Rheumatology Flashcards
Clinical Features of Osteoarthritis
Typically affects women aged >55yrs (3:1 F:M ratio)
- Typically bilateral affecting one joint at a time. DIP and CMC affected more than PIP
- Episodic joint pain & stiffness - provoked by movement + relieved by rest
- Herbedens nodes - at DIP
- Bouchard’s nodes - PIP
- Squaring of the base of the thumb
X ray changes; (LOSS)
* Loss of joint space
* Osteophytes (boney spurs)
* Subarticular sclerosis
* Subchondral cysts
Clinical Features of Rheumatoid Arthritis
Inflammatory arthritis - autoimmune condition.
Genetic associations = HLA DR4 & HLA DR1
Antibodies = **RF and Anti-CCP **(more sensitive)
Presentation;
* Symmetrical distal polyarthropathy (mainly hands & feet)
* Morning stiffness + pain improves on activity
* Gradually worsens with large joints getting involved
* May have systemic Sx = fatigue, weight loss, flu-like Sx
* Rheumatoid nodules - typically on the elbow
* Hand signs = Z shaped thumb deformity, Swan-neck deformity, Boutonnieres + ulnar deviation of the fingers.
O/E - Positive Squeeze test + palpation of synovium gives a **boggy **feeling due to inflammation.
Diagnosis + Management of Rheumatoid arthritis
Diagnostic criteria; Score >6 is diagnostic
* Joints that are involved (1 per joint)
* Serology +ve
* Inflammatory markers raised
* Duration of symptoms (> 6 weeks)
Do RF first line and then check Anti-CCP if its +ve.
Xray changes;
* Joint destruction/deformity
* Soft tissue swelling
* Pertiarticular osteopenia
* Boney erosions (= worse prognosis)
Joint aspiration = high WBC & Yellow turbid fluid.
Management;
1. DMARDs 1st line (e.g methotrexate, leflunomide or Sulfalazine)
2. Add in Anti-TNF or Rituximab.
How to manage an acute flare of RA
IM steroids (e.g methylpred) - as DMARDs take up to 2-3months to be effective
Poor prognostic factors in Rheumatoid arthritis
Younger onset
Male
More joints affected
Presence of RF or Anti-CCP
Boney erosions on xray
Extra articular manifestations in Rheumatoid arthritis
- Caplan’s syndrome - Pulmonary fibrosis due to pulmonary nodules
- Felty’s syndrome = RA + Neutropenia + Splenomegaly
- Sicca’s syndrome = Sjogren’s symptoms due to RA
- Episcleritis + Scleritis
- Lymphadenopathy
- Carpel tunnel
- Amyloidosis
NOTE - another complication is Atlantoaxial subluxation - this occurs in the C spine and can resultin spinal cord compression. (especially important if a patient is going for surgery due to head positioning - they should have anterior and lateral cervical spine radiographs prior to surgery).
What is palindromic rheumatism
Short episodes of inflammatory arthritis with joint pain, stiffness + swelling affecting a few joints. Typically only lasts 1-2 days then completely resolves.
Psoriatic arthritis
An inflammatory arthritis associated with psoriasis.
It is a **seronegative spondylarthropathy (HLA-B27) **
Typically occurs 10 years after skin changes and is present in 20% of psoriasis patients.
Features;
* Mostly a symmetrical polyarthritis (V similar to RA) but 20% are asymmetrical oligoarthritis affecting hands & feet (this is the main differentiator from RA)
* Sacroilitis
* Telescoping fingers
Other signs = Psoriatic skin lesions, Tenosynovitis & Enthesitis (e.g achilles tendinopathy) + dactylitis. May have psoriatic nail change.s
Xray changes;
* Shows periarticular erosions + new bone formation
* Periositis = periosteum inflammation (thickened bone outline)
* Osteolysis + Ankylosis
* **Pencil in cup appearance. **
Management;
* Basically the same as RA however for mild disease NSAIDs are enough (DMARDs not needed unless severe)
* Monocloncal antibodies e.g Ustekinumab can be trialled after Anti-TNF therapy
Reactive arthritis
Can’t see, can’t pee can’t climb a tree
A spondylarthropathy - HLAB27
Commonly triggered by Chlamydia or Gastroenteritis.
