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Semester 2 W3 Connective tissue > Connective tissue disorder > Flashcards

Connective tissue disorder Flashcards

1
Q

Describe the pathology of SLE

A

T and B cell dysfunction, causing B cell hyperactivity and impaired immune complex clearance fro tissues

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2
Q

Name the musculosleletal features of SLE

A

Arthralgia, usually polyarticular. Deformity fromtenosynovitis anf fibrosis

Myalgia

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3
Q

What is myalgia

A

muscle pain

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4
Q

What is myositis

A

Muscle inflammation

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5
Q

What are the dermatological signs of SLE

A

“butterfly” maar rash over nose and cheeks,

discoid lupus, alopecia, mucosal ulceration of the nose, mouth, vagina, cutaneous vasculitis

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6
Q

What does SLE stand for

A

Systeatic lupus erythematosus

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7
Q

What are the cardiovascular features of SLE

A

Serositis, pericarditis, myocarditis (arrythmia or heart failure)

Reynauds

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8
Q

Describe reynaud phenomenon

A

Vasospasm especially incold, causing peripheral ischaemia, seen in digits, tip of nose, earlobes and occasionally tongue, turning pale and numb, then blue. Final phase is red flushing due to vasodilation

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9
Q

Describe the pulmonary features of SLE

A

Pleurisy and pleural effusions

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10
Q

Describe the renal features of SLE

A

Glomerulonephritis. Does not have symptoms until significant renal damage

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11
Q

Describe the neurological features of lupus

A

Headaches and neuropsychiatric problems, depression, anxiety

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12
Q

Describe the haematological features of SLE

A

Lymphopenia, neutropenia

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13
Q

Describe the spectrum of lupus

A

All overlap with classic lupus,
Late stage lupus, drug induced lupus

Antiphospholipid syndrome and latent lupus also overlap

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14
Q

Does lupuc cause multiple oral ulcers

A

yes

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15
Q

How does the lupus rash vary from acute to subacute to chronic

A

Acute is malar butterfly rash, subacute is annular, chronic is discoid

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16
Q

What are the serological tests for SLE

A

Antinuclear antibodies, (ANA) Anti-Ro and anti-La antibodies
Anti-double stranded DNA antibodies (Anti-DNA)
Antihistone antibodies
Antiphospholipid and anticardiollpin ntibodies

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17
Q

What are the non-serological tests of SLE

A

Urine for kidney function, FBC, ESR, CRP, urea, creatinine, electrolyte,complement, coombs tests, skin IgG and renal biopsy

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18
Q

What is antiphospholipid syndrome

A

Arterial and venous thrombosis, fetal loss, Thrombocytopenia associated with antiphospholipid levels

Detected with Lupus anticoaglualnt and anticardiolipin antibodies

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19
Q

How is mild SLE treated pharmacologically

A

Patients with arthralgian, lethargy, or a faint rash may respond to NSAIDs or antimalarials like hydroxychloroquine

20
Q

How is moderate SLE treated pharmacologially

A

Severe clinical features like serositis, severe arthritis, nephritis, thrombocytopenia or psychiatric issues may be treated with corticosteroids.

Upon remission, steroid-saving agents used like methotrexate often used

21
Q

How is Severe SLE treated pharmacologically

A

life threatening complications like acute renal failure, neurological or haematological problems must be treated promptly with cytotoxic medication and corticosteroids

22
Q

What treatment other than pharmacological is used for SLE

A

education against factors that could cause flares (Overexposure to sunlight, oestrogen contraception, infection, stress, drugs like hydralazine

23
Q

What is the general advice for minimising risk of antiphospholipid syndrome

A

Avoid oral contraceptive pill, avoid smoking, treat hypertension, diabetes, hyperlipidaemia.

Asymptomatic patients not treated, but monitored.

