Lupus, Gout, Reactive Arthritis & CPPD

Question 1

What is Systemtic Lupus Erthematosus?

Answer 1

An auto-immune disease that is usually characterized by clinical manifestations that are multi-system.

Question 2

What is the pathogenesis of SLE?

Answer 2

• Results from recurrent activation of the immune system
• Production of antibodies and protein products that then contribute to high levels of inflammation and tissue destruction
• Activation of B & T cells (adaptive immune response)

Question 3

What are the risk factors for SLE?

Answer 3

• Genetic: Siblings of someone with SLE are 30% more likely to have the disease.
• Environmental (UV over, Epstein-Barr Virus, Drug induced Lupus)
• Hormonal (High Oestrogen, OCP use, Pregnancy)

Question 4

How does SLE present?

Answer 4

CAN AFFECT ANY ORGAN SYSTEM 
Most common key clinical features:
o	Cutaneous manifestations
o	Malar and/or Discoid rash 
o	Usually photosensitive

Question 5

How is SLE Diagnosed?

Answer 5

Blood testing

• Serological hallmark (98% sensitive)
• Antinuclear antibodies (ANA)
• Double Stranded DNA (dsDNA)
• ENA antibodies
• Raised ESR/CRP

Question 6

How is ‘mild’ Lupus managed?

Answer 6

• Reduce environmental triggers
• UV, hormone therapy, CV risk
• NSAIDs Antimalarials

Question 7

How is ‘severe’ Lupus managed?

Answer 7

• Corticosteroids
• Be aware of osteoporosis risk.
• Immunosuppressive drugs

Question 8

What is reactive arthritis?

Answer 8

“Reactive arthritis (or Reiter’s syndrome) is a form of inflammatory arthritis that develops in response to an infection in another part of the body (cross-reactivity). Coming into contact with bacteria and developing an infection can trigger the disease.

Question 9

What condition does reactive arthritis present similarly to?

Answer 9

Septic Arthritis

Question 10

Reactive arthritis is associated with an acute monoarthitis, what does this mean?

Answer 10

Affecting one joint, usually in the lower limb (knee or talocrural)

Question 11

What two bodily systems usually respond to reactive arthritis?

Answer 11

Seronegative spondyloarthropathy secondary to a bacterial infection in the GI or GU tract

Question 12

Postulated pathogenesis of Reactive Arthritis?

Answer 12

Unclear but the common theories postulate that:

• Immune response to the bacteria in systemic circulation
• T-cell activation, leading to synovitis, monoarticular arthritis
• Cross-reactivity with HLA-B27

Question 13

Common pathogens to trigger reactive arthritis?

Answer 13

Most common: chlamydia, salmonella and shingella

Less common: Ecoli

Question 14

What are some clinical manifestations of reactive arthritis?

Answer 14

• Asymmetrical Oliglioarthritis
• Ocular involvement
• Penile Lesions
• Skin Lesions
• Cardiac Involvement

Question 15

What does “The patient cant see, cant pee, cant climb a tree” refer to for reactive arthritis?

Answer 15

It refers to the clinical manifestations
- Ocular: Uveitis, Conjunctivitis 
- Penile lesions: Urethritis,
Balanitis circinate
- Oliglioarthritis: Larger joints of lower extremity, Dactylitis, Enthesitis (achillies or PF), Bursitis, Sacroiliitis

Question 16

How is reactive arthritis diagnosed?

Answer 16

No specific testing or imaging!

• Hx taking and clinical features ensuring to rule out other causes of arthritis and the presence of predisposing infection
• Blood testing: ESR and CRP to rule out an inflammatory arthritis
• Testing for specific infection

Question 17

What is the suggested treatment and management for reactive arthritis?

Answer 17

• Management of underlying infection with antibiotics
• NSAIDs and paracetamol: NSAID use may be contraindicated in patients with gastric issues
• DMARDs: For late-stage diseases
• General mobility and treatment of arthritis compensations

Question 18

What is GOUT?

Answer 18

A form of arthritis characterised by severe pain, redness and tenderness in joints.

Question 19

What is a Monosodium urate crystal disorder?

Answer 19

An abnormality of uric acid metabolism that results in hyperuricemia and urate crystal deposition
(Inflammatory crystal monoarthropathy)

Question 20

Where do the crystals usually deposit in the body?

Answer 20

• Joints- acute gout arthritis
• Soft tissue- tophi and tenosynovitis
• Urinary tract- urate stones

Question 21

What age group/ sex is most likely to suffer from gout?

Answer 21

Mainly a disorder of men between 40-50

In women onset is usually 60+

Question 22

What nationalities are at an increased risk for gout?

Answer 22

More common in Maori, Pacific Islander and Indigenous Australians

Question 23

What is the enzyme that transforms xanthine into uric acid?

Answer 23

Xanthine oxidase

Question 24

Pathogenesis of gout?

