Rheuma Flashcards
(36 cards)
Sjogren’s Syndrome
- Autoimmune disorder
- dry mucosal surfaces
- may be primary/secondary to RA
Clinical Features:
- Dry eyes: Keratoconjunctivitis sicca
- Arthralgia
- Raynaud’s phenomenon
- Dry mouth
Complications: Inc. risk of lymphoid malignancy
Labs:
(+) Rheumatoid Factor (RF)
(+) ANA
Anti-Ro (SSA); Anti-La (SSB)
Schrimer’s Test (result: decreased tear production)
Rose Bengal stain will document corneal ulcerations
Systemic Lupus Erythematosus (SLE)
- Multisystem, autoimmune disorder
- Women > Men
- Afro-Caribbean origin
- Remitting and relapsing course
Sx:
- Malar rash, discoid rash (Sun-exposed areas), photosensitivity, Raynaud’s phenomenon
- Arthralgia
- Pericarditis
- Pleurisy, Fibrosing alveolitis
- Proteinuria, Glomerulonephritis
-Psychosis, Seizures
Labs:
(+) ANA (most sensitive) - initial screening test
(+) RF
anti-dsDNA (active) - highly specific
ESR - normal (active disease)
Complement levels (C3, C4) - low (active disease)
Gout
- form of inflammatory arthritis, microsynovial synovitis
- caused by chronic hyperuricemia (uric acid > 450 umol/L)
Sx: pain (1st Metatarsophalangeal joint) - usually not always
Radiologic Sx:
- Joint effusion (earliest sign)
- well-defined “punched-out” erosions with sclerotic margins
- soft tissue tophi may be seen
Tx:
- NSAIDs/Colchicine - 1st line
- Oral steroids (Prednisolone 15mgd) if NSAIDs/Colchicine is contraindicated
- Urate-lowering therapy (ULT) should be started at least 2-4 weeks after a gout flare has settled. (Allopurinol - 1st line with colchicine cover. If Allopurinol is not effective use Febuxostat (Xanthine Oxidase Inhibitor)
Exam Tip:
-consumption of excessive amounts of alcohol
-started taking diuretics such as thiazide diuretics/furosemide
Marfan’s Syndrome
- Autosomal Dominant (AD) connective tissue disorder
- FBN1 gene on chromosome 15 w/c codes for Fibrillin-1
S/Sx:
- Tall stature with arm span to height ratio >1.05
- Heart: Dilatation of aortic sinuses (90%) –> Aortic Aneurysm, Aortic Dissection, AR, MVP (75%)
- Eyes: Blue sclera, Myopia
Ankylosing Spondylitis
- HLA-B27 linked with spondyloarthropathy (~90% of pxs)
- males (3:1) aged 20-30
- associated with Peripheral Enthesitis (occurs approximately in a third of patients) : Achilles tendonitis (behind the heel), Plantar fasciitis (heel pad) and the tibial tuberosity.
S/Sx:
- Lower back pain and stiffness of sudden onset. Stiffness is worse in the morning, improves with exercise
P.E: - Schober’s test (line is drawn 10cm above and 5cm below the back dimples). Px bends forward as far as possible distance should increase by >5cm
Diagnostic Test:
- plain Xray of sacroiliac joints
Late changes in Xray:
- Sacroiliitis: subchondral erosions and sclerosis
- Squaring of lumbar vertebrae
- Bamboo spine
CXR: Apical fibrosis
Tx: - Regular exercise, NSAIDs (1st line), anti-TNF therapy (2nd line)
Osteoarthritis
- Commonest joint condition
- > 50 yrs of age (women>men) 3:1; monoarthritis
- hip & knee joints are commonly affected
S/Sx:
- pain on movement and worsening by the end of the day.
- Bouchard’s nodes (PIP) and Heberden’s nodes (DIP) are typically present
X-ray: (Mnemonic - LOSS)
- (Loss of joint space) Decreased joint space
- (Osteophytes) Osteophytes forming at joint margins
- Subchondral cysts
- Subchondral sclerosis
Tx:
- 1st line analgesics: Topical NSAIDs (knee/hand OA)
- 2nd line - Oral NSAIDs/ COX-2 inhibitors, opioids, capsaicin cream & intra-articular corticosteroids
- PPI should be co-prescribed with NSAIDs & COX-2 inhibitors. Caution: should be avoided if px takes ASA
- Joint replacement
- exercise and weight management
Polymyalgia Rheumatica
- > 60 years old
S/Sx:
- typically rapid onset (<1month)
- morning stiffness in proximal limb muscles (but not weakness)
- shoulder, neck, upper arm and pelvic girdle pain (May start asymmetrically but progresses to become bilateral quickly)
- Sx for >= 2 weeks
- dramatically improve with corticosteroids
Diagnostics:
- ESR > 40mm/hr
- CK and EMG - normal
- CRP > 6 mg/mL
Tx: Prednisolone 15mg OD
Lateral Epicondylitis
- Common among people aged 45-55 yrs old
- Usually on the dominant arm
S/Sx:
- pain and tenderness localised to the lateral epicondyle.
