Speciality: Rheumatology Flashcards
Systemic sclerosis
- Causes
- Presentation
- Ix
- Rx
- UK
- Associated w/ anti-centromere antibodies.
- ANA +’ve
- RF positive - CREST - Calcinosis, Raynaud’s, Oesophageal dysmotility, sclerodactyly, telangiectasia.
- Limited SS = Raynaud’s, scleroderma (face and hands)
- Diffuse SS = scleroderma all over, interstitial lung disease and pulmonary HTN. - Autoantibody testing.
- ANA = all
- Anti-centromere = limited
- Anti-SCL = diffuse
- Nailfold capillaroscopy - Steroid and immunosuppressants in diffuse disease.
- Stop smoking
- Gentle skin stretching
- Avoid cold
- Physio
- MDT
Marfan’s syndrome
- Causes
- Presentation
- Ix
- Rx
- AD inherited
- FBN1 gene on chromo 15
- Protein fibrillin-1 - Tall, thin with increased arm ratio.
- High arch palate
- Pectus excavatum
- Scoliosis
- Dilatation of the aortic sinuses (dissection TAA)
- Pneumothorax - Clinical (Ghent criteria)
- Echo, ECG
- Genetic testing - Nil
- Manage risks
- Regular Echo
- BB and ACEI improves CV mortality etc.
Dermatomyositis
- Causes
- Presentation
- Ix
- Rx
- Autoimmune; RA, SLE
- Related to infection; HIV
- Drugs; Statins and colchicine
- Malignancy; breast, ovarian, lung - Skin = photosensitive.
- Macular rash on the back and shoulders
- Heliotrope rash in the peri-orbital region
- Gottron’s papules on the extensor surfaces of the fingers.
Others = Proximal muscles weakness and tenderness
- Raynauds
- Resp muscle weakness
- ILD
- Dysphagia. - ANA Positive
- Muscle/skin biopsy
- MRI for inflamm
- Look for malignancy - Exercise and physio
- Steroids, immunosuppress and DMARD.
Antiphospholipid syndrome
- Causes
- Presentation
- Ix
- Rx
- Antiphospholipid antibodies.
- Associated w/ SLE, RA, SS, GCA.
- UK pathology - Recurrent venous and arterial thrombosis
- Recurrent miscarriage
- Livedo reticularis
- Thrombocytopenia
- Prolonged APTT (due to lupus anticoagulant autoantibodies)
- Pre-eclampsia. - FBC - thrombocytopenia and haemolytic anaemia
- Clotting - long APTT and normal PT
- CT/MRI/USS - clot hunting.
- Autoantibodies - anticardiolipin and lupus anticoagulant. - General - stop smoking, exercise, health diet and weight, avoid excessive alcohol.
- Thrombosis management - warfarin or other
Osteogenesis imperfecta
- Causes
- Presentation
- Ix
- Rx
- AD inherited
- Abnormal type 1 collagen due to dec synthesis of pro collagen polypeptides. - Presents in kids
- multiple ### in different states of healing
- Atypical ###
- Blue sclera
- Deafness due to otosclerosis
- Dental caries. - In severe forms, prenatal USS for Dx.
- XR
- DEXA
- Genetics - MDT
- Bisphosphonates bind to and stabilise bone by inhibiting osteoclasts.
- Surgery; rods to stabilise etc.
Psoriatic Arthritis
- Causes
- Presentation
- Ix
- Rx
- Psoriasis
- Autoimmune - Arthropathy often precedes skin lesions.
- Predilection for the DIP and fingers.
- Nail changes; yellow, splitting etc.
- Dactylitis
- Arthritis mutilans - telescoping of the digit (pencil in cup deformity)
- Tenosynovitis. - Clinical
- ESR/CRP high
- RF negative
- XR - As for RA.
Ankylosing Spondylitis
- Causes
- Presentation
- Ix
- Rx
- HLA-B27 associated arthropathy.
- Males 20-30.
- Back pain worse in the morning which improves with movement.
- Anterior uveitis
- Stiffness in hips
- Loss of ROM of back
- 5 A’s = Apical fibrosis, anterior uveitis, aortic regurgitation, achilles tendonitis, AV node block, Amyloidosis. - ESR/CRP high
- XR/MRI demonstrating sacroiliitis is required for Dx.
- Modified New York criteria = low back pain for more than 3 months + limited chest expansion + limited ROM + sacroiliitis on XR. - Regular exercise; swimming
- NSAID for pain
- Physio
- - TNF; etanercept and adalimumab if severe
- DMARDS can be used when peripheral joint disease, but not proven to work.
Complications of Rheumatoid arthritis
- Resp = pulmonary fibrosis, pleural effusions, pulmonary nodules, pleurisy.
- Ocular = Dry eyes, episcleritis, scleritis, osteoporosis
- IHD
- Infections
- Depression.
