Rheuma Flashcards

1
Q

Sjogren’s Syndrome

A
  • 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

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2
Q

Systemic Lupus Erythematosus (SLE)

A
  • 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)

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3
Q

Gout

A
  • 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

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4
Q

Marfan’s Syndrome

A
  • 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

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5
Q

Ankylosing Spondylitis

A
  • 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)

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6
Q

Osteoarthritis

A
  • 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

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7
Q

Polymyalgia Rheumatica

A
  • > 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

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8
Q

Lateral Epicondylitis

A
  • 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

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9
Q

Rheumatoid Arthritis

A
  • 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

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10
Q

Osteoporosis

A

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

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11
Q

Drug-induced Lupus

A

Most common causes:
- Procainamide
- Hydralazine

Less common:
- Isoniazid
- Minocycline
- Phenytoin

Diagnostics:
+ ANA (100%),
+ Anti-histones antibodies (80-90%)

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12
Q

Paget’s Disease

A
  • 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

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13
Q

Raynaud’s

A

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.

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14
Q

Bisphosphonates

A
  • 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

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15
Q

Reactive Arthritis

A
  • 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)

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16
Q

Systemic Sclerosis

A
  • 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%)
17
Q

Antiphospholipid Syndrome

A

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)

18
Q

ANCA

A

> 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)

19
Q

Osteomalacia

A
  • 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

20
Q

Methotrexate

A

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

21
Q

Dermatomyositis

A

> 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

22
Q

Temporal Arteritis

A
  • 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

23
Q

Pseudogout

A
  • 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

24
Q

Behcet’s Syndrome

A
  • 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

25
Q

Hydroxychloroquine

A
  • used to manage RA, SLE/Discoid Lupus Erythematosus

A/E:
> Bull’s eye retinopathy (May result in severe and permanent visual loss) - common

Monitor: Visual Acuity annually

26
Q

Psoriatic Arthropathy

A
  • precedes the development of skin lesions

TYPES:
> Rheumatoid-like polyarthritis (most common type)
> Asymmetrical oligoarthritis: usually affects hands and feet (~20-30%)
> Sacroiliitis
> DIP joint disease
> Arthritis mutilans (severe deformity of fingers/hand, ‘telescoping fingers’)

Clinical features:
- nail changes + skin changes + arthritis

Xray:
- erosions in the center of the R distal interphalangeal joints (pencil-in cup appearance)- asymmetrical oligoarthritis

Tx: same as RA

27
Q

Osteoarthritis

A

> Pain exacerbated by exercise and relieved by rest
Decrease in internal rotation is often the 1st sign

28
Q

Inflammatory Arthritis

A

> Pain in the morning
Systemic features
Raised inflammatory markers

29
Q

Greater Trochanteric Pain Syndrome (Trochanteric Bursitis)

A

> Due to repeated movement of the fibroelastic iliotibial band
Pain and tenderness over the lateral side of the thigh
Most common in women aged 50-70 yrs

30
Q

Meralgia Paraesthetica

A

> Caused by compression of a lateral cutaneous nerve of the thigh
Usually burning sensation over the anterolateral aspect of the thigh

31
Q

Avascular Necrosis

A

> Sx may be gradual or sudden onset
May follow high-dose steroid therapy or previous hip fracture of dislocation

32
Q

Leflunomide

A

> Disease Modifying Anti-Rheumatic Drug (DMARD) mostly used in the management of RA
very long half-life

CONTRAINDICATIONS:
> Pregnancy (contraception during Tx for at least 2 yrs post Tx in women, at least after 3 mos in men)

A/E:
> Peripheral neuropathy
> Myelosuppressjon
> GI (Diarrhea)
> HPN
> Wt loss/Anorexia
> Pneumonitis

Monitoring:
> FBC/ LFT and BP

Stopping:
- very long wash-out period of up to a year, so it requires co-administration of Cholestyramine

33
Q

Granulomatosis with polyangitis (GPA)

A
  • formerly known as Wegener’s Granulomatosis
  • idiopathic small to medium vessel vasculitis
  • appears around middle age

S/Sx:
> Upper Respiratory Tract (Nosebleeds, Crust around nostrils, Chronic sinusitis, Saddle-nose deformity, Bloody rhinorrhea)

> Lungs (Haemoptysis, Cough)

> Kidneys (Haematuria)

Dx: (+) cANCA (antineutrophil cytoplasmic antibodies)

34
Q

Churg-Strauss Syndrome

A
  • rare diffuse vasculitis disease affecting the coronary, pulmonary, cerebral, abdominal visceral and skin circulations
  • affects small- and medium-sized arteries and veins associated with asthma.
  • Cardinal manifestations: Asthma, Eosinophilia and lung involvement.
  • Six criteria for the diagnosis of Churg-Strauss Syndrome: (presence of 4/6 = high specificity & sensitivity)
    1. Asthma
    2. Eosinophilia of > 10% in peripheral blood
    3. Paranasal sinusitis
    4. Pulmonary infiltrates (may be transient)
    5. Histological confirmation of vasculitis with extra vascular eosinophils
    6. Mononeuritis multiplex or Polyneuropathy

Dx/Labs:
> (+) p-ANCA (perinuclear staining positive antimyeloperoxidase antibodies)
> Eosinophilia and Anemia on FBC
> Elevated ESR & CRP
> Increased serum IgE levels
> CXR: pulmonary opacities, transient pulmonary infiltrates, and bilateral multifocal consolidation
> CT: ground-glass attenuation
> Biopsy: small necrotising granulomas, as well as necrotising vasculitis (found esp in the lung)

35
Q

Polyarteritis Nodosa (PAN)

A
  • rare disease, characterized by necrotizing vasculitis of the medium-sized arteries. It can lead to cutaneous ulcers, kidney infarction and GI haemorrhage.
  • affects any organ but spares the pulmonary and glomerular arteries
  • some cases of PAN are linked with Hepatitis B, pxs should be screened for viral hepatitis

Tx: corticosteroid therapy

36
Q

Mikulicz’s Syndrome & Disease

A
  • benign persistent swelling of lacrimal and parotid (or submandibular) glands due to lymphocytic infiltration.
  • No specific cause = Mikulicz’s disease
  • Secondary cause = Mikulicz’s sydrome

Sx:
> Symmetrical enlargement of all salivary glands
> Narrowing of the palpebral fissures due to enlargement of the lacrimal glands
> Dryness of the mouth