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Musculoskeletal System > Multi-System Autoimmune Disease > Flashcards

Multi-System Autoimmune Disease Flashcards

1
Q

What is diagnosis of autoimmune disease based upon?

A 
Cardinal clinical features picked up on history and examination
Immunology
Imaging
Tissue biopsy
Exclusion of differential diagnosis
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2
Q

What conditions can mimic autoimmune disease?

A
  • Drugs - cocaine, minocyline, PTU
  • Infection - HIV, endocarditis, Hepatitis, TB
  • Malignancy - lymphoma
  • Cardiac myxoma
  • Cholesterol emboli
  • Scurvey
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3
Q

What populations are most commonly affected by systemic lupus erythematous?

A

Mainly affects population aged 15-50
F:M ratio of 9:1
Significant ethnic diversity (afro-carribeans most affected, then Asian populations then Caucasian populations)

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4
Q

What are the signs and symptoms of systemic lupus erythematous?

A
  • Face- butterfly rash and photosensitivity
  • Alopecia
  • Nervous system- fits, hemiplegia, ataxia, peripheral neuropathy and cranial nerve lesions
  • Heart- pericarditis, endocarditis and aortic valve lesions
  • Chest- pleurisy, pleural effusion and fibrosis
  • Skin- vasculitis, purpura and urticaria
  • General- fever and depression
  • Abdominal pain
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5
Q

What are the possible skin manifestations of SLE?

A

Skin manifestations in SLE include a malar rash that spares the nasal folds and is painless. The rash can be confused with rosacea, but rosacea is often painful. Discoid rashes can also be present on the skin that have a scaly centre and a dark rim. SLE can also cause arthritis and kidney problems.

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6
Q

What are the clinical manifestations of limited scleroderma?

A
  • Calcinosis (calcium deposits in skin that can burst)
  • Renaulds
  • Esophageal dismobility
  • Sclerodactyly (swollen, puffy fingers)
  • Telangiectasia
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7
Q

What are the characteristics of diffuse scleroderma?

A

Diffuse scleroderma can affect the whole body and can result in flexural deformities of the fingers due to fibrosis

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8
Q

What is Sjogren’s syndrome?

A

Sjogren’s syndrome is a slightly more benign condition, with lots of patients never seeking medical treatment. The disease involves inflammation of the exocrine glands.

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9
Q

What are the clinical features of Sjogren’s syndrome?

A
  • Central nervous system- fits, hemiplegia, ataxia and cranial nerve lesions
  • Gastrointestinal- dysphagia and abnormal oesophageal motility
  • Kidneys- renal tubular necrosis
  • Respiratory- interstitial lung disease
  • Skin- palpable purpura, Raynaud’s syndrome
  • Joints- arthralgia
  • Peripheral nervous system- sensory neuropathy and mononeuritis multiplex
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10
Q

What is the most common presentation of Sjogren’s syndrome?

A

Patients with Sjogren’s syndrome most commonly present with dryness affecting the eyes and mouth lasting for at least three months. There may also be thick, gritty secretions arounf the eyes

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11
Q

How is eye and mouth dryness measure in Sjogren’s syndrome?

A

Eye dryness- Schermer’s test

Mouth dryness- ask the patient to collect all saliva for 15 mins after an hour’s fast.

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12
Q

What are the potential complications of Sjogren’s syndrome?

A
  • Lymphoma- 5% of patients will develop
  • Neuropathy
  • Purpura
  • Interstitial lung disease
  • Renal tubular acidosis
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13
Q

What are the two types of auto-immune myositis?

A

Polymyositis

Dermatomyositis

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14
Q

What are the characteristics of polymyositis?

A

Polymyositis does not have any cutaneous manifestations and will present with muscle weakness

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15
Q

What are the characteristics of dermatomyositis?

A

Dermatomyositis can cause a rash similar to Lupus rash but the rash in myositis does not spare the nasal folds
Muscle weakness also present

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16
Q

What are the possible complications of auto-immune myositis?

A

Cancer

Interstitial lung disease

17
Q

What common syndromes can overlap with auto-immune myositis?

A 
Mixed connective tissue disease
Soft tissue swelling
Primary Raynauds phenomenon
Myositis
Arthralgia
18
Q

What is the classification criteria for giant cell arteritis?

A

3 of the following:

  • Age at onset ≥50 years
  • New headache
  • Temporal artery tenderness/reduced pulsation
  • ESR≥50
  • Abnormal temporal biopsy
19
Q

What is ANCA associated vasculitis?

A

ANCA associated vasculitis can cause a saddle-shaped nose as well as ocular, skin and respiratory manifestations. ANCA associated vasculitis can be granulomatosis with polyangiitis, microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis.

20
Q

How is granulomatosis with polyangiitis characterised?

A
  • Necrotising granulomatous inflammation
  • Usually involving the upper and lower respiratory tract
  • Affecting predominantly small to medium vessels
  • Necrotising glomerulonephritis is common
21
Q

How is microscopic polyangiitis characterised?

A
  • Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels.
  • Necrotising arteritis involving small and medium arteries may be present
  • Necrotising glomerulonephritis is very common
  • Pulmonary capillaritis often occurs
  • Granulomatous inflammation is absent
22
Q

How is eosinophilic granulomatosis with polyangiitis characterised?

A
  • Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
  • Necrotising vasculitis predominantly affecting small to medium vessels
  • Associated with asthma and eosinophilia
  • ANCA is more frequent when glomerulonephritis is present
23
Q

In what conditions are ANA antibodies positive?

A 
Systemic lupus erythematous 
Systemic sclerosis
Polymyositis/dermatomyositis
Sjogrens syndrome
MCTD
Drug induced lupus
24
Q

In what conditions are ANA antibodies unhelpful?

A

Rheumatoid arthritis
Infection
Multiple sclerosis

25
Q

How is multisystem autoimmune disease treated?

A

Mild disease – hydroxychloroquine
Moderate- azathioprine, methotrexate, mycophenolate
Severe- cyclophosphamide, rituximab

26
Q

What populations are most commonly affected by scleroderma?

A

4th-6th decades

F>M 3:1

27
Q

How is scleroderma classified?

A

Scleroderma causes morphea to develop on the skin. These lesions can be circular or linear, and classified as either localised or systemic. Systemic scleroderma can be further classified as limited or diffuse

28
Q

How is limited scleroderma characterised?

A

Limited to areas proximal to the elbow and spares the trunk

29
Q

What are the complications of limited scleroderma?

A

Pulmonary hypertension (usually 10-15 years post-diagnosis)

30
Q

What are the complications of diffuse scleroderma?

A
  • Pulmonary fibrosis
  • Renal crisis
  • Small bowel bacterial overgrowth
31
Q

What populations are most commonly affected by Sjogren’s syndrome?

A

F>M 9:1

Most commonly diagnosed in those aged 40-50

Musculoskeletal System (28 decks)

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