Rheumatology Flashcards
Rheumatoid arthritis
- joints most affected
- differences between RA and OA
- investigations
- Mx
Joints most affected are the DIP, PIP, MCP, wrist, ankle, knee and cerivical spine.
Differences between OA and RA:
* Morning stiffness in OA worsens with movement.
* Wrists are not normally affected in OA.
* PIP and DIP are affected in OA (Heberden’s or Bouchard’s nodes).
Investigations:
- mild elevation of ALP & GGT
- RF +ve in 60-70%
- anti-CCP more specific than RF
- ANA +ve in SLE and 30% of RA
Mx:
NSAIDs + PPI
Corticosteroid in acute flare
DMARDS (methotrexate, leflunomide or sulfsalazine)
Biologics if resistant to methotrexate (adalimumab)
Psoriatic arthritis
- associations
- features
- bloods
- difference with RA
- Mx
Associations - HLA-B27, HLA-Cw6 genes
Sx:
- dactylitis
- joint pain, swelling
- enthesitis
- nail disease
- anterior uveitis
- psoriasis rash
Bloods:
- ESR/CRP elevation
Difference with RA:
- dactylitis
- no anti-CCP
Management
- NSAIDs + PPI
- DMARDS (methotrexate)
- Biologics (etanercept, infliximab, adalimumab) if no standard DMARD response
Systemic sclerosis
- features
- subtypes
- blood test
- management
CREST syndrome - calcinosis, raynauds, oesophageal dysmotility, sclerodactyly, telangiectasia
Subtypes:
1. Limited—Cutaneous fibrosis, most commonly of distal extremities, morphea (fibrotic plaques over face), CREST syndrome symptoms
2. Diffuse—Cutaneous fibrosis more widespread. Raynaud syndrome, telangiectasias, and also organ involvement, including renal (hypertension, MAHA), myocardial fibrosis, pericarditis, and pulmonary fibrosis
Blood tests:
- ANA +ve
- anti scl-70 = most commonly diffuse
- anti-centromere antibodies = most commonly limited
Mx:
- NSAIDs
- Steroids
- Raynauds = CCBs
- Renal Sx = ACEi
- GI Sx = PPI
Polymyositis and dermatomyositis
- Sx
- Bloods
- Gold standard investigation
- Mx
- Association
Sx - weakness of limbs, swollen eyelids with periorbital rash, rashes on hands, gottrons papules
Bloods:
- elevated CK
- Anti-Mi-2 antibodies (dermatomyositis)
- Anti-Jo-1 antibodies (polymyositis)
- Antinuclear antibodies (non-specific for autoimmune)
Gold standard: muscle biopsy for diagnosis of myositis
Mx
1st line: IV/oral corticosteroids
Association:
Of dermatomyositis patients, 25% have an associated malignancy (most common is ovarian cancer).
SLE
- Symptoms
- Investigations
- Management
Symptoms:
* Fatigue, mild fever, weight loss
* Malar butterfly rash
* Photosensitive rash
* Discoid rash
* Arthritis and arthralgia
* Oral ulcer
* Raynaud phenomenon
* Pleuritis, pericarditis
* Alopecia
Investigations:
* FBC—Anaemia, leukopenia, thrombocytopenia (but rarely pancytopenia)
* Elevated CRP/ESR
* Reduced C3 and C4
* Positive ANA and anti-dsDNA
* Urinalysis—Haematuria, casts, proteinuria
Management:
* Lifestyle changes with regard to avoiding sunlight and wearing sunscreen
* NSAIDs, HYDROXYCHLOROQUINE (first-line), glucocorticoids (particularly useful for acute flares)
* If severe or life-threatening, then high-dose IV methylprednisolone
* Biologics for patients unresponsive to standard medical care
* Be sure that the patient is immunised before administering immunosuppressants.
Giant cell arteritis
- Sx
- Investigations
- Management
- Complications
Symptoms:
* Unilateral/bilateral headache (typically over temples)
* Tenderness over temporal arteries (Figure 12.11 shows temporal artery inflammation resulting in a hard cord-like structure)
* Scalp tenderness (sleeping and combing hair can elicit pain)
* Jaw claudication
* Limb claudication
* Vision defects (monocular blindness)
* Absent pulse
Investigations:
* Blood tests—↑ ESR, ↑ CRP, possible normochromic normocytic anaemia, ↑ ALP, and ↑ transaminases
* Temporal artery biopsy (gold standard to confirm diagnosis)—Granulomatous inflammation, multinucleated giant cells
* Vascular ultrasonography of temporal arteries—Oedema and thickening of vessel wall, non-compressible, stenosis, and occlusion
Management:
* If GCA is suspected, glucocorticoids should be given immediately, even if diagnosis is not confirmed yet.