Features;
* Bilateral conjunctivitis
* Anterior uveitis
* Circinate balantitis (dermatitis of the penis)
* Arthritis - typically a hot swollen knee
Management;
1. Rule out septic arthritis - aspirate & culture the joint
2. Rule out gout - send for crystal examination
NSAIDs + steroid injections can be of use. Will resolve in 6 months and unlikely to recur. If recurrent start DMARDs.
Enteric arthritis
Part of the seronegative spondylarthropathies - HLAB27
10-15% of patients will have IBD (mainly UC) & Remission of disease usually remits joint disease (may persist in chrons)
Can also be secondary to intestinal bypass, bacterial overgrowth syndrome, coeliac etc
Features;
* Typically asymmetrical and worse in lower limb
* Sacroiliits or spondylitis common
* Joint pain may precede the bowel disease
Gout Fluid aspirate + Xray findings
Fluid aspirate shows;
* No bacteria.
* Monosodium urate crystals
* **Needle-shaped crystals with negative bifringement of polarised light. **
Xray shows;
* Joint space is maintained!!!
* Lytic lesions in bone + Punched out erosions with sclerotic borders.
Causes of gout
DART
Diuretics - particularly thiazides
Alcohol
Renal disease
Trauma
Management of gout
NSAIDs or Colchicine are 1st line
Steroids 2nd line
Prophylaxis = Allopurinol (do not start this until the acute attack has settled) + lifestyle changes
Measure serum urate 2 weeks after inflammation settles
Pseudogout Features
Caused by calcium pyrophoshate crystals.
Aspiration shows **rhomboid shaped crystals with +ve birefringement. **
Xray shows chondrocalcinosis - thin white line in middle of joint. May have OA features also.
Types of seronegative spondylarthropathies + features
Psoriatic arthritis
Reactive arthritis
Enteric arthritis / IBD
Ankylosing spondylitis
Features;
* Enthesopathy - e.g achilles tendinopathy / plantar fasciitis
* Pulmonary fibrosis
* Sacroiliitis
* Uveitis
* Amyloidosis
* Aortic regurg
Ankylosing spondylitis
Inflammation / fusion of Vertebral column + Sacroiliac joints.
Presentation;
* Typically young male aged 20ish yrs with Low back pain, stiffness + sacroiliac pain in buttocks.
* Develops gradually.
* Pain worse at rest + improves with activity.
* Morning stiffness
O/E
- Reduced lateral flexion
- Schober’s test: *reduced forward flexion, distance between points does not increase >5cm *
- Reduced chest expansion
Xray;
- Bamboo spine (squaring of the vertebral bodies)
- subchondral sclerosis + erosions
- syndesmophytes: areas of bone growth where the ligaments insert into bone
CRP/ESR will be high. HLABS27 gene testing. Pelvic radiograph useful to see sacro-ileitis.
Management = 1st line is phsyiotherapy for 4 weeks. NSAIDs. Steroids may be useful in flairs. Commence Anti-TNF if failure to respond to 2 NSAIDs
Still’s disease
Bimodal age distribution - 15-25yrs and 35-46yrs.
Presentation;
* Salmon-pink maculopapular rash
* Fever - typically highest in the evening
* Athralgia
This condition may mimic sepsis as patient can be severely unwell.
**Raised Ferritin **- is a clue for this condition + progression marker
Management = NSAIDs, steroids or Anti-TNF.
SLE
Autoimmune connective tissue disease (T3 hypersensitivity = immune deposition in organs)
MarkerS = ANA & Anti-dsDNA, Also Anti-smith + Antiphospholipid antibodies (syndrome is a complication of SLE)
Presentation;
* More commin in young-middle aged females & Asians
* Systemic = Fatigue + weight loss + fever
* MSK = Arthralgia + Myalgia
* Derm = Photosensitive bufferly rash on cheeks
* Lymphadenopathy / Splenomegaly
* Raynaud’s
* SOB + Chest pain
* Mouth ulcers + Hair loss
Management;
1. NSAIDs & Steroids/Hydroxychloroquine & Suncream 1st line
2. other immune modulators e.g methotrexate, tacrolimus, ciclosporin etc can be used
3. For severe disease can try rituximab or belimumab
Complications of SLE
- Lupus nephritis - severe complication which can lead to ESRF. Has a ‘wire-loop’ appearance to the glomeruli. Treat with glucocorticoids + Mycophenolate
- CVD
- Infection
- Pericarditis - the most common cardiac manifestation
- Interstitial lung disease
- Antiphospholipid syndrome
- Neuropsychiatric SLE
DLE
Discoid lupus erythematous
RF = more common in women + young adults + Smokers + people with darker skin
It is associated with an increased risk of SLE and SCC.
Presentation;
* Discoid crusty lesions on face, ears + scalp which are photosensitive
* Scarring alopecia
Management = Confirm with skin biopsy. Sun protection. Topical steroids. Hydroxychloroquine.
Limited cutaneous systemic sclerosis
CREST Syndrome
Markers =** Anti-Centromere antibodies
Features;
* Calcinosis
* Raynaud’s
* Esophageal dysmotility
* Sclerodactyly - restricted ROM in joints of hands
* Scleroderma - face + distal limbs mainly
* Telangiectasia
Diffuse cutaneous systemic sclerosis
Markers =** Anti-Scl70 antibodies **
Features;
* CREST syndrome +
* Scleroderma - mainly affecting trunk + proximal limbs
* CVD problems
* Resp = Pulmonary HTN + Fibrosis
* Glomerulonephritis + Scleroderma renal crisis
Management of systemic sclerosis
Steroids + Immunosupressants.
Management of complications;
* Nifedipine - for Raynaud’s
* PPIs for GI involvement
* ACEi for hypertension
* Lifestyle changes
Polymyalgia rheumatica
Inflammatory condition causing pain in shoulders and pelvic girdle mainly. Strongly associated with giant cell arteritis (temporal arteritis).
Presentation;
* Typically rapid onset of Sx in pt >60yrs
* Bilateral shoulder pain + Pelvic girdle pain with morning stiffness
* Pain worse on movement + inteferes with sleep
Diagnosis = Inflammatory markers may be raised.
Note that CK and EMG will be normal (CK raised in myositis). Consider checking autoantibodies for SLE and RA and maybe check bence-jones proteins for myeloma.
Management;
* 15g oral Prednisolone - reassess after 1 week. If no response then unlikely to be PR as a good response should occur
* 70% improvement in Sx after 4 weeks is diagnostic
Which drugs can induce SLE
Procainamide
Hydralazine
isoniazid
Temporal arteritis
Medium/large vessel vasculitis - strong link with polymyalgia rheumatica.
Sx;
* Severe unilateral headache around temples
* Scalp tenderness
* Jaw claudication + pain on chewing
* Blurred vision - due to ischemia to the anterior optic nerve = opthamological emergency!!!1
Diagnosis;
- Raised ESR/CRP
- Temporal artery biopsy may show multinculeated giant cells, *however occurs in skip lesions so a normal biopsy does not rule this out *
- Duplex USS of temporal artery = **hypoechoic halo sign **
Management;
1. High dose prednisolone
2. IV Methylpred- immediately if vision loss is present to prevent permanent loss + opthamology same day review!!!
2. Aspirin
3. PPIs
Complications = Vision loss + Cerebrovascular accident (stroke) + Aortitis (leading to aortic aneurysm)
Polymyositis
= inflammatory disorder causing symmetrical proximal muscle weakness. Typically affects middle aged females
Cause = Idiopathc / Associated with connective tissue disorders / Malignancy (Lung, breast, ovarian + gastric)
Features;
* Proximal muscle weakness +/- Pain (typically bilateral) -* typically affects shoulder + pelvic girlde *
* May also have respiratory muscle weakness, pulmonary fibrosis / interstitial lung disease
* Dysphagia / Dysphonia
Dx;
* Elevated CK
* LDH, AST, ALT may also be raised
* EMG + Muscle biopsy may be required for diagnosis
* Anti-synthetase antibodies e.g Anti-Jo-1
Management;
1. Corticosteroids 1st line
2. Consider Azathioprine as steroid-sparing agent
Dermatomyositis
Subtype of polymyositis characterised by skin involvement.
