Rheumatology Flashcards
Radiological features of OA
Loss of joint space
Osteophytes
Subchondral cysts
Subchondral sclerosis
Radiological features of RA
Loss of joint space Soft tissue swelling Peri-articular osteopenia Deformity Subluxation
Radiological features of Gout
Normal joint space
Soft tissue swelling
Periarticular erosions
Red flags for back pain
Age <20 or >55yrs Neurological disturbance (inc. sciatica) Sphincter disturbance/ urinary retention Saddle anaesthesia Bilateral or alternating leg pain/ Leg claudication Current or recent infection Fever, wt. loss, night sweats History of malignancy Thoracic back pain Morning stiffness Acute onset in elderly people Constant or progressive pain Nocturnal pain Abdo mass Immunosupressed
Causes of back pain
Mechanical Strain/idiopathic Trauma Pregnancy Disc prolapse Spondylolisthesis (forward shift of one vertebra)
Degenerative: spondylosis, vertebral collapse, stenosis
Inflammatory: Ank spond, Paget’s
Neoplasm: Mets, myeloma
Infection: TB, abscess
Describe what a disc prolapse is and the presenting features
Herniation of nucleus pulposus through annulus fibrosus
- often L4/5 or L5/S1
- severe pain on sneezing, coughing or twisting a few days after low back strain
Lumbago: low back pain
Sciatica: shooting radicular pain down buttock and thigh
Signs of disc prolapse
Limited spinal flexion and extension Free lateral flexion Pain on straight-leg raise Lateral herniation → radiculopathy Central herniation → corda equina syndrome
L4/5 → L5 Root Compression Weak hallux extension ± foot drop weak inversion (tib. post.) helps distinguish from peroneal N. palsy. ↓ sensation on inner dorsum of foot
L5/S1 → S1 Root Compression Weak foot plantarflexion and eversion Loss of ankle-jerk Calf pain ↓ sensation over sole of foot and back of calf
Ix and Rx of Disc Prolapse
Ix: MRI (emergency if cauda equina)
Rx
Brief rest, analgesia and mobilisation effective in ≥90%
+/- steroid injection
Surgical: discectomy or laminectomy (microscopic-resection of nucleus pulposus) may be needed in cauda-equina syndrome, continuing pain or muscle
weakness.
Presenting features of spondylolisthesis
Displacement of one lumbar vertebra on another
Usually forward
Usually L5 on S1
May be palpable
Presentation
Onset of pain usually in adolescence or early adulthood
Worse on standing
± sciatica, hamstring tightness, abnormal gait
Causes of spondylolisthesis
Congenital malformation
Spondylosis
Osteoarthritis
Dx and Rx of spondylolisthesis
Dx
Plain radiography
Rx
Corset
Nerve release
Spinal fusion
Features and presentation of Spinal Stenosis
Developmental predisposition ± facet joint OA
→ generalized narrowing of lumbar spinal canal.