- pain is worse on wrist extension against resistance with the elbow extended or supination of the forearm with the elbow extended. (Mills’ test) - straighten arm, palpate lateral epicondyle, fully bend (flex) the wrist, pronate px’s forearm)
Tx:
- Steroid injection, Physiotherapy, Avoid muscle overload, simple analgesia
Rheumatoid Arthritis
- Joint stiffness worse in the morning
- Bilateral symmetrical polyarthropathy
Diagnostics:
- Rheumatoid Factor (RF+) 70-80% of pxs
- Anti-Cyclic Citrullinated Peptide Antibody (highly specific) - more specific and sensitive than RF
- Elevated ESR & CRP
- Anemia of chronic disease
- X-ray: early erosive changes of hands and feet
Tx: cDMARDs (Methotrexate) +/- Prednisolone
> Steroids: Rapid reduction in inflammation & symptoms and useful for managing acute exacerbations.
> NSAIDs: provide symptomatic relief only
> DMARDs - first line and should be started within 3 mos.
Low Disease activity: Pick Hydroxychloroquine
Otherwise: Pick Methotrexate, Leflunomide or Sulfasalazine
Osteoporosis
Risk Factor:
- Glucocorticoid use (Risk increases once px takes an equivalent of prednisolone 7.5mg OD for 3 or more months)
Diagnostics:
DEXA Scan:
T score
> -1.0 = normal
-1.0 to -2.5 = osteopenia
< -2.5 = osteoporosis
Tx: Alendronate (1st line) + Calcium and Vitamin D
Drug-induced Lupus
Most common causes:
- Procainamide
- Hydralazine
Less common:
- Isoniazid
- Minocycline
- Phenytoin
Diagnostics:
+ ANA (100%),
+ Anti-histones antibodies (80-90%)
Paget’s Disease
- Increased but restrained bone turnover
CLINICAL FEATURES: (~5% symptomatic):
- bone pain
- Classical, untreated features (bowing of tibia, bossing of skull)
Diagnostics:
- Increased ALP (Alkaline Phosphatase), Normal Calcium and Phosphate
Tx: Bisphosphonate
Complications:
> Skull thickening
> Deafness
> Bone sarcoma
> Fractures
Raynaud’s
Clinical Features:
- symmetrical, young women
Tx:
CCB (i.e. Nifedipine) - inhibits platelet activation and increases Ca2+ influx to the heart which relaxes blood vessels and increases supply of blood and oxygen to the heart while reducing its workload.
Bisphosphonates
- lowers demineralisation in bone
-inhibits osteoclasts by reducing recruitment and promoting apoptosis
Uses:
- prevention and treatment of osteoporosis
- hypercalcemia
- Paget’s disease
- pain from bone metastases
A/E: Oesophageal ulcers
Reactive Arthritis
- Typically seen in young adults
- HLA-B27-associated seronegative spondyloarthropathies
Clinical Features:
- Asymmetrical, predominantly lower extremity, oligoarthritis is the major presenting symptom (usually ankles & knees)
- Reiter’s syndrome: Urethritis, Conjunctivitis (50%) and Arthritis (Mnemonic: Can’t see, pee or climb a tree)
- develops within 2-4 weeks after an infection (sexually acquired/ GI in origin)
+ Skin manifestations: Rash| Circinate balanitis (painless vesicles on the coronal margin of the prepuce)|Keratoderma blenorrhagica (waxy yellow/brown papule on the palms and soles)| Erythema nodosum (tender, red nodules over shins)
Tx:
> Analgesia, NSAIDs, intra-articular steroids
> Sulfasalazine and Methotrexate (for persistent disease)
Systemic Sclerosis
- Hardened, sclerotic skin and other connective tissues
- more common in women
- Limited Cutaneous Systemic Sclerosis/ Limited Scleroderma
> Raynaud’s phenomenon may be the 1st sign
> Slow onset and progression
> Affects the face and distal limbs
> Associated with anti-centromere antibodies
> CREST syndrome (Calcinosis, Raynaud’s phenomenon, (O)Esophageal dysmotility, Sclerodactyly, Telangiectasia)
> (+) ANA - Diffuse Cutaneous Systemic Sclerosis
> affects the trunk and proximal limbs
> more severe (involvement of the internal organs such as the GI, heart, lungs or kidneys)
> rapid and fast progression
> Associated with scl-70 antibodies
> (+) ANA (90%), (+) RF (30%)
Antiphospholipid Syndrome
Clinical Features:
> Predisposition to both venous and arterial thromboses
> Recurrent miscarriages
> Thrombocytopenia
> Increase in APTT (due to ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade)
> (+) Anti-Cardiolipin antibody
Tx:
Initial: Warfarin (Target INR: 2-3 for 6 mos)
Maintenance:
> Repeated venous and thromboembolic events (Warfarin INR: 3-4)
> Arterial thrombosis (INR: 2-3)
Pregnant: ASA 75mg and heparin (lower further risk of miscarriages)
ANCA
> cANCA - Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- has correlation with disease activity
> pANCA - Churg-Strauss syndrome + others
- cannot be used to monitor disease activity
Other causes of (+) ANCA (typically pANCA)
> IBD (UC>Crohn’s)
> Connective Tissue Disorders: RA, SLE, Sjogren’s)
Osteomalacia
- decreased mineral content
- Rickets if growing; Osteomalacia if epiphysis fusion has occurred.
