Rheumatology Flashcards
Systemic sclerosis (CREST / Scleroderma)
Features: Perioral skin thickening, decreased oral aperture, nail fold changes (capillarisation), skin/mucocutaneous telangiectasias, palmar erythema, digital ulcers
Pulmonary involvement - Pulmonary HTN, Interstitial Lung Disease. Less common PE, Pleural effusion, pneumothorax
Ix: CT Chest, ECG (Right heard strain), TTE (increased pulmonary arterial pressure, RV dysfunction), Spirometry (reduced carbon monoxide diffusion capacity)
Rheumatoid Arthritis
Rx:
Conventional Synthetic DMARDs
- methotrexate 10mg weekly orally (Or SC) + weekly 5-10mg folic acid (another day of the week). Methotrexate can take 6-12 weeks for response
Corticosteroid for rapid onset reduction of inflammation
- methylprednisolone 120mg IM OR 5-15mg prednisolone orally.
- lower dose/cease when remission evident
Biological DMARDs/targeted synthetic DMARDs
- tofacitinib can be started by rheumatologist
Ankylosing Spondylitis - features/dx
Features:
- Onset <40 years
- Insidious onset >3 months
- Improves with exercise
- Moderate morning stiffness >45 mins
- Elevated inflammatory markers (elevated in 50-70% patients)
- Good response to NSAIDs
- Lumbar/alternating buttocks pain. Can radiate down thigh.
0.5% of populatiuon, usually men
Elevated risk if FHx of ank spond
Enthesitis (heels) and dactylitis (inflammation of whole finger/toe) and extra-articular features (anterior uveitis = 30% of people with ank spond. Presents as unilateral eye pain, photophobia and increased lacrimation = urgent referral. Conjunctival injection around the rim of the iris is a characteristic finding).
- Less common extra-articular features of ankylosing spondylitis include aortic insufficiency secondary to aortitis, cardiac conduction defects, and apical pulmonary fibrosis.
Can lead to spinal osteoporosis
HLA-B27 not useful in diagnosis, but >90% with ank spond have elevated. 10% normal individuals have elevation
Use Xray + ESR/CRP
- MRI through rheumatologist
- Avoid CT
Xrays = fusion both sacroiliac joints, calcification of intervertebral discs (white line running down spine), dagger spine (calcification of interspinous and supraspinous ligament), bamboo spine
Ankylosing Spondylitis - Rx
controlling symptoms and inflammation normalising physical function enabling participation in social and work-related activities preventing progressive structural damage minimising cardiovascular complications.
Smoking cessation
Exercise
- mobility, cardiovasc function, mental health. Consider PT/EP referral.
NSAIDs
- symptom control. Contraversial re slowing down ankylosis.
DONT USE ORAL CORTICOSTEROIDS
Local corticosteroid injections may be used for peripheral arthritis or enthesitis or for symptom control at SIJ
When treatment with the combination of exercise and an NSAID is inadequate to control symptoms, or disease is severe, disease-modifying therapy is added. The choice of drug depends on the site(s) of disease activity
Rheum may use DMARDs (Ie TNF inhibitors like etanercept or adalimumab)
Myasthenia Gravis
Fluctuating degree of weakness in ocular, bulbar, limb and respiratory muscles
Bimodal 10-30yo (F>M) + 50-70yo (M>F)
Symptoms typically worse at end of the day or after exercise
DOES NOT CAUSE: Sensory loss, reflex changes, sphincter disturbance
Antibodies: Acetylcholine receptor (Ach-R) = most common, or muscle-specific tyrosine kinase (MuSK) = less common
Ix:
- Above Abs + neurophysiological testing (decremental response to repetitve stimulation or positive single-fire electromyelography)
- B12 + TSH (as more likely to develop autoimmune disorders)
- CT chest to check for Thymoma
- Can consider MRI brain if only occular signs and serology negative (?structural abnormality)
Paget’s Disease of Bone
1-2% of population
>55 years
Typically asymptomatic
Isolated ALT rise
Bone pain, arthritis, fractures, compression neuropathy
Most commonly affects pelvis, spine, femur, tibia
Ix: Plain film. Can use radionucleotide bone scan
Mx: Bisphosphonate (Zoledronic acid) for SYMPTOMATIC patients as can increase fracture risk.