Presentation
Spinal claudication
Aching or heavy buttock and lower limb pain on walking
Rapid onset
+/- paraesthesiae/numbness
Pain eased by leaning forward (e.g. on bike)
Pain on spine extension
Ix and Rx 0f Spinal Stenosis
Ix –> MRI
Rx Corsets NSAIDs Epidural steroid injection Canal decompression surgery
How does presentation of nerve root lesions differ from L2-S1
L2 - weak Hip flexion + adduction
L3 - weak Knee extension +
Hip adduction
–> ↓ Knee Jerk
L4 - weak Foot inversion + dorsiflexion + Knee extension
–> ↓ Knee Jerk
L5 - weak - Great toe dorsiflexion + Foot inversion + dorsiflexion + Knee Flexion
Hip extension + abduction
S1- weak - Foot eversion +
Foot and toe plantarflexion + Knee flexion
–> ↓ Ankle Jerk
Ix for back pain
IF RED FLAGS
FBC, ESR, CRP , ALP , se electrophoresis, PSA
MRI
General Mx of back pain
Conservative
Max 2d bed rest
Education: keep active, how to lift / stoop
Physiotherapy
Psychosocial issues re. chronic pain and disability
Warmth
Medical
Analgesia: paracetamol ± NSAIDs ± codeine
Muscle relaxant: low-dose diazepam (short-term)
Facet joint injections
Acute cord compression presentation
Bilateral pain: back and radicular
LMN signs at compression level
UMN signs and sensory level below compression
Sphincter disturbance
Acute Cauda Equina Compression presentation
Alternating or bilateral radicular pain in the legs
Saddle anaesthesia
Loss of anal tone
Bladder ± bowel incontinence
Ix Mx of acute cord/ cauda equina compression
- bldder scan, MRI,
Large prolapse: laminectomy / discectomy
Tumours: radiotherapy and steroids (if hx cancer) - and stabilise spine
Abscesses: decompression
Causes of acute cord/ cauda equina compression
- bony met
- large disc protrusion
- myeloma
- cord/ paraspinal tumpour
- TB
- Abscess
Yellow flags for back pain
Attitudes Beliefs Compensation Diagnosis Emotions Family hx
Work
UMN v LMN lesions
UMN
- hypertonia
- hyperreflexia
- clonus
- +ve babinski
- clasp - knife relflex
- +/- weakness
LMN =
- Hypotonia
- hyporeflexia
- wasting
- fasciculations
- weakness
Define OA
Degenerative joint disorder in which there is
progressive loss of hyaline cartilage and new bone
formation at the joint surface and its margin.
Risk factors for OA
Age (80% > 75yrs) Obesity Joint abnormality Smoking Vit D def Hormone status
Classification of OA
Primary: no underlying cause
Secondary: obesity, joint abnormality (Trauma, AVN, RA, Gout, Pagets, Perthes, SUFE), acromegaly, haemophilia
Symptoms of OA
Affects: knees, hips, DIPs, PIPs, thumb CMC
Pain: worse ̄c movement, background rest/night pain,
worse @ end of day.
Stiffness: especially after rest, lasts ~30min (e.g. AM)
+/.- crepitus
Deformity and instability
↓ ROM
Locking (loos intra-articular bodies)
+/- mild synovitis
Signs and Ix in OA
Bouchard’s (prox), Heberden’s (dist.) nodes
Thumb CMC squaring
Fixed flexion deformity
- CRP may be mildly elevated, rest normal
- MRI - synovial thickening, effusions, oedema, periarticular lesions
Pathophysiology of OA
Softening of articular cartilage → fraying and fissuring
of smooth surface → underlying bone exposure.
Subchondral bone becomes sclerotic c¯ cysts.
Proliferation and ossification of cartilage in unstressed
areas → osteophytes.
Capsular fibrosis → stiff joints.
Differentials of OA
Septic Crystal Trauma
Features in the hx in OA
Pain severity, night pain Walking distance Analgesic requirements ADLs and social circumstances Co-morbidities Underlying causes: trauma, infection, congenital
Mx of OA
CONSERVATIVE
↓wt.
Alter activities: ↑ rest, ↓ sport
Physio: muscle strengthening & joint mobility
Walking aids, supportive footwear, home mods - ↓ load
Education
Hot/cold packs
MEDICAL
1) Paracetamol +/- Topical NSAIDs
2) Codiene
3) NSAIDS +/- PPI protection
Joint injection: local anaesthetic and steroids
Capsaicin
SURGICAL Arthroscopic washout: esp. knee - Trim cartilage, remove foreign bodies. Arthroplasty Osteotomy: small area of bone cut out. Arthrodesis
Symptoms of Septic Arthritis
Acutely inflamed tender, swollen joint
↓ROM
Systemically unwell
Causative organisms of septic arthritis
Staph aureus
N. Gonococcus
Streps
Gm-ve bacilli
Risk factors for getting septic arthritis
Joint disease (e.g. RA) Chronic renal fialure Immunosuppression (e.g. DM) Prosthetic joints IVDU
Investigations for septic arthritis
URGENT Joint aspiration - MCS --> ↑↑ WCC mostly PMN Baseline obs -/↑ESR or CRP , ↑WCC, Blood cultures X-ray (usually normal at presentation - later bone destruction
Management of septic arthritis
IV Abx for 2 weeks - flucloxacillin then oral 4 wks Consider joint washout under GA Splint joint Physiotherapy after infection resolved NO STEROIDS
Complications of septic arthritis
Osteomyelitis
Arthritis
Ankylosis: fusion
Differentials of septic arthritis
Crystal arthropathy
Reactive arthritis
Trauma
Cellulitis
Features and presentation of RA
ANTI CCP Or RF
Arthritis
- Symmetrical, polyarthritis of MCPs, PIPs of hands and feet → pain, swelling, deformity
1. Swan neck (PIPJ hyperextension) 2. Boutonniere (PIPJ prolapse)
3. Z-thumb 4. Ulnar deviation of the fingers 5. Dorsal subluxation of ulnar styloid
Morning stiffness >1h
Improves with exercise
Nodules - elbows also fingers, feet, heal
Firm, non-tender, mobile or fixed Lungs
Tenosynovitis
De Quervain’s Tenosynovitis
Atlanto-axial subluxation
Immune - AIHA; Vasculitis; Amyloid; Lymphadenopathy
Cardiac: pericarditis + pericardial effusion
Carpal Tunnel Syndrome
Pulmonary
Fibrosing alveolitis (lower zones) Pleural effusions (exudates)
Ophthalmic
Epi-/scleritis
2O Sjogren’s Syndrome
Raynaud’s
Felty’s Syndrome
RA + splenomegaly + neutropenia
Diagnosis of RA
American college of rheumatlogy
A) Joint involvement
- swelling & tenderness +/- imaging
(1) 2-10 med large (2) 1-3 small
(3) 4-10 small (5) >10 small
B) Serology
(2) low +ve Rf/Anti-CCP
(3) High +Ve RF/ Anto-CCP
C) Duration of synovitis
(1) > 6 weeks
D) Acute phase reactants
(1) Abnormal CSR/ ESR
Ix for RA
Bloods: FBC (anaemia of CD, ↓PMN, ↑plat), ↑ESR, ↑CRP RF +ve in 70% (false +ve in HCV, SLE, Sarcoid, Sjogrens)
High titre assoc. ̄c severe disease, erosions and extra-articular disease
Anti-CCP: 98% specific (Ag derived from collagen)
ANA: +ve in 30%
Radiography, US, MRI
- thickened synovium and widespread synovitis
Mx of RA
Conservative Refer to rheumatologist Regular exercise PT OT: aids, splints annual flu jab Medical DAS28: Monitor disease activity - no swollen joints, global health and inflammatory markers DMARDs and biologicals: use early Steroids: IM, PO or intra-articular for exacerbations/ until DMARD kick in NSAIDs: symptom relief
Mx CV risk: RA accelerates atherosclerosis
Prevent osteoporosis and gastric ulcers
Surgical
Ulna stylectomy
Joint prosthesis
Athroscopy/ plasty
Differentials of RA
- reactive
- SLE/ CT disease
- Seronegative
- Polyarticualr gout
- PMR
If just in hands - Psoriatic
DMARDs used in RA
Ideally, treatment should be started within 3 months of the onset of symptoms.
1) Methotrexate + Sulfasalazine/ Hydroxycholoquine
2) If tried 2 DMARDs and ↑ DAS28 on 2 occasions 1 m apart can start Infliximab
Action and SE of Hydroxycholoquine
Reduces inflammation by inhibiting stimulation of the TLR (cellular receptors for microbial products) that induce inflammatory responses through activation of the innate immune
retinopathy, seizures
- pre-treatment and annual eye-screen
Action SE, monitoring and patient advice of methotrexate
Inhibits the enzyme dihydrofolate reductase, essential for the synthesis of purines and pyrimidines.