Diagnostics:
- Vitamin D (low)
- Calcium and Phosphate (low)
- Alkaline Phosphatase (high)
Tx: Calcium and Vitamin D tablets
Methotrexate
INDICATIONS:
> RA
> Psoriasis
> Acute Lymphoblastic Leukemia
A/E:
> Pulmonary fibrosis
> Liver cirrhosis
> Myelosupression
- Women - avoid pregnancy for at least 6 mos after Tx
- Men - Use effective contraception for at least 6 mos after Tx
Rx: taken weekly (starting dose: 7.5mg weekly) + Folic acid 5mg once weekly (>24H after Methotrexate dose), FBC, U&E and LFTs need to be regularly monitored
!!! Avoid prescribing Trimethoprim/Cotrimoxazole concurrently - Increases risk of marrow aplasia
Dermatomyositis
> an inflammatory disorder
idiopathic or also be associated with connective tissue disorders
polymyositis (if skin manifestations are not prominent)
CLINICAL FEATURES;
> symmetrical, proximal muscle weakness (+/- tenderness) and characteristic skin lesions
> photosensitivity
> macular rash over the back and shoulder (shawl sign)
> Heliotrope rash in the periorbital region
> Gottton’s papules - roughened red papules over extensor surfaces of fingers
> Raynaud’s
> Respiratory muscle weakness
DIAGNOSTICS:
(+) ANA
> Creatine Kinase (increased)
> LDH (increased)
> EMG
> Muscle biopsy
Tx: Steroids and sun-blocking agents
Temporal Arteritis
- large vessel vasculitis overlaps with polymyalgia rheumatica
FEATURES:
> 60 yrs old
- Rapid onset (<1 month)
- Headache (85%) - temporal
- Jaw claudication (65%)
- visual disturbances secondary to anterior ischemic optic neuropathy
- tender and palpable temporal artery
- polymyalgia rheumatica (50%) (aching, morning stiffness in proximal limb muscles)
DIAGNOSTICS:
- ESR (>50mm/hr) or <30 (10%) *initial diagnostic
- CRP (Elevated)
- Temporal artery biopsy (skip lesions) *definitive diagnostic
- Creatine kinase and EMG (normal)
Tx:
- high dose prednisolone (60mg OD) +/- low dose Aspirin (75mg OD)
- urgent ophthalmology review
Pseudogout
- form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dehydrate in the synovium
Risk Factors:
> Haemochromatosis
> Acromegaly
> Hyperparathyroidism
> Hypothyroidism
FEATURES:
> knee, wrist and shoulders are most affected
> Joint aspiration: weakly-positive birefringent rhomboid-shaped crystals
> X-ray: chondrocalcinosis
DIAGNOSTICS:
> Joint aspiration (to exclude septic arthritis)
Tx:
> NSAIDs/ intra-articular, intra-muscular/oral steroids
Behcet’s Syndrome
- Complex multisystem disorder linked with presumed autoimmune-mediated inflammation of the arteries and veins
Epidemiology:
> Eastern Mediterranean
> Men (more common), Young adults (20-40 y/o)
> Associated with HLA B5 and MICA6 allele
CLINICAL FEATURES
* to diagnose: oral ulcers + 2 of the ff:
> Classic triad of symptoms (Oral ulcers, Genital ulcers and Eye lesions - anterior/posterior uveitis, retinal vasculitis)
> Skin lesions (Erythema nodosum, pseudofolliculitis, papulopustular lesions/acneiform nodules in post-adolescent pxs not on corticosteroids)
> Pathergy (puncture site ff needle prick becomes inflamed with small pustule forming which is read by physician at 24-48H)
Others:
- Thrombophlebitis, Arthritis, Nx involvement, GI (Abd pain, diarrhoea, colitis), DVT
Tx: Topical corticosteroids