* This will reduce the risk of permanent vision loss and stroke.
Complications:
* Irreversible blindness (most common) due to occlusion of the central retinal artery (a branch of the internal carotid artery)
* Aortic aneurysms
* Large-vessel stenosis
* Myocardial infarction
* Stroke/TIA
Polyarteritis nodosa
- Aetiology
- Symptoms
- Investigations
- Management
Aetiology
* Commonly associated with hepatitis B (HBV)
* Childhood with a peak incidence at around 10 years
* Adults between ages 40 and 50 years are the most commonly affected
Symptoms
* Fever, weight loss, malaise
* Myalgia, arthralgia
* Renal (hypertension, renal failure, nephritic/nephrotic syndrome)
* Cardiac (MI, pericarditis)
* Skin (vasculitis rash, nodules, ulcerations)
* Neurological (mononeuritis multiplex, polyneuropathy)
* Abdominal pain
Investigations
* Hepatitis B surface antigen is positive in 30%.
* p-ANCA test is usually negative.
* Biopsy of small arteries will show evidence of necrotising inflammation.
Management:
Involves immunosuppression with corticosteroids/DMARDs
* In HBV-related PAN, antiviral therapy may be required.
* Severe cases of PAN (e.g., renal failure) may require additional plasma exchange.
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- triad
- investigations
- management
Triad:
1. Necrotising vasculitis of small arteries
2. Upper and lower respiratory tract involvement
3. Pauci-immune rapidly progressive glomerulonephritis (causing the haematuria)
Investigations:
* FBC, ESR & U&Es
* cANCA may be detected in 80%–90%, with 10% being positive for p-ANCA.
* Chest X-ray to look for cavity formation and pulmonary infiltrates
* Sinus CT scan to exclude sinus disease
* Biopsy of vasculitic vessels, upper and lower airways, and kidney
Management:
-*methotrexate in asymptomatic pt
* severe disease—Immunosuppression with cyclophosphamide (and adjunctive prednisolone)
* Rituximab as an alternative to cyclophosphamide
* Steroids are given as adjuvant therapy for 1 year, after which they can be titrated down, provided improvement occurs.
* Patients in remission can be switched from cyclophosphamide to methotrexate or azathioprine.
* Plasma exchange (in severe renal or pulmonary disease)
* Surgical treatment can be offered for correction of nasal deformity.
Fibromyalgia
- associations
- symptoms
- management
Associations:
* Depression and anxiety
* Chronic fatigue syndrome
* Irritable bowel syndrome
* Systemic lupus erythematosus
* Chronic headache syndrome
Symptoms:
* Chronic widespread pain (primarily at sites of tendon attachment to bone)
* Fatigue
* Headache
* Cognitive dysfunction (e.g., memory, concentration)
* Sleep and mood difficulties
* Morning stiffness
* Paresthesia
Management:
* Non-pharmacological—Graded exercise therapy, meditation, acupuncture, education, cognitive behavioural therapy
* Pharmacological—Analgesia, TCAs (amitriptyline), SNRIs (duloxetine), gabapentin, pregabalin
Marfans syndrome:
- inheritance pattern
- what chromosome is affected?
- symptoms
Autosomal dominant
Chromosome 15 which codes for fibrillin-1
Sx:
tall stature
high-arched palate
arachnodactyly
pectus excavatum
pes planus
scoliosis of > 20 degrees
heart:
- aortic aneurysm, aortic dissection, aortic regurgitation
- mitral valve prolapse (75%),
lungs: repeated pneumothoraces
eyes:
- upwards lens dislocation (superotemporal ectopia lentis)
- blue sclera
- myopia
- dural ectasia (ballooning of the dural sac at the lumbosacral level)
Behcets disease triad & association
1) oral ulcers
2) genital ulcers
3) anterior uveitis
Associated with HLA-B27
What test when it is positive suggests the hip pain is referred lumbar spine pain?
positive femoral nerve stretch test