24
Q

How are patients with antiphospholipid syndrome venous/arterial thrombosis treated

A

Lifelong conventional anticoagulation such as warfarin

25
Q

How are patients with recurrent fetal loss treated

A

Warfarin stopped before conception when attempting another pregnancy. Heparin begins

26
Q

What is Sjogren’s syndrome?

A

A slowly progressive inflammatory autoimmune disease affecting primarily the exocrine glands as lymphocytic infiltratrs replace functional epthelium, reducing secretions

27
Q

What are the characteristic antibodies of Sjogrens

A

Ro and La

28
Q

What are the clinical features of Sjogren’s syndrome regarding the eyes

A

Reduced tear secretion causes destruction of corneal and conjunctival epithelium, giving dry, gritty sore eyes that could be reddened. Bacteril conjuntivitis common

29
Q

What are the clinical features of Sjogrens regarding the mouth

A

Dryness of mouth, difficulty swallowing dry food, very little saliva

30
Q

What are systematic features of sjogrens

A

Constitutuional (fever, fatigue, weight loss)

Non erosive polyarthritis, reynauds, digital ulcers, lung/kidney, vasculitis, neuropathy, increased lymphoma risk

31
Q

What are the investigations for sjogrens

A

Bloods: Anaemia, high ESR, normal CRP, hypergammaglobulinaemia, ANA, Ro, La

Shirmers test,

Major salivary gland biopsy

32
Q

What is shirmers test

A

Filter paper shows how much it is wetted. less than 5mm is evident

33
Q

How is Sjogrens treated

A

Topical and symptomatic treatment, with eyedrops and saliva substitutes

Hydroxychloroquine can help with the symptoms

34
Q

What is systemic sclerosis

A

Scleroderma means a hardening of skin, and can be used to describe a wide spectrum of disease which may be cutaneous or systemic

35
Q

What are the clinical features of scleroderma

A

reynauds, progressive fibrosis of skin, lung, heart, GI tract and kidney.

May be diffuse cutaneous, or limited scleroderma

36
Q

What is slcerodactyly

A

Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes.

37
Q

What is microstomia

A

eduction in the size of the oral aperture that is severe enough to compromise cosmesis, nutrition, and quality of life.

38
Q

What are the skin manifestations of scleroderma

A 
Sclerodactyly,
Microstomia
Furrowing of skin around the lips
Loss of normal skin creases
Tethering of skin to underlying structures, skin hypo/hyper-pigmentation
Flexion contractures at joints
Thinning and atrophy
39
Q

What does CREST stand for

A

Calcinosis, Reynauds, oEsopahgeal disease, sclerodactyly, Telangiectasia

40
Q

What is telangiectasia?

A

condition in which widened venules (tiny blood vessels) cause threadlike red lines or patterns on the skin. These patterns, or telangiectases, form gradually and often in clusters.

41
Q

Is CREST syndrome referring to limited or diffuse scleroderma?

A

Limited

42
Q

What are the sgns of diffuse systemic sclerosis

A

Skin fibrosis of limbs, face, trunk, neck

Less common skin tethering to underlying surfaces

Prominent swelling and pruritis (itching)

Less common telangiectasia and calcinosis

43
Q

Where does limited SS see skin thickening

A

Distal sites, with hands and feet (sclerodactyly_, face and neck

44
Q

How can scleroderma affect the kidneys

A

Slceroderma renal crisis is rapidly progressive renal failure associated with severe hypertension

45
Q

How does systemic sclerosis have pulmonary manifestations

A

Interstitial lung disease, pulmonary hypertension

46
Q

What are the seroligical tests for SS

A

Anticentromere antibodies indicate good prognosis antitoposomerase, associated with diffuse diseasse and pulmonary fibrosis
ANA in most patienets

47
Q

How is systemic sclerosis treated

A

No definitive treatment, treating subsequent issues. No steroids, ACE inhibitors in renal crisis

Semester 2 W3 Connective tissue (3 decks)

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