+ folic acid
SE - Bone marrow suppression, hepatotoxic, pulmonary fibrosis, alopecia, headaches, GI distubrance
Monitoring - FBC, U&E, LFT,
- 1–2 wks until stabilised, then every 2–3 months
- BE wary of signs of myelosuppression infection/ unexpected bruising/ anaemia
Stop 3m before trying to get pregnant
SE of sulphasalazine
hepatotoxic, SJS, ↓ sperm count, nausea, headache/ dizzy, oral ulcers, blood disturbances/ myelosuppresion
Action, SE and pre-treatment requirementsof infliximab
Anti-TNFα ↓ inflammation – ↓ angiogenesis ↓ joint destruction SE ↑ risk of new malignancy if have prior malignancy. ↑ risk of skin/soft tissue infection Chronic leg ulcers; recurrent chest infections; indwelling catheter Reactivation of TB & HBV Worsens HF
Before start - CXR (TB), Bloods for HCV/ HIV/HBV
Pathophysiology of Gout
Deposition of monosodium urate crystals in and around joints → erosive arthritis
May be ppted by surgery, infection, fasting or diuretic
Presentation of gout
M>F=5:1
severe joint inflammation
- great toe MTP = Podagra
- ankle, foot, hand, wrist, elbow, knee
- Pain, hot, tender, swollen, shiny red skin
- develop rapidly (hr) and last 3-10 days
Urate deposits in pinna and tendons = Tophi
Renal disease: radiolucent stones and interstitial
nephritis
Differentials of gout
Septic arthritis
Pseudogout
Haemarthrosis
Causes of gout
Hereditary
Reduced excretion
- diuretics, NSAIDs, renal impairement
Excess production
cytotoxics, purine rich foods (beef, pork), alcohol
↑ cell turnover: lymphoma, leukaemia, psoriasis,
haemolysis, tumour lysis syndrome
Ix for gout
Polarised light microscopy
- Negatively birefringent needle-shaped crystals
-/↑ serum urate
X-ray changes occur late
- Punched-out erosions in juxta-articular bone & ↓ joint space
Acute Rx of gout
Rest and elevate joint
NSAIDS + Colchicine
CFR - steroids
Dont stop allopurinol
CI and SE NSAIDS
CI - warfarin, PUD, HF, CRF
SE - diarrhoea
Prevention of Gout
Conservative
Lose wt.
Avoid prolonged fasts and EtOH excess
Allopurinol
- dont start until 3wk after attack (+NSAID/ Colchicine cover)
Febuxostat (XO inhibitor) if hypersensitivity
Action and SE of Allopurinol
Xanthine Oxidase Inhibitors:
- use to prevent attacks, prevent damage
- if > 1 attacks in 12m or if on cytotoxics/ renal stones
SE: rash, fever, ↓WCC ( ̄c azathioprine)
Acute v Chronic CPPD
ACUTE - Presents as acute monoarthropathy Usually knee, wrist or hip
Usually spontaneous and self-limiting
CHRONIC
Destructive changes like OA
Can present as poly-arthritis (pseudo-rheumatoid)
Risk factors for CPPD
↑age OA DM Hypothyroidism Hyperparathyroidism Hereditary haemochromatosis Wilson’s disease
Ix and Rx for CPPD
Ix
ASPIRATION
Polarized light microscopy
- +ve birefringent rhomboid-shaped crystals
X-ray may show chondrocalcinosis - Soft-tissue Ca deposition
Rx
Cool pack, rest,
Analgesia
NSAIDs +/- colchicine
May try steroids: PO, IM or intra-articular
Common features of seronegative spondyloarthropathies and the types
Axial arthritis and sacroiliitis
Asymmetrical large-joint oligoarthritis or monoarthritis
Enthesitis
Dactylitis
Extra-articular: iritis, psoriaform rashes, oral ulcers, AR, IBD
HLA-B27 allele
RF -ve
PEAR P = Psoriatic E = Enteric A = Ankylosing spondylitis R = Reactive
Presentation of Ankylosing Spondylitis
Gradual onset back pain
Typically man <30
Radiates from SI joints to hips and buttocks
Worse @ night ̄c morning stiffness
Relieved by exercise.
