4 quadrants of the body
- Allodynia (heightened and painful response to innocuous stimuli)
- Unrefreshed sleep
- Fatigue
- Numbness
- Headaches
- IBS / bladder syndrome
- Fibro-fog
- Depression and anxiety
How diagnosis is made:
- No specific diagnostic test
- May have tender points on palpation, but otherwise no abnormalities
- May want to rule out other differentials with blood tests
Management:
Conservative
- Improve sleep hygiene
- Increase physical activity levels
- CBT
Medical
- Low dose Amitriptyline
- Pregabalin or Gabapentin
List some risk factors for fibromyalgia
- Female (9:1)
- 40-50
- Obvious triggers e.g. emotional or physical event
Psoriatic arthritis - state the following:
- Pathophysiology
- 3 typical patterns of affected joints
- Presentation
- Investigation
- Management
Pathophysiology:
- An inflammatory arthritis associated with psoriasis
- Varies in severity, from mild pain/stiffness to complete joint destruction (arthritis mutilans)
- Occurs in 10% of patients with existing psoriasis
- Seronegative spondyloarthropathy: related to reactive arthritis and ankylosing spondylitis (HLA-B27 gene)
3 typical patterns of affected joints:
1. Symmetrical polyarthritis - similar to RA but MCP joints less likely
2. Asymmetrical pauciarthritis - mainly digits of hands and feet
3. Spondylotic pattern - back stiffness, sacroiliitis and atlantoaxial joint
Presentation:
- Joint pain/stiffness (as described previously)
- Psoriatic plaques
- Nail pitting
- Onycholysis
- Dactylitis (sausage fingers)
- Inflammation of tendon insertion points
- Anterior uveitis or conjunctivitis
Investigation:
- Routine bloods including CRP
- X-ray showing pencil-in-cup (central erosions of bone beside joints)
Management:
- Similar to RA management
- NSAIDs
- DMARDs
- TNF inhibitors
Reactive arthritis - state the following:
- Pathophysiology
- Presentation
- Investigation
- Management
Pathophysiology:
- Inflammation of synovial joint, as a reaction to a recent infective trigger
- Seronegative spondyloarthropathy: related to reactive arthritis and ankylosing spondylitis (HLA-B27 gene)
Presentation:
- Generally an acute monoarthritis
- Hot, swollen and painful joint
Investigation:
- Joint aspiration and send for gram staining and MC&S
- Crystal examination if gout suspected
Management:
- Give Abx until possibility of septic arthritis can be excluded
- Treat underlying infection
- NSAIDs
- Steroid infection into affected joint
- Systemic steroids may be needed if multiple joints affected
Most cases resolve within 6 months (may need DMARDs or anti-TNF if not)
List 2 conditions that can commonly cause reactive arthritis
- Gastroenteritis
- Sexually transmitted infection (chlamydia or gonorrhoea)
List some conditions associated with reactive arthritis (helpful phrase)
- Balanitis
- Anterior uveitis
- Bilateral conjunctivitis
+ reactive arthritis
‘can’t see, can’t pee, can’t climb a tree’
Systemic sclerosis - state the following:
- Pathophysiology (list 2 types)
- Autoantibodies (2)
- Management
Pathophysiology:
- Autoimmune inflammatory and fibrotic connective tissue condition
- Cause is actually unclear
- Mostly affects the skin, but also affects internal organs
2 types:
1. Limited
2. Diffuse
Autoantibodies:
- ANA antibodies
- Anti-centromere antibodies (limited mainly)
- Anti Scl-70 antibodies (diffuse mainly)
- Nailfold capillaroscopy (helps to find systemic sclerosis in patients with Raynaud’s)
Management:
Conservative
- No smoking
- Emollients
- Occupational /physiotherapy
Medical:
- Manage complications
- Can use steroids and immunosuppressants in diffuse
List some features of limited and diffuse systemic sclerosis
Limited: CREST
Calcinosis
Raynaud’s
Eosophageal dysmotility
Sclerodactyly
Telangiectasia
Diffuse:
Cardiovascular problems - HTN and coronary artery disease
Lung problems - pulmonary HTN and pulmonary fibrosis
Kidney problems - glomerulonephritis
Giant cell arteritis (GCA) - state the following:
- Pathophysiology
- Typical patient
- Presentation
- Investigation
- Management
Pathophysiology:
- Systemic vasculitis of the medium and large arteries
- Strong link with polymyalgia rheumatica
Typical patient:
- Over 50
- Female
- Caucasian
- Association with polymyalgia rheumatica
Presentation:
- Severe unilateral temporal headache
- Scalp tenderness
- Jaw claudication
- Vision changes (blurred or double vision)
- Systemic symptoms e.g. fever, muscle aches, fatigue, loss of appetite
Investigation:
- Temporal artery biopsy which show multinucleated giant cells (although can be falsely negative)
- Bloods including ESR
Management:
Initial
- High dose steroids (Prednisolone)
- Aspirin
- PPI while on steroids
If vision changes - same day referral to ophthalmologist
If no vision changes - discuss with specialist and refer using GCA fast-track pathway
Continue on steroids until symptoms have resolved, can take years
List some complications of giant cell arteritis (early and later)
Early:
- Permanent vision loss
- Stroke
Late:
- Relapse of GCA
- Long-term steroid complications
- Stroke
