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MSK block > Connective tissue diseases (R4) > Flashcards

Connective tissue diseases (R4) Flashcards

1
Q

In connective tissue diseases multisystem organ involvement is common, list 4 examples of this

A
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2
Q

What is SLE?

A

Multisystem autoimmune disorder with multiorgan involvement and a broad range of disease manifestation that vary over time

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3
Q

State the following about SLE:

  • Who it primarily affects
  • Peak age
  • Ethnicitys at an increased risk
A

Primarily affects women of childbearing age M:F: 1:12

Peak incidence 15-45 years old

African, Caribbean and Chinese are at higher risk

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4
Q

What is the most common form of chronic SLE?

A

Discoid SLE

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5
Q

SLE presentation summary

A
  • Fatigue, Wt loss, Fever
  • Arthralgia, Myalgia and non-erosive arthritis
  • Photosensitive malar rash
  • Lymphadenopathy and splenomegaly
  • SOB, Pleuritic chest pain
  • Mouth ulcers, hair loss
  • Raynaud’s phenomenon
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6
Q

Describe the typical Rash of SLE

A

Photosensitive malar rash - “butterfly” shaped across the nose and cheek bones

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7
Q

List 4 clinical musculoskeletal manifestations of SLE

A
  1. Non-erosive Arthritis
  2. Jaccoud arthropathy
  3. Arthralgia, Myalgia
  4. Myositis
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8
Q

List 4 dermatological manifestations of SLE

A
  1. Photosensitive malar rash
  2. Mouth ulcers
  3. Hair loss
  4. Cutaneous vasculitis (splinter haemorrhages/ purpura)
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9
Q

List 2 hematological manifestations of SLE

A
  1. Anemia of chronic disease (normocytic)
  2. Lymphopenia

Hemolyticanemia, Leukopenia, Thrombocytopenia, Pancytopenia

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10
Q

List 2 reticuloendothelial manifestations of SLE

A

Lymphadenopathy and Splenomegaly

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11
Q

List 2 pulmonary manifestations of SLE

A
  1. Pleural effusion
  2. Pneumonitis

ILD, Pulmonary hemorrhage, pulmonary hypertension, PE

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12
Q

List 3 cardiac manifestations of SLE

A
  1. Pericarditis (most common)
  2. Raynaud’s
  3. Myocarditis (arrhythmias and HF)
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13
Q

List 4 renal manifestations of SLE

A

Lupus nephritis - most common cause of SLE related death

ankle oedema, active sediment, hematuria, proteinuria, nephrotic syndrome, reduced eGFR

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14
Q

List 4 SNS and 4 PNS manifestations ‘neuropsychiatric SLE’

A
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15
Q

List 2 autoantibodies associated with SLE

State which is the most specific

A
  1. ANA
  2. anti-dsDNA (specific to SLE)

+ Anti; Smith, centromere, Ro, La, Scl70, Jo1

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16
Q

What 2 criteria allow a diagnosis of SLE to be made?

A

SLICC Criteria or the ACR Criteria

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17
Q

Explain the SLICC criteria

A
  1. At least 4 criteria incl at least one clinical AND one immunologic OR
  2. Biopsy proven lupus nephritis with positive ANA or Anti-DNA
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18
Q

First line treatment for SLE

A
  1. NSAIDs
  2. Steroids (prednisolone)
  3. Hydroxychloroquine (first line for mild SLE)
  4. Suncream and sun avoidance
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19
Q

What is Sjogren Syndrome?

A

A slowly progressive, inflammatory autoimmune disease affecting primarily the exocrine glands

20
Q

Compare primary vs secondary Sjogrens syndrome

A

Primary: condition occurs in isolation.

Secondary: occurs related to SLE or RA

21
Q

Pthophysiology of Sjogren Syndrome

A

Lymphocytic infiltration replaces functional epithelium leading to decreased exocrine secretions

22
Q

Is Sjogren Syndrome more common in males or females?

What is the peak age affected?

A

F:M= 9:1

40-50 years old

23
Q

List 3 clinical features of Sjogren Syndrome

A
  • Fatigue
  • Dry eyes, sensation of sand in eyes/ burning, redness of the eyes
  • Dry mouth may be associated with difficulty eating and swallowing food
24
Q

List 4 EXTRA-glandular manifestations of Sjogren Syndrome

With these manifestations what other disease must we always consider?

