Rheumatoid Arthritis and Connective Tissue Diseases Flashcards
Define Rheumatoid Arthritis
Inflammatory AI joint disease with synovial swelling
What is the epidemiology and aetiology of R. Arthritis?
E: 2/3x ^ in women, 1% of population
A: Auto-antibodies lead to a defective cell-mediated immune response
What is the pathophysiology of RA?
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
What are the S/S of RA?
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
How does RA extra-articular involvement affect different organs?
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
Describe the RF
Antibody works against FC portion of IgG
What investigations and treatment would be carried in someone with suspected RA?
I: Blood tests, Test for RF and anti-CCP, Test for anaemia
T: NSAIDs, Corticosteroids, DMARDs and Biological Agents
What are 2 examples of inherited connective tissue disorders?
1) Ehler-Danlos syndrome
2) Marfan’s Syndrome
What are examples of autoimmune connective tissue diseases?
1) Systemic Lupus Erythematosus (SLE)
2) Systemic Sclerosis
3) Sjogren’s syndrome
4) Dermatomyositis
What is SLE and the main epidemiology?
Inflammatory multi-system disease characterised by presence of serum ANA (anti-nuclear antibodies)
Ep: Afro-Caribbean, Genetic Association. 90% young women
What can cause thrombosis and what autoantibody is specific to SLE?
- Antiphospholipid antibodies
Anti-dsDNA
What is the pathogenesis of SLE?
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
What are the S/S and what investigations for SLE?
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
What would the P and N-P treatment of SLE involve?
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
What is scleroderma and what are the 2 main types?
- Multi-system disorder shown by skin hardening and Raynaud’s phenomenon
1) Limited Scleroderma
2) Diffuse Scleroderma
What are the 4 main signs of diffuse scleroderma?
1) Proximal scleroderma
2) Pulmonary Fibrosis
3) Bowel involvement
4) Myositis
5) Renal crisis
What are the 5 main signs of limited scleroderma?
1) Calcinosis
2) Raynaud’s phenomenon
3) Oesophageal reflux
4) Sclerodactyly
5) Pulmonary arterial hypertension
6) Telangiectasia
Whats the PP and management of Scleroderma?
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
What is the PP of Sjogrens syndrome?
Immunologically mediated destruction of epithelial exocrine glands (Lacrimal and Salivary)
(Occurs secondarily to other auto-immune disorders)
What are the main symptoms of sjogrens syndrome?
1) Dry eyes and dry mouth 9Destruction of lacrimal and salivary glands)
2) Inflammatory arthritis
3) Rash
4) Neuropathies
5) Vasculitis
What are the investigations and treatment for Sjogren’s syndrome?
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
What is dermatomyositis?
Rare disorder of unknown cause with inflammation and necrosis of skeletal muscle fibres and skin
What are the symptoms, investigations and treatment of dermatomyositis?
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
What term is given for inflammation of an entire digit of hand or foot?
Dactylitis