- Aortitis (risk of aortic aneurysm and dissection)
Sjogren’s syndrome - state the following:
- Pathophysiology
- Typical patient
- Presentation
- Investigation
- Management
Pathophysiology:
- Autoimmune condition targeted against exocrine glands
- Leads to dry mucous membranes such as dry eyes, dry mouth and dry vagina
2 types:
Primary Sjogren’s: occurs alone
Secondary Sjogren’s: associated with SLE or RA
Typical patient:
- Female (80%)
- 40-50
- Caucasian
Presentation: (MADFRED)
Myalgia
Arthralgia
Dry eyes
Fatigue
Raynaud’s
Enlarged parotids
Dry mouth
Investigations:
- Specific blood tests for anti-Ro and anti-La
- Schirmer’s test (tear production/collection)
- May need salivary gland biopsy
Management:
- Artificial tears
- Artificial saliva / sugar free gum/pastilles
- Vaginal lubricants
- Hydroxychloroquine to halt disease progression
List some complications of Sjogren’s disease
Eyes:
- Conjuntivitis
- Corneal ulcers
Mouth:
- Dental cavities
- Candida
Vagina:
- Sexual issues/dysfunction
- Candidiasis
Briefly describe the pathophysiology of vasculitis
Pathophysiology:
- Inflammation of the blood vessels (can be arteries or veins)
- Categorised by the size of vessel affected (small, medium or large)
- Leads to damage of blood vessel walls and subsequent thrombus formation, ischaemia, bleeding or aneurysm formation
Briefly describe the pathophysiology of vasculitis
Pathophysiology:
- Autoimmune condition resulting in inflammation of the blood vessels (can be arteries or veins)
- Categorised by the size of vessel affected (small, medium or large)
- Leads to damage of blood vessel walls and subsequent thrombus formation, ischaemia, bleeding or aneurysms
Give 2 examples of each type of vasculitis
- Small vessel vasculitis
- Medium vessel vasculitis
- Large vessel vasculitis
Small vessel vasculitis
- Granulomatosis with polyangiitis (Wegner’s)
- Microscopic polyangiitis
Medium vessel vasculitis
- Kawasaki disease
- Polyarteritis nodosa
Large vessel vasculitis
- Giant cell arteritis
- Takayasu’s arteritis
List some generic features of most types of vasculitis
- Purpura (small purple spots under skin)
- Peripheral neuropathy
- Renal impairment
- HTN
- Anterior uveitis
- Scleritis
- Joint and muscle pain
- GI disturbance
Systemic features
- Fatigue
- Weight loss
- Fever
- Loss of appetite
List investigations for vasculitis including antibodies
Bloods:
- Inflammatory markers (CRP and ESR raised)
Antibodies:
- ANCA (anti-neutrophil cytoplasmic antibodies)
Describe the general medical approach to managing vasculitis
Steroids:
- Tailor administration route to target affected areas
- Oral / IV / nasal / inhaled
Immunosuppressants:
- Cyclophosphamide
- Methotrexate
- Azathioprine
- Rituximab / other MAB
Hypermobility spectrum disorder - state the following:
- Pathophysiology
- Presentation
- Investigation
- Management
Pathophysiology:
- Pain syndrome in people whose joints move beyond normal limits
- Due to laxity of ligaments, tendons and capsules
- Can affect any number of joints
Presentation:
- Pain around joints, worsens after activity
- Abnormal skin
- Marfan qualities e.g. arachnodactylyl
- Hernias and prolapses
Investigations:
- Do a Beighton’s score to assess likelihood
Management:
Aim to reduce pain and reduce disability
- Analgesia (Paracetamol)
- Physiotherapy
- Splinting
- Surgical interventions
Raynaud’s syndrome - state the following:
- Pathophysiology
- Presentation (including classic pattern)
- Management
Pathophysiology:
- Vasospasm of the blood vessels of the digits
- Precipitated by cold or stress
- Common in young women (may be familial)
Presentation:
- Painful cold fingers
Classic pattern
- White (inadequate blood flow)
- Blue (venous stasis)
- Red (re-warming hyperaemia)
Management:
Conservative
- Avoid cold / keep hands warm
- Avoid smoking
Medical
- CCB
- Phosphodiesterase-5 inhibitors
- Prostacyclins
List some diseases associated with Raynaud’s disease
If symptoms develop < 30 years old, suspect underlying disease
- SLE
- Systemic sclerosis
- Sjogren’s syndrome
- Dermatomyositis and polymyositis
PLUS - Cervical rib
- Coagulopathies (sticky blood) e.g. cryoglobulinemia
List a physical cause and a drug that causes Raynaud’s
Physical cause:
- Use of heavy vibrating tools
Drug:
- Beta blockers
List 3 complications of Raynaud’s phenomenon
- Digital ulcers
- Severe ischaemia
- Infection
Behcet’s disease - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management
Pathophysiology:
- Rare multi-organ disease caused by a systemic vasculitis
Presentation:
- Recurrent oral ulcers
- Recurrent genital ulcers
- Uveitis
- Erythema nodosum
Investigations:
- No specific test for Behcet’s
- Usually aim is to rule out other differentials e.g. rheumatoid factor, ANA or ANCA
- Can use Pathergy test for exaggerated skin injury after minor trauma, however is unreliable
- May investigate system related to specific symptoms
Management:
- Immunosuppressive treatment with biologic drugs, corticosteroids, Colchicine, Azathioprine or Methotrexate
- Some need treatment of flares only, some need ongoing treatment