Answer 24

• Lack the ability to degrade uric acid therefore deposited out of the urine
• Hyperuricemia: Urate increases in the kidneys to a point where there is too much in the urine. It then congregates in the blood/ synovial fluid/ soft tissues then crystal precipitate and development of tophi.
• Accumulation of crystals in the soft-tissues then leads to macrophages engulfing them

Question 25

What are the risk factors for gout?

Answer 25

- Genetic factors: 35% increase in males if another male relative has had gout - Natonality: Maori, Pacific Islander and indigenous - Long term renal disease - Long term medication use: Diuretics, Salcylates - Diet: High purine diet (Shellfish, somefish, crab, lobster), Meats (especially bacon, turkey, veal and organ meats), - High alcohol consumption (Beer higher than spirits or wine) - High soft-drink consumption (Especially high fructose corn syrup)

Question 26

What are the four stages of gout?

Answer 26

Stage 1: Asymptomatic hyperuricaemia Stage 2: Acute Gouty Arthritis Stage 3: Intercrtial gout Stage 4: Chronic tophacious gout and chronic gouty arthritis.

Question 27

How does Stage 1 (asymptomatic hyeruricaemia) present?

Answer 27

Usually no clinical manifestations and no treatment necessary other than lifestyle modifications. Elevated serum uric acid: o >0.42 mmol/L in men o >0.36mmol/L in women

Question 28

How does Stage 2 (Acute gouty arthritis) present?

Answer 28

- Acute attack of severe pain - Usually in great toe but can be elsewhere – knees, other toes, ankle - Often in the early hours of the morning and wakes the patient - Skin over the joint is red, shiny, hot and swollen - VERY tender to touch - Sheet overlying is too much- Can’t bear socks - May be precipitated by: o Alcohol or dietary excess o Starvation o Surgical operation - Can subside in 3-10 days

Question 29

How does Stage 3 (Intercritical gout) present?

Answer 29

* Time between attacks. * Low to no pain. * Low-level inflammation causing joint destruction * Time for aggressive management strategies.

Question 30

How does Stage 4 (Chronic tophacious gout and chronic gouty arthritis) present?

Answer 30

- Unlikely to be the first indication of Gout - Stone like deposits of monosodium urate in soft tissue, synovial tissue or in bone near joints (Ears, elbows, big toe, fingers) - Pathognomonic - Uric acid levels remain high over a number of years. - Attacks more frequent and more painful - Permanent joint destruction

Question 31

How is gout Diagnosed?

Answer 31

``` Synovial fluid aspirate: - Uric acid crystals present - Only real diaganostic feature Elevated Serum Uric acid: - Around 30% of all gout sufferers will be in normal limits. Xray: - Punched out joint erosions - Visible tophi and joint effusion ```

Question 32

What are some pharmaceutical management for gout?

Answer 32

NSAIDs - In acute attacks - Contraindicated in kidney disease Corticosteroids - For chronic inflammation Allopurinol- Serious potential adverse reaction (hypersensitivity) - Xanthine oxidase inhibitor - Indicated when frequent attacks - Presence of tophi, kidney stones or uric acid nephropathy - Usually not indicated in kidney disease or elderly - Used as both a prophylaxis and a maintenance

Question 33

What is Calcium Pyrophosphate Deposition Disease (CPPD)?

Answer 33

A disease of crystal deposition in the joint and soft tissue, almost exclusive to articular cartilage.

Question 34

What are some risk factors for CPPD?

Answer 34

* Disease of the elderly – most common in 65+ years * OA * Previous joint trauma * Metabolic disease – diabetes * Family Hx

Question 35

What is the pathophysiology of CPPD?

Answer 35

-Largely unknown how it is exactly caused but what occurs in the joint is an excess of pyrophosphate production in the cartilage

Question 36

What are the clinical features of CPPD?

Answer 36

- Acute attacks that mimic gout - Pain, synovitis or tenderness in one OR multiple joints (Knee, MCP, wrist and hand most common) - Systemic features - Can also mimic RA, OA and tendonitis

Question 37

How is CPPD diagnosed?

Answer 37

- Synovial fluid analysis (differs to gout via rhomboid shaped crystals) - X-ray (shows chondrocalcinosis which reduces joint space and forms new bone around the cartilage making it appear hyper-white)

Question 38

How is CPPD managed?

Answer 38

- Treating acute flare ups and symptoms of an attack - After being diagnosed cortisone is usually injected into the joint - Managed with paracetamol/oral corticosteroids depending on age and co-morbidities

Question 39

How can you distinguish CPPD from GOUT?

Answer 39

- Present with similar symptoms and often indistinguishable in early stages - Joint aspiration for fluid analysis necessary - Treatment is also similar in early stages - In chronic stage gout must be treated with hypouricemic agent to prevent severe deformity.