Progressive loss of all spinal movements
- Schober’s test <5cm
- ↓ thoracic expansion
Some develop thoracic kyphosis and neck hyperextension and spinocranial ankyloses = question mark posture
Enthesitis: Achilles tendonitis, plantar fasciitis
Costochondritis
Extraskeletal manifestations of ankylosing spondylitis
A’s
o Apical fibrosis (pulmonary)– can lead to resp failure
o AR
o Aortitis
o Ant uveitis/ acute iritis -
o AV block
o Achilles tendonitis and plantar fasciitis (enthesisitis)
o Amyloidosis
o Anterior mechanical chest pain (costochondritis – fatigue & SOB)
o Arthritis (peripheral) - Pain in non-axial joints
Osteoporosis~ 60%(↑ risk of osteoporotic spinal fractures) – stripping of end-plates
IBD; Cardiomyopathy; IHD; Psoriasis; dactylitis;
General Malaise/ Fatigue; weight loss
Ix of Ank spond
XRAY (or MRI/USS ealry) Sacroliliitis: irregularities, sclerosis, erosions Vertebra: corner erosions, squaring syndesmophytes (bony proliferations) Bamboo spine: calcification of ligaments, periosteal bone formation Blood --> FBC (anaemia), ↑ESR, ↑CRP , HLA-B27 DEXA scan and CXR
Indications for referral for ank spond
- low back pain that started <45 y/o and lasted > 3m with:
- 4 + or 3+ & +ve HLA-B27
Additional criteria are: - low back pain that started < 35 y/o
- waking during the second half of the night because of symptoms
- buttock pain
- improvement with movement
- improvement within 48 hours of taking NSAIDs
- a first-degree relative with spondyloarthritis
- current or past arthritis
- current or past enthesitis
- current or past psoriasis.
Mx of ank spond
1) Physio/ exercise - maintain posture and stability and NSAIDs (symptom relief)
2) Anti-TNF/ Local steroid injections/ Bisphosphonates
Surgical
3) Hip replacement to ↓ pain and ↑ mobility
Psoriatic Arthritis presentation
- Asymmetrical mono/oligoarthropathy – often DIPJ
- Nail changes – pitting, onkolysis
- Skin changes - Acneiform rashes; palmo-plantar pustulosis (rheumatoid nodules absent), psoriasis plaques (scalp, natal cleft and umbilicus)
- Dactylitis and Enthesitis (Achilles tendonitis and plantar fasciitis)
- Hyperuricaemia
- Symmetrical polyarthritis (like RA - Joint stiffness, pain, swelling)
- Arthritis mutilans - resorption of bones and the consequent collapse of soft tissue –> telescoping (shortening)
- ¼ develop AI & vertebral changes like those in AS
Ix of Psoriasitic arthritis
X-Ray
Erosion → “pencil-in-cup” deformity
- no pero-articular ostoepenia
Rx of Psoriasis arthritis
Conrtrol skin disorder education, physio, OT NSAIDs Sulfasalazine, methotrexate, ciclosporin (if at serveral sites/ ↑CRP. erosions) Anti-TNF
CASPAR score - current/hx psoriasis, nail changes
Reactive arthritis presentation
1-4wks after urethritis (chlamydia) or dysentery
(campy, salmonella, shigella)
Asymmetrical lower limb oligoarthritis: esp. knee
Iritis, conjunctivitis
Keratoderma blenorrhagica: plaques on soles/palms
Circinate balanitis: painless serpiginous penile ulceration Enthesitis
Mouth ulcers
reiters –> arthritis, urethritis and conjuncitivitis
Ix for reactive arthritis
↑ESR, ↑CRP
Stool culture if diarrhoea
Urine chlamydia PCR and sexual health review
culture cervix/ vagina
Mx of reactive arthritis
NSAIDs and local steroids (skin/eyes) Physiotherapy to maintain ROM Splint Monitor FBC, FT Relapse may need sulfasalazine or methotrexate
Presentation and Rx of enteropathic arthritis
Asymmetrical large joint oligoarthritis mainly affecting the lower limbs.
