Rheumatoid Arthritis and Connective Tissue Diseases

Question 1

Define Rheumatoid Arthritis

Answer 1

Inflammatory AI joint disease with synovial swelling

Question 2

What is the epidemiology and aetiology of R. Arthritis?

Answer 2

E: 2/3x ^ in women, 1% of population
A: Auto-antibodies lead to a defective cell-mediated immune response

Question 3

What is the pathophysiology of RA?

Answer 3

1) Chronic Inflammation occurs where B%T cells and neutrophils infiltrate
2) Proliferation: Pannus formation occurs
3) Pro-Inflammatory Cytokines –> Proteinases occur –> Destruction of cartilage

Question 4

What are the S/S of RA?

Answer 4

Signs: Symmetrical, deforming, polyarthropathy, erosion on XR, 80% RF positive
Symptoms: Pain eases with usage, swelling, early morning stiffness, general fatigue and extra-articular involvement

Question 5

How does RA extra-articular involvement affect different organs?

Answer 5

STissues: Muscle wasting, bursitis and nodules
Eyes: Dry eyes, scleritis and episcleritis
Neurology: Peripheral neuropathy, entrapment neuropathy and cervical spine instability
Haematology: Palpable lymph nodes, splenomegaly and anaemia
Pulmonary: pleural effusion
Heart: Pericardial rub and pericardial effusion
Kidneys: Amyloidosis

Question 6

Describe the RF

Answer 6

Antibody works against FC portion of IgG

Question 7

What investigations and treatment would be carried in someone with suspected RA?

Answer 7

I: Blood tests, Test for RF and anti-CCP, Test for anaemia
T: NSAIDs, Corticosteroids, DMARDs and Biological Agents

Question 8

What are 2 examples of inherited connective tissue disorders?

Answer 8

1) Ehler-Danlos syndrome

2) Marfan’s Syndrome

Question 9

What are examples of autoimmune connective tissue diseases?

Answer 9

1) Systemic Lupus Erythematosus (SLE)
2) Systemic Sclerosis
3) Sjogren’s syndrome
4) Dermatomyositis

Question 10

What is SLE and the main epidemiology?

Answer 10

Inflammatory multi-system disease characterised by presence of serum ANA (anti-nuclear antibodies)
Ep: Afro-Caribbean, Genetic Association. 90% young women

Question 11

What can cause thrombosis and what autoantibody is specific to SLE?

Answer 11

• Antiphospholipid antibodies

Anti-dsDNA

Question 12

What is the pathogenesis of SLE?

Answer 12

1) Inefficient phagocytosis: Cell fragments transported to lymphoid tissue where taken up by APC, T cells stimulate B cells to produce antibodies against self-antigens
2) Immune complex deposition –> neutrophil and cytokine influx –> Inflammation and tissue damage

Question 13

What are the S/S and what investigations for SLE?

Answer 13

S/S: Butterfly rash, mouth ulcers, Raynaud’s phenomenon, fatigue, depression and weight loss
Investigations: Blood count shows normocytic anaemia, neutropenia, thrombocytopenia, Increased ESR
- Serum Antibodies: ANA and anti-dsDNA

Question 14

What would the P and N-P treatment of SLE involve?

Answer 14

P: Corticosteroids, NSAIDs, Anti-malarials, Anti-coagulants and Biological therapy (Target B cells)
N-P: Education/support, UV protection, screening for organ involvement and reduction in CV risk factors

Question 15

What is scleroderma and what are the 2 main types?

Answer 15

• Multi-system disorder shown by skin hardening and Raynaud’s phenomenon
  1) Limited Scleroderma
  2) Diffuse Scleroderma

Question 16

What are the 4 main signs of diffuse scleroderma?

Answer 16

1) Proximal scleroderma
2) Pulmonary Fibrosis
3) Bowel involvement
4) Myositis
5) Renal crisis

Question 17

What are the 5 main signs of limited scleroderma?

Answer 17

1) Calcinosis
2) Raynaud’s phenomenon
3) Oesophageal reflux
4) Sclerodactyly
5) Pulmonary arterial hypertension
6) Telangiectasia

Question 18

Whats the PP and management of Scleroderma?

Answer 18

PP: Various factors cause endothelial lesion and vasculopathy, with excessive collagen deposition, inflammation and auto-antibody production
Manage the following:
a) GORD: Prescribe PPI’s
b) Raynaud’s: physical protection and vasodilators
c) Annual Echo and Pulmonary Function tests to monitor arterial pulmonary pressure
d) ACEi to prevent renal impairment

Question 19

What is the PP of Sjogrens syndrome?

Answer 19

Immunologically mediated destruction of epithelial exocrine glands (Lacrimal and Salivary)
(Occurs secondarily to other auto-immune disorders)

Question 20

What are the main symptoms of sjogrens syndrome?

Answer 20

1) Dry eyes and dry mouth 9Destruction of lacrimal and salivary glands)
2) Inflammatory arthritis
3) Rash
4) Neuropathies
5) Vasculitis

Question 21

What are the investigations and treatment for Sjogren’s syndrome?

Answer 21

Inv: Look for serum auto-antibodies e.g. anti-RO, RF and ANA (Raised immunoglobulins and ESR)
T: Tear/Saliva replacement and immunosuppression for systemic complications

Question 22

What is dermatomyositis?

Answer 22

Rare disorder of unknown cause with inflammation and necrosis of skeletal muscle fibres and skin

Question 23

What are the symptoms, investigations and treatment of dermatomyositis?

Answer 23

S: Rash, muscle weakness and lungs affected
Inv: ^ Muscle enzymes, EMG, muscle/skin biopsy, screen for malignancy and chest x-ray
T: Steroids and immunosuppressants

Question 24

What term is given for inflammation of an entire digit of hand or foot?

Answer 24

Dactylitis