Features;
* Gottron lesions - scaly erythematous patches on knuckles, elbows and knees
* Photosensitive rash on back, shoulders + neck
* **purple rash on face + eyelids **
* Perioribtal oedema
Diagnosis;
* Same as polymyositis e.g raised CK
* **Anti-Jo-1 antibodies & Anti-Mi-2 antibodies + ANA **
NOTE - Should consider doing CT chest/abdo/pelvis to look for malignancy as commonly occurs secondary to malignancy
Antiphospholipid syndrome
Acquired disorder characterised by a predisposition to VTE, Miscarriage + thrombocytopenia. Commonly occurs secondary to SLE.
Features;
* VTE
* Miscarriage
* Thrombocytopenia
* Prolonged APTT
Management;
* If primary = low-dose aspirin
* If Secondary;
a. Initial VTE = Warfarin lifelong(INR 2-3)
b. Recurrent VTE = Warfarin lifelong (INR 3-4)
Sjogren’s syndrome
Autoimmune disorder of exocrine glands - resulting in dry mucosal surfaces.* Often is secondary to RA or other connective tissue disorders *
Features;
* Keratoconjunctivitis sicca (dry eyes)
* Dry mouth
* Vaginal dryness
* Arthralgia + Myalgia
* Raynaud’s
* Recurrent parotitis
* Renal tubular acidosis
Diagnosis;
* ANA +ve in 90%
* Anti-Ro (70%) & Anti-La (30%)
* **Schimer’s test +ve **= To measure tear formation
* Histology = Focal lymphocytic infiltration
* C4 low
* Hypergammaglobulinemia
Management = All about symptomatic control e.g Artificial saliva + tears. Pilocarpine may stimulate saliva production. Oestrogen cream for vaginal dryness.
*NOTE - increased risk of lymphoid malignancy (MALT lymphoma)
p-ANCA vasculitis’
Microscopic polyangitis + Churg-Strauss* (Eosinophilic granulomatosis with polyangitis)*
c-ANCA vasculitis
Wegeners - Granulomatosis with polyangitis
Features;
* Saddle nose defomirty
* Recurrent epistaxis
* Sinusitis
* Glomerulonephritis
Features of Churg-Strauss syndrome
- Features of eosinophilia: Asthma + blood eosinophilia
- Paranasal sinusitis
- Mononeuritis multiplex
- SOB
Microscopic polyangitis features
- Renal failure
- SOB
- Haemoptysis
Types of immune mediated small vessel vasculitis
HSP
Goodpasture’s
Cyroglobulinemic vasculitis
Medium vessel vasculitis’
- Polyarteris nodosa = mottled purple lace rash. *Associated with hep B/C & HIV. Causes renal impairment, strokes + MI. *
- **Kawasaki disease **= High fever, bilateral conjunctivitis, strawberry tongue + desquamation. Give aspirin
Large vessel vasculitis’
- Giant cell arteritis (temporal arteritis)
- Takayasu’s arteritis - mainly affects the aorta + its branches. Aneurysms + “pulseless disease” common. Typically presents <40yrs wth arm claudication or syncope.
Behcet’s syndrome
Autoimmune condition of arteries + veins. Typically affects young males (20-40yrs) more common in mediterean & turkey.
Associated with** HLA-B51 **
Features; The classic triad is;
1. Mouth ulcers - *sharply circumscribed eroision with red halo *
2. Genital ulcers - usually 2 opposing surfaces
3. Anterior uveitis
Others = Erythema nodosum / Morning stiffness / GI inflammation / Aseptic meningitis or memory impairment.
Diagnosis = mainly clinical. Can do Pathergy test.
Management = Topical steroids/anaesthetic for ulcers. Systemtic oral steroids. Colchicine. Azathioprine can be useful.
