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MSK > Connective tissue diseases > Flashcards

Connective tissue diseases Flashcards

1
Q

Pathology of CREST syndrome

A 
Formed of systemic sclerosis 
Calcinosis 
Raynaud's syndrome 
Esophageal dysmotility 
Sclerodactyly 
Telangiectasia
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2
Q

Causes of CREST syndrome

A

Chronic autoimmune inflammation triggered by anticentromere antibodies

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3
Q

S+S CREST

A 
Calcific nodules under skin 
White-blue-red skin colour transitions in cold 
Dysphagia 
Sclerodactyly 
Telangiectasias on hands + face
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4
Q

What are the complications of CREST syndrome?

A

Ischemic ulcers
Gangrene
Predisposition to skin infections
Upper GI bleeds

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5
Q

Lab results for CREST syndrome

A

ANA + ACAs present

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6
Q

Management of CREST syndrome

A

Steroids

Immunosuppressants (cyclosporine)

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7
Q

Pathology of fibromyalgia

A

Central sensitisation, hypersensitivity to pain, sleep disturbances
Reduced serotonin
Increased substance P
Increased nerve growth factor

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8
Q

S+S of fibromyalgia

A

Low threshold to pain
Widespread muscle pain
Sleep disturbances
Difficulty concentrating

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9
Q

What are the symptom severity measures for fibromyalgia?

A 
Fatigue, waking unrefreshed, cognitive symptoms, somatic symptoms 
0 = no problem 
1 = mild 
2 = moderate 
3 = severe
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10
Q

Diagnostic criteria for fibromyalgia

A

Pain in >7 areas of body with symptom severity of >5 in >5 areas
Symptoms present > 3 months

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11
Q

Management of fibromyalgia

A

Antidepressants - TCAs, SNRIs
Anticonvulsants
CBT

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12
Q

Pathology of mixed connective tissue disease

A

Overlap autoimmune syndrome

SLE, systemic sclerosis, polymyositis

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13
Q

Complications of mixed connective tissue disease

A

Pulmonary hypertension
Interstitial lung disease
Renal disease

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14
Q

S+S of mixed connective tissue disease

A 
Arthralgias 
Myalgias 
Swollen hands + puffy fingers 
Sclerodactyly 
Raynauds 
Fatigue 
Low grade fever
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15
Q

Lab results for mixed connective tissue disease

A

High anti-U1 ribonucleoprotein antibodies (anti-U1-RNP)

High ANA, RF

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16
Q

Management of mixed connective tissue disease

A

Steroids

17
Q

Pathology of scleroderma

A

AKA systemic sclerosis
Chronic inflammatory autoimmune disease resulting in damage to small blood vessels, excessive fibrosis
T helper cells activated + release cytokines, stimulate fibroblasts causing chronic inflammation

18
Q

What organs does scleroderma affect?

A 
Skin
GI tract
Kidneys 
Muscles 
Heart 
Lungs
19
Q

Types of scleroderma

A

Limited - limited to fingers, forearms + face. Associated with anticentromere antibodies, may develop CREST
Diffuse - widespread skin involvement, rapid progression, associated with anti-DNA TI antibodies

20
Q

RF for scleroderma

A

Females
35-50
Genetics

21
Q

Complications of scleroderma

A

Excessive skin fibrosis - painful ulcers, disfigurement, disability
Renal + cardiac failure
Pulmonary insufficiency
Intestinal malabsorption

22
Q

S+S of scleroderma

A

Raynauds (precedes other symptoms)
Cutaneous changes of face + extremities (skin thickening + tightening, sclerosis, edema, erythema)
GI involvement - esophageal fibrosis, abdo pain, obstruction

23
Q

How is the diagnosis of scleroderma made?

A

Upper endoscopy (fibrosis)
High ANAs
High ACAs
Anemia

24
Q

Management of scleroderma

A

PPIs for reflux
CCB for Raynauds
ACEi for renal hypertension
Immunosuppressant therapy

25
Q

Pathology of Sjogren’s syndrome

A

autoimmune inflammatory disease
Lymphocytic infiltration
Destruction of exocrine glands of eyes + mouth

26
Q

Causes of Sjogren’s syndrome

A 
Sicca syndrome (primary)
Secondary (to RA)
27
Q

S+S of Sjogren’s syndrome

A

Dry eyes, irritation + itching
Oral dryness
Salivary gland enlargement
MSK symptoms, rashes, vasculitis + interstitial nephritis

28
Q

Complications of Sjogren’s syndrome

A

Periodontal complications
Oral infections
MALT lymphoma

29
Q

Diagnostic tests for Sjogren’s syndrome

A 
Parotid gland MRI shows honeycomb pattern 
Leukopenia, thrombocytopenia, anemia 
High ESR
ANA + RF high 
Schirmer test (reflex tear production)
Salivary gland tests
30
Q

Management of Sjogren’s syndrome

A

Secretagogues

Immunosuppressive therapy

31
Q

Pathology of SLE

A

Systemic autoimmune disorder
Periods of flare ups + remittance
Immune complexes deposit in kidneys, skin, joints + heart
Type 3 hypersensitivity reaction
Ab targeting molecules - type 2 hypersensitivity reaction

32
Q

RF for SLE

A 
Genes 
UV radiation 
Smoking
Viral + bacterial infections 
Medications 
Females 30-50
33
Q

Complications of SLE

A 
Libman-Sacks endocarditis 
MI 
Renal failure 
HTN
Antiphospholipid syndrome - prone to developing clots
34
Q

S+S of SLE

A 
Fever
Joint pain 
Rash (malar rash over cheeks, discoid rash, general photosensitivity) 
Ulcers in oral/ nasal mucosa 
Serositis 
Myocarditis 
Renal disorders 
Seizures + psychosis 
Anemia, thrombocytopenia, leukopenia
35
Q

Management of SLE

A

Animalarial agents
NSAIDs
Corticosteroids
Immunosuppressants

36
Q

What are the heritable connective tissue disorders?

A 
Marfans
Ehlers-Danlos 
Osteogenesis imperfecta 
Stickler syndrome 
Alport syndrome 
Congenital contractural arachnodactyly 
Loeys-Dietz syndrome

MSK (10 decks)

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