Gout management
1) NSAID if not contraindicated
2) Colchicine (SE = diarrhoea)
3) Joint steroid injection
4) Urate-lowering therapy = allopurinol
Bouchards nodes
- PIPJ
- Hard/bony growths.
- OA
Heberdens nodes
- DIPJ
- Chronic swelling
- Permanent bony growth
- Skews fingertip laterally.
Pseudogout
- Causes
- Presentation
- Ix
- Rx
- Microcrystal synovitis caused by calcium pyrophosphate crystals.
- RF’s = HH, hyperparathyroidism, acromegaly, low phosphate, Wilson’s disease. - Gout Sx
- Knee, wrist and shoulders most commonly affected.
- Joint aspiration; weakly positive birefringent crystals.
- XR - chondrocalcinosis. - NSAID’s or joint injection of steroids.
Tennis elbow (lateral epicondylitis)
- Causes
- Presentation
- Ix
- Rx
- Following activity such as house painting or tennis.
- Worse pain on resisted wrist extension with the elbow extended. - Pain and tenderness over the lateral epicondyle.
- Pain lasts 6mo-2y - Clinical
- CRP, MRI if required. - Avoiding excessive use
- Simple pain relief
- Steroid injection
- Physio.
Sjogren’s syndrome
- Causes
- Presentation
- Ix
- Rx
- Autoimmune affecting exocrine glands.
- Results in dry mucus membranes.
- Associated w/ CTD and RA.
- More common in women.
- Link with developing lymphoma. - Dry eyes
- Dry mouth
- Dry fanny
- Arthralgia
- Raynauds
- Sensory polyneuropathy.
- Parotitis. - RF positive
- ANA positive
- Anti-RO/LA
- Schirmers test for tears. - Artifical tears/saliva/vaginal lubricants.
DEXA scan
- Bone density calculation
- XR of hip/spine
- > -1 = normal
- -1 to -2.5 = mildly reduced
- below -2.5 = osteoporosis.
Osteoporosis management
1) Calcium + D AND alendronate
2) If can’t tolerate –> ridendronate
3) If can’t tolerate –> strontium ranelate and raloxifene.
4) Denosumab
- Most people fail to tolerate due to upper GI Sx such as reflux.
- After Rx for 5 years; treatment should be reassessed w/ an updated FRAX and DEX
- Often has all normal blood tests.
Paget’s disease of the bone
- Causes
- Presentation
- Ix
- Rx
- Increased and uncontrolled bone turnover.
- Osteoclasts disorder increase resorption and increased bone remodelling.
- RF’s - age, male, northern countries, FHx - Older male with bone pain and raised ALP.
- Bone pain in the skull, spine, pelvis, femur.
- Bowing or tibia and bossing of skull.
- Raised ALP in isolation.
- Cranial nerve 8 entrapment - deafness.
- Pathological ###
3) raised ALP
- Calcium, phos and PTH normal.
- XR - osteolysis and excessive bone formation.
- NSAIDS
- Bisphosphonates
- Surgery for deformity
- Monitor for osteosarcoma.
Gout
- Causes
- Presentation
- Ix
- Rx
- Deposition of uric acid crystals in the joint space.
- RF’s = Male, meat, alcohol, diuretics, obesity ,HTN, CKD - Acute pain in a joint which becomes swollen, tender and red.
- Knee
- 1st MTP
- small hand joints
- Ankles.
- Gouty tophi. - Acute 1st MTP inflammation is clinically suggestive.
- Crystals in synovial fluid is diagnostic.
- Serum uric acid (high, but normally normal or low in attack)
- XR for changes; punched-out lesions, sclerosis. - General - elevated and rested
- NSAID, colchicine (main SE is diarrhoea) in acute flare.
- Steroids injections.
- Allopurinol to prevent attack (urate lowering therapy).
Polymyalgia Rheumatica
- Causes
- Presentation
- Ix
- Rx
- Vasculitis w/ giant cells.
- Overlaps w/ GCA
- Muscle arteries affected. - Typical patient >60yo
- Usually rapid onset <1mo
- Aching and morning stiffness in non-wasted proximal muscles.
- Lethargy, arthralgia, depression, fever, anorexia and night sweats. - ESR raised
- Others include, bloods, bone profile, TFT, CK etc.
- Always consider and rule out GCA
- Response to steroids - Prednisolone daily 15mg/od
MTX interactions
- Trimethroprim
- Co-trimoxazole
- High dose aspirin
RA management
1) First line = DMARD + short course of steroids
- Monitor CRP and DAS28 for disease activity.
- Beware of using sulfasalazine in those who are allegic to aspirin.
2) TNF when inadequate response to atleast 2 DMARD’s including MTX.
- Etanercept
- Infliximab
- Adalimumab
- Rituximab
Large vessel vasculitis
- Temporal arteritis
- Takayasu’s
Medium vessel vasculitis
- Polyarteritis nodose
- Kawaski disease
Small vessel Vasculitis
- ANCA associated (wegeners, churg-strauss)
- HSP