Osteomalacia
A condition of defective bone mineralisation causing soft bones secondary to low vit D levels.
*(remember vit D is activated in the kidneys so CKD is a common cause of low Vit D. Vit D also needed to absorb calcium + phosphate in intestines so low Vit D = low calcium and phosphate levels too which then = defective bone metabolism)
Features;
* Fatigue
* Bone pain & muscle aches & proximal muscle weakness
* Pathological fractures
Diagnosis;
* Low Serum 25-hydroxyvitamin D
* Low calcium
* Low phosphate
* High ALP + PTH (as a compensatory mechanism)
Xray shows osteopenia (more radiolucent bones) and DEXA confirms low bone mineral density.
Management;
1. Colecalciferon (Vit D supplements) for life.
Paget’s disease of the bone
Disorder of bone turnover secondary to excessive/uncoordinated osteoblast + osteoclast activity resulting in areas of High density (sclerosis) and low density (lysis)
Features; Typically older adults with;
* Bone pain
* Bone deformities: Bowing of tibia + frontal bossing of skull
* Pathological fractures
* Hearing loss - *if ear bones affected *
Xray shows;
* Bone enlargement / deformity + Osteolysis
* Cotton-wool appearance = lysis + sclerosis
* V shaped defects in long bones (e.g candle flame or blade of grass sign)
Bone scintrigraphy shows increase patchy focal uptake.
Isolated rise in ALP is diagnostic! - *Vit D, calcium + phosphate will be normal *
Management;
1. Bisphosphonates (e.g oral risodronate)
Sarcoidosis
Multisystem disorder characterised by non-caseating granulmomas
RF = young adults / Afro-carribean descent
Features;
* Erythema nodosum
* Bilateral hilar lymphadenopathy
* Swinging fever
* Polyarthraglia
* Lupus pernio
*** Hypercalcemia **
Associated syndromes;
1. Lofgren’s = Acute form of the disease. Good prognosis
2. Heerfordt’s = Uveoparotid fever, characterised by parotid enlargement + fever + uveitis - secondary to Sarcoidosis.
Diagnosis;
* Hypercalcemia & Raised ECR. CXR shows upper zone fibrosis and bilateral hilar lymphadenopaty. Tissue biopsy confirms non caseating granulomas.
Management = Steroids
Indications for steroids in sarcoidosis + poor prognostic factors
Steroids if CXR changes, Hypercalcemia or eye/heart/neuro involvement
Poor prognostic factors;
* Insidious onset, Symptoms for >6 months
* Absence of erythema nodosum
* Extrapulmonary manifestations e.g lupus pernio or splenomegaly
* CXR findings
* BAME people
Marfan’s syndrome
Autosomal dominant connective tissue disorder. Caused by a defective FBN1 gene (chromosone 15)
Features;
* Tall stature - *Arm span: height >1.05 *
* High arched palate
* Arachnodactyly
* Pectus excavatum
* Pes lanus
* Scoliosis > 20 degrees
* Dural ectasia
* Aortic root/sinus dilation + Mitral valve prolapse
Management = optimise complications risks. Control BP and HR (B-blockers & ACEi) + Genetic counselling
Monitoring = yearly echo’s and opthamology review.
Complications of marfan’s syndrome
- Aortic regurgitation, dissection + Aneurysm (due to root dilation)
- Repeated pneumothoraxes
- Upwards lens dislocation
- Blue sclera
Ehler’s danlos
Autosomal dominant connective tissue disorder of **Type 3 collagen. ** Resulting in hypermobility.
Features;
* Elastic, fragile skin
* Joint hypermobility = recurrent dislocations
* Easy bruising
* Aortic regug + mitral valve prolapse + aortic dissection
* Subarachnoid haemorrhage
Management;
* Beighton score - to determine extent of hypermobility
* Manage complications
* Physiotherapy + OT.
What can be used to monitor flares of SLE
Complement levels - these will be low
What medication commonly causes osteonecrosis of the jaw
Bisphosphonates
Which antibodies are present in drug induced lupus
Anti-histone antibodies
Which medication should be avoided in raynauds
Beta blockers