A
  • Arthritis, arthralgia
  • Skin : Raynaud’s phenomenon, palpable purpura
  • Pulmonary: ILD, hilar lymphadenopathy
  • Renal: interstitial nephritis, GN (rare)
  • Neuromuscular: peripheral sensomotor neuropathy, cranial neuropathy

ALWAYS THINK LYMPHOMA!!

25
Q

List 4 Laboratory findings of Sjogren Syndrome

A
  1. Anemia of chronic disease
  2. Lymphopenia
  3. Increased ESR, normal CRP
    • ANA, Ro, La, RA
  5. Hypergammaglobulinemia
  6. Reduced C3, C4
26
Q

Which antibodies are specifically associated with Sjogren’s syndrome?

A

Anti-Ro and Anti-La

27
Q

List 3 diagnostic investigations for Sjogren Syndrome

A
  1. Salivary flow assessment: <1.5ml/15 min
  2. SCHIRMER TEST: <5mm in both eyes in 5 min
  3. Rose Begal staining: (stain of damaged epithelium)
28
Q

Explain the Schirmer Test

A

Filter paper inserted under lower eyelid with strip hanging out for 5 minutes

Distance along strip that becomes moist is measured

Normally tears should travel 15mm, < 10mm is significant

29
Q

List 4 treatments for Sjogren Syndrome

A
  1. Artificial tears
  2. Artificial saliva
  3. Vaginal lubricants
  4. Hydroxychloroquine is used to halt the progression of the disease
30
Q

What is Scleroderma?

A

Autoimmune disease characterised by fibrosis across multiple organ systems and increased production of collagen in tissues

Also known as Systemic Sclerosis

31
Q

Is scleroderma more common in males or females?

What is the peak age affected?

A

F:M: 4:1

45-60 y.o

32
Q

What are the 4 criteria that classify scleroderma?

A
  1. SKIN THICKENING PROXIMAL TO THE MCP +
  2. Sclerodactyly
  3. Digital pitting scars
  4. Bibasilar lung fibrosis
33
Q

What are the 2 types of Scleroderma?

A

Limited and Diffuse

34
Q

What is Diffuse Cutaneous Systemic Sclerosis?

A

Subcategory of the scleroderma that affects multiple systems

Highest association with African Americans

35
Q

What is Limited Cutaneous Systemic Sclerosis?

A

Subcategory of Scleroderma that affects fewer organs - sparing the kidneys and skin on the trunk and proximal limbs

Also known as CREST syndrome

36
Q

List 5 features of limited cutaneous systemic sclerosis

(Hint: CREST)

A
  • Calcinosis
  • Raynaud’s phenomenon
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
37
Q

What antibody is associated with limited vs diffuse scleroderma?

A

Limited: Anti-centromere antibody

Diffuse: Anti-scl 70

38
Q

List 3 initial features of systemic sclerosis

A
  1. Skin becomes tight
  2. Fingers become stiff and inflexible
  3. Symptoms of Raynauds
39
Q

List 4 later features of systemic sclerosis

A
  1. Tightening of skin on the face and around lips
  2. Fatigue
  3. Joint and muscle pain
  4. Ulceration
  5. Vascular disease, Incl CAD
  6. Pulmonary fibrosis and PAH
  7. Renal failure (“scleroderma renal crisis”)
40
Q

List 4 signs of systemic sclerosis

A
  1. Telangiectasia and calcinosis
  2. Barrnett’s sign: vertical striation with neck extension
  3. Mauskopf facies
  4. Salt and pepper (changes in pigmentation)
41
Q

List 4 differences between limited vs diffuse scleroderma

A
42
Q

What is Raynaud’s phenomenon

A

Episodic peripheral ischaemia in response to cold or emotion

Can be Primary or Secondary

43
Q

Is Raynaud’s phenomenon more common in males of females?

A

F:M: 9:1

44
Q

Explain the ‘triphasic reaction’ of Raynaud’s

A

Skin changes from white to blue to red

  1. Pallor: vasoconstriction of digital vessels
  2. Cyanosis: deoxygenation of static venous blood
  3. Red : reactive hyperemia due to reperfusion
45
Q

Raynaud’s is most commonly associated with what disease?

A

SLE

46
Q

First line management for Raynauds

A

Nifedipine (dihydropyridine CCBs)

47
Q

Revise slide on connective tissue diseases

A

MSK block (30 decks)

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