Connective tissue disorder

Question 1

Describe the pathology of SLE

Answer 1

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

Question 2

Name the musculosleletal features of SLE

Answer 2

Arthralgia, usually polyarticular. Deformity fromtenosynovitis anf fibrosis

Myalgia

Question 3

What is myalgia

Answer 3

muscle pain

Question 4

What is myositis

Answer 4

Muscle inflammation

Question 5

What are the dermatological signs of SLE

Answer 5

“butterfly” maar rash over nose and cheeks,

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

Question 6

What does SLE stand for

Answer 6

Systeatic lupus erythematosus

Question 7

What are the cardiovascular features of SLE

Answer 7

Serositis, pericarditis, myocarditis (arrythmia or heart failure)

Reynauds

Question 8

Describe reynaud phenomenon

Answer 8

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

Question 9

Describe the pulmonary features of SLE

Answer 9

Pleurisy and pleural effusions

Question 10

Describe the renal features of SLE

Answer 10

Glomerulonephritis. Does not have symptoms until significant renal damage

Question 11

Describe the neurological features of lupus

Answer 11

Headaches and neuropsychiatric problems, depression, anxiety

Question 12

Describe the haematological features of SLE

Answer 12

Lymphopenia, neutropenia

Question 13

Describe the spectrum of lupus

Answer 13

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

Antiphospholipid syndrome and latent lupus also overlap

Question 14

Does lupuc cause multiple oral ulcers

Answer 14

yes

Question 15

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

Answer 15

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

Question 16

What are the serological tests for SLE

Answer 16

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

Question 17

What are the non-serological tests of SLE

Answer 17

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

Question 18

What is antiphospholipid syndrome

Answer 18

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

Detected with Lupus anticoaglualnt and anticardiolipin antibodies

Question 19

How is mild SLE treated pharmacologically

Answer 19

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

Question 20

How is moderate SLE treated pharmacologially

Answer 20

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

Question 21

How is Severe SLE treated pharmacologically

Answer 21

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

Question 22

What treatment other than pharmacological is used for SLE

Answer 22

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

Question 23

What is the general advice for minimising risk of antiphospholipid syndrome

Answer 23

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

Asymptomatic patients not treated, but monitored.

Question 24

How are patients with antiphospholipid syndrome venous/arterial thrombosis treated

Answer 24

Lifelong conventional anticoagulation such as warfarin

Question 25

How are patients with recurrent fetal loss treated

Answer 25

Warfarin stopped before conception when attempting another pregnancy. Heparin begins

Question 26

What is Sjogren's syndrome?

Answer 26

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

Question 27

What are the characteristic antibodies of Sjogrens

Answer 27

Ro and La

Question 28

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

Answer 28

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

Question 29

What are the clinical features of Sjogrens regarding the mouth

Answer 29

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

Question 30

What are systematic features of sjogrens

Answer 30

Constitutuional (fever, fatigue, weight loss) | Non erosive polyarthritis, reynauds, digital ulcers, lung/kidney, vasculitis, neuropathy, increased lymphoma risk

Question 31

What are the investigations for sjogrens

Answer 31

Bloods: Anaemia, high ESR, normal CRP, hypergammaglobulinaemia, ANA, Ro, La Shirmers test, Major salivary gland biopsy

Question 32

What is shirmers test

Answer 32

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

Question 33

How is Sjogrens treated

Answer 33

Topical and symptomatic treatment, with eyedrops and saliva substitutes Hydroxychloroquine can help with the symptoms

Question 34

What is systemic sclerosