Autoimmune rheumatological disorders Flashcards

1
Q

Examples

A
  • Systemic Lupus Erythematosus
  • Antiphospholipid syndrome
  • Sjogrens syndrome
  • Polymyositis (and dermatomyositis)
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2
Q

Describe Systemic Lupus Erythematosus

A

An inflammatory, hetergenous, multisystem autoimmune disorder with antinuclear antibodies as wells as arthralgia and rashes
as the most common clinical features, and cerebral and renal disease as the
most serious problems.

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3
Q

Clinical presentation of Systemic Lupus Erythematosus

A

General:
Fatigue, Photo-sensitive rash, malaise, fever, splenomegaly, lymphadenopathy, pleuritic chest pain, headache, Raynaud’s. mild hair loss myalgia
*Erythema in a Butterfly distribution on the cheeks of the face and across the bridge of the nose
*Arthralgia: (like RA)
Symmetrical joint and muscle pain
Mucocutaneous:
Discoid lupus, potosensitive rash, *butterfly rash
Pulmonary:
Pleurisy, Fibrosing alveolitis, obliterative bronchiolitis
CVS:
Pericarditis, hypertension
Renal:
Nephritis, Haematuria, *glomerulonephritis
Neuro:
Anxiety and depression

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4
Q

Pathophysiology of Systemic Lupus Erythematosus

A

Apoptotic cells and cell fragments are cleared inefficiently by phagocytes

  • > transfer to lymphoid tissue
  • > taken up by antigen presenting cells
  • > self-antigens including nuclear components are presented to T cells
  • > T cells stimulate B cells to produce autoantibodies against the antigens.

Clinical manifestations are mediated by antibody formation and the development and deposition of immune complexes, complement activation and influx of neutrophils and abnormal cytokine production.

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5
Q

Aetiology of Systemic Lupus Erythematosus

A

Unknown
Likely multifactorial
Strong genetic association (HLA genes have been linked or family hx)
UV light (onset of rash)
Epstein-Barr virus can also trigger SLE
Higher incidence premenopausal (oestrogen has an effect)

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6
Q

Epidemiology of Systemic Lupus Erythematosus

A

More common in premenopausal females
Peak age of onset between 20-40 years (typically child bearing age)
More common in Asians and African-Caribbeans

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7
Q

Diagnostic tests of Systemic Lupus Erythematosus

A

FBC
ESR
Antinuclear factor

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8
Q

Treatment of Systemic Lupus Erythematosus

A

Avoid sunlight and smoking
NSAIDs and corticosteroid for arthralgia
Immunosuppressives for severe symptoms
Rituximab

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9
Q

Complications of Systemic Lupus Erythematosus

A

Depends on organ involvement

Characterised by relapse and remission

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10
Q

Describe Antiphospholipid syndrome

A

Autoimmune disorder characterised by arterial and venous thrmobosis and raised levels of antiphospholipid antibodies

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11
Q

Clinical presentation of Antiphospholipid syndrome

A
Varied clinical features
Coagulation problems;
Livedo reticularis;
Obstetric problems;
Thrombocytopenia

Also Peripheral artery thrombosis, deep venous thrombosis, cerebrovascular disease, pregnancy loss, MI, retinal thrombosis, pulmonary embolism and pulmonary hypertension

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12
Q

Pathophysiology of Antiphospholipid syndrome

A

Proposed mechanisms for hypercoagulable effect of aPL-antibodies include complement activation, the production of antibodies against coagulation factors, activation of platelets, activation of vascular endothelium and a reaction of antibodies to oxidised low density lipoprotein

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13
Q

Aetiology of Antiphospholipid syndrome

A

Unknown - some genetic association

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14
Q

Epidemiology of Antiphospholipid syndrome

A

Higher prevalence in black people

Occurs most commly in women of fertile age

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15
Q

Diagnostic test of Antiphospholipid syndrome

A

Screen for aPL antibodies

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16
Q

Treatment of Antiphospholipid syndrome

A

Healthy lifestyle

Acute management of thrombosis - Heparin

17
Q

Complications of Antiphospholipid syndrome

18
Q

What is Sjogrens syndrome

A

Lymphocytic infiltration of exocrine glands

19
Q

Sjogrens syndrome clinical presentation

A

Dry eyes
Dry mouth
Enlargement of parathyroid glands
Possibly blepharitis

20
Q

Sjogrens syndrome pathophysiology

A

Not fully known

Possibly inflammation of the glands prompts AI response

21
Q

Sjogrens syndrome aetiology

A

Primary - idiopathic

Secondary - associated with another AI condition, often rheumatoid arthritis

22
Q

Sjogrens syndrome epidemiology

A

Classically disease of middle aged women

23
Q

Sjogrens syndrome diagnostic tests

A

Serum antibodies

Schirmer’s test - whether the eye can self hydrate

24
Q

Treatment of Sjogrens syndrome

A

Artificial tear and saliva replacement

25
Sjogrens syndrome complications
Infection of eyes or mouth
26
What is polymyositis
Inflammation and necrosis of skeletal muscle fibres
27
Clinical presentation of polymyositis
Symmetrical progressive muscle weakness and wasting, affecting the proximal muscles of the shoulder and pelvic girdle. Difficulty squatting and with stairs, and with raising hands above head. Can involve dysphagia and dysphonia if pharyngeal and laryngeal muscles are involved.
28
Pathophysiology of molymyositis
Mediated by cytotoxic T cells | Involves autoimmune factors
29
Aetiology of polymyositis
Unknown
30
Epidemiology of polymyositis
Presents between 30 and 60 (small peak at 15) | Twice as common in females
31
Diagnostic tests of polymyositis
Muscle biopsy
32
Treatment of polymyositis
Oral prednisolone
33
Complications of polymyositis
Interstital lung disease, dysphagia, infection
34
``` Woman 23 years old presents with mouth ulcers, fever, painful white fingers (Raynaud's) and pleuritic chest pain. ANA positive ESR raised and white cell count low Which of these features would you NOT expect to be associated with her illness? Deforming arthritis Photosensitive rash Seizures Pulmonary embolism Thrombocytosis ```
Thrombocytosis | Case of SLE