Seronegative Spondyloarthropathies
Sacroiliitis may occur
Rx
Treat the IBD
NSAIDs or articular steroids for arthritis
Colectomy → remission in UC
Presentation of Behcet’s disease
Recurrent oral and genital ulceration nt/post uveitis Erythema nodosum Vasculitis non-erosive large joint oligoarthropathy CN palsies diarrhoea, colitis
Sjogren’s pathophysiology and presentation
Due to lymphocytic infiltration of exocrine gland
primary or secondary (RA/SLE)
↓ tear production → dry eyes - keratoconjunctivitis sicca
↓ salivation → xerostomia
Bilateral parotid swelling
Vaginal dryness → dyspareunia
Polyarthritis; Raynaud’s
Bibasal pulmonary fibrosis Vasculitis; Myositis
Ix adn Rx of Sjogren’s
Schirmer tear test
Abs:ANA–RoandLa,RF
Hypergammaglobulinaemia
Parotid biopsy
Rx
Artificial tears
Saliva replacement solutions
NSAIDs and hydroxychloroquine for arthralgia
Immunosuppression for severe systemic disease
Pathology of Raynaud’s
Peripheral digital ischaemia precipitated by cold or emotion.
Primary or Secondary (SLE, Syst Sclerosis, RA, Sjogrens, β-b
Presentation of Raynaud’s
Digit pain + triphasic colour change: WBC - White (Vasoconstriciton), - Blue (Ischaemia) - Crimson (Reperfusion) Digital ulceration and gangrene
Rx of Raynaud’s
Wear gloves CCBs: nifedipine ACEi IV prostacyclin Advice and education - avoid cold,
Features of limited systemic sclerosis
Calcinosis Raynaud’s Esophageal and gut dysmotility → GOR Sclerodactyly Telangiectasia
Skin involvement limited to face, hands and feet
Beak nose
Microstomia
Pulmonary HTN in 15%
- fatigue, arthralgia
Features of diffuse systemic sclerosis
Organ fibrosis
GI: GOR, aspiration, dysphagia, anal incontinence
Lung: fibrosis and Pul HTN
Cardiac: arrhythmias and conduction defects
Renal: acute hypertensive crisis
Ix for systemic sclerosis
Bloods: FBC (anaemia), U+E (renal impairment)
Abs
Centromere: limited
Scl70 / topoisomerase: diffuse
Urine: stix, PCR
Imaging
- CXR: cardiomegaly, bibasal fibrosis
- Hands: calcinosis
ECG + Echo: evidence of pulmonary HTN
Annual, ECHO and Spirometry
Mx of systemic sclerosis
Advise on red flags of renal crisis and internal obstruction
Exercise and skin lubricants: ↓ contractures
Raynaud’s Mx
Medical
EARLY - anti-histamine and low dose corticosteroid
Immunosuppression - IV cyclophosphamide
Raynaud’s Mx
Renal: BP control – ACEi
Oesophageal: PPIs, prokinetics (metoclopramide)
PHT: sildenafil, bosentan
Complications of systemic sclerosis
Renal crisis - obliterative vasculopathy and luminal narrowing of renal arcuate arteries. Progressive reduction in renal blood flow. Aggravated by vasospasm, leads to juxtaglomerular hyperplasia and increased renin secretion –> malignnat HTN
Malignancy - lung, breast, ovarian, lympoma
Sjogrens
Depression
CTS
Features of Polymyositis
Progressive symmetrical proximal muscle weakness.
Wasting of shoulder and pelvic girdle
Dysphagia, dysphonia, respiratory weakness
Assoc. myalgia and arthralgia
Commoner in females
Often a paraneoplastic phenomenon - Lung, pancreas, ovarian, bowel
Presentation of Dermatomyositis
= myositis + skin signs
Heliotrope rash on eyelids ± oedema
Macular rash (shawl sign +ve: over back and shoulders) Nailfold erythema
Gottron’s papules: knuckles, elbows, knees
Mechanics hands: painful, rough skin cracking of finger
tips
Retinopathy: haemorrhages and cotton wool spots
Subcutaneous calcifications
Extra-muscular features of Polymyositis and Dermatomyositis
Fever Arthritis Bibasal pulmonary fibrosis Raynaud’s phenomenon Myocardial involvement: myocarditis, arrhythmias
Ix and Rx for Polymyositis and Dermatomyositis
Muscle enzymes: ↑CK, ↑AST, ↑ALT, ↑LDH Abs: Anti-Jo1 EMG Muscle biopsy Screen for malignancy - Tumour markers; CXR; Mammogram; Pelvic/abdo US; CT
Rx - Immunosupression - steroids/ Azathioprine/ methotrexate
Differentials of Polymyositis and Dermatomyositis
Inclusion body myositis Muscular dystrophy Polymyalgia rheumatica Endocrine / metabolic myopathy Drugs: steroids, statins, colchicine, fibrates
Features of SLE
AutoAbs to a variety of autoantigens result in the formation and deposition of immune complexes.
Relapsing, remitting history
Constitutional symptoms: fatigue, wt. loss, fever, myalgia
Diagnosis of Lupus
A RASH POINts MD
Arthritis - Non-erosive, involving peripheral joints
Renal - proteinuria and ↑BP
ANA (+ve in 95%)
Serositis - Pleuritis/ Pericarditis
Haematological - AIHA; ↓WCC; ↓Plats; ↓C3&C4
Photosensitivity and non-scarring alopecia
Oral ulcers (and nasal)
Immune phenomenon
- Anti-dsDNA (VERY SPECIFIC); Anti-Sm
Neurological - Seizures, psychosis
Malar Rash - Facial erythema sparing the nasolabial folds
Discoid Rash - Erythema → pigmented hyperkeratotic papules → atrophic depressed lesions
- Mainly affects face and chest
Monitoring disease activity in SLE
Anti-dsDNA titres Complement: ↓C3, ↓C4 ↑ESR Bloods: FBC, U+E, CRP, clotting (usually normal) Urine: stix, PCR CT/MRI if other systems involved
Causes and Ab in drug-induced lupus
procainamide, phenytoin, hydralazine, isoniazid
Anti-histone Abs
Features of anti-phospholipid syndrome
CLOTs: venous (e.g. DVT) and arterial (e.g. stroke)
Coagulation defect: ↑APTT
Livido reticularis
Obstetric complications: recurrent 1st trimester abortion Thrombocyotpenia
Classification and Ab’s in anti-phospholipid syndrome
Primary: 70%
Secondary to SLE: 30%
Pathology
Anti-phospholipid Abs: anti-cardiolipin and lupus anticoagulant
Rx of anti-phospholipid syndrome
Low-dose aspirin
Warfarin if recurrent thromboses: INR 3.5
Mx of SLE
Acute flare
- High-dose prednisolone + IV cyclophosphamide
Moderate - mycophenolate/ DMARD
Cutaneous Symptoms
- topical steroids and sun cream
Maintenance - skin & joint
NSAIDs and hydroxychloroquine/ ± low-dose steroids
- or azathioprine/ rituximab
Lupus Nephritis
Proteinuria: ACEi
Aggressive GN: immunosuppression
May need renal replacement therapy
Complications of SLE
↑ risk of osteoporosis and CV disease
Features of Giant Cell Arteritis
Granulomatous inflammation Increased CRP/ESR Age >50 New headache Temporal artery and scalp tenderness
Claudication of jaw
Amaurosis fugax
Prominent temporal arteries ± pulsation
Systemic signs: fever, malaise, fatigue, weight loss
Mx and Ix of giant cell arteritis
Do ESR and start pred 60mg/d PO
↑↑ESR and CRP
↑ALP
↓Hb (normo normo), ↑Plats
Visual symptoms - IV methylpredisolone 100mg 3d
Temporal artery biopsy w/i 2wk: but skip lesion occurrs
CK and EMG normal
- Taper steroids gradually, guided by symptoms and ESR
PPI and alendronate cover (~2yr course usually)
Presentation of polymyalgia rheumatica
>50yrs old
Severe pain and stiffness in shoulders, neck and hips
- Sudden / subacute onset
- Symmetrical
- Worse in the morning: stops pt. getting out of bed
No weakness (cf. myopathy or myositis)
± mild polyarthritis, tenosynovitis and CTS
Systemic signs: fatigue, fever, wt. loss
15% develop GCA
Ix and Rx of PMR
Ix
↑↑ESR and CRP (+ ↑plasma viscosity)
↑ALP
Normal CK
Rx
Pred 15mg/d PO: taper according to symptoms and ESR
PPI and alendronate cover (~2yr course usually)
Features and rx of Takayasu’s arteritis
Large vessel vasculitis fever, fatigue, wt. loss Weak pulses in upper extremities Visual disturbance HTN ↑↑ESR/CRP
- Prednisolone
Features, symptoms and Rx of polyarteritis nodosa
Medium vessel vasculitis
Assoc. HBV
Rash
Renal → HTN
GIT → melaena and abdo pain
Rx
Pred + cyclophosphamide
What is Kawasaki’s disease
Childhood PAN variant
Features 5-day fever Bilat non-purulent conjunctivitis Oral mucositis Cervical lymphadenopathy Polymorphic rash (esp. trunk) Extremity changes (erythema + desquamation) Coronary artery aneurysms
Rx –> IVIg + aspirin
Pathology and Features of Granulomatosis with polyangiitis
Necrotizing granulomatous inflammation and small vessel vasculitis with a predilection for URT, LRT and Kidneys
Features URT Chronic sinusitis Epistaxis Saddle-nose deformity Nasal obstruction LRT Cough Haemoptysis Pleuritis Renal RPGN Haematuria and proteinuria Other Palpable purpura Ocular: conjunctivitis, keratitis, uveitis peripheral neuropathy
Ix and Rx for Granulomatosis with polyangiitis
cANCA +ve
Dipstick: haematuria and proteinuria
CXR: bilat nodular and cavity infiltrates
↑↑ESR/CRP
CT chest - diffuse alveolar haemorrhage
Rx –> corticosteroids and cyclophosphamide.
Maintenance - azathioprine
Features of Churg-Strauss
Small vessel vasculitidies Late-onset asthma Eosinophilia Granulomatous small-vessel vasculitis - RPGN - Palpable purpura - GIT bleeding pANCA +ve
Features of Microscopic polyangitis
RPGN
Haemoptysis
Palpable purpura
pANCA
- no granulomas
- give steroids and methotrexate
Name some ANCA -ve small vessel vasculitidies
HSP (IgA variatn - post URTI, purpura on buttocks, colicky abdo pain, arthragia, haematuria)
Goodpastures
Anti-GBM Abs; RPGN; Haemoptysis
CXR: Bilat lower zone infiltrates (haemorrhage)
- Immunosuppresion + plasmapheresis
Risk factors for developing fibromyalgia
Risk Factors Neurosis: depression, anxiety, stress Dissatisfaction at work Overprotective family or lack of support Middle age Low income Divorced Low educational status
Chronic fatigue syndrome; IBS;
Features of fibromyalgia
Chronic (>3) , widespread musculoskeletal pain (L,R and above and below waist) and tenderness (at 9 sites) Morning stiffness Fatigue Poor concentration Sleep disturbance Low mood headache
Mx of fibromyalgia
- rule out organic cause
Educate pt and impact questionnaire CBT and relaxation techniques Graded exercise programs Amitriptyline or pregabalin Paracetamol
MOA and SE of Azathioprine
Metabolised to Mercaptopurine
- Check levels of TPMT enzyme before use.
Onset of action takes at least 6 weeks.
S.E – colitis & increase risk infection in pts also receiving corticosteroids; hypersensitivity reaction; hypotension; interstitial nephritis; liver impairment; malaise; malagia; n&v&d; neutropenia; rash; rigors; thrombocytopenia
FBC weekly for first 4 weeks and tell to look out for signs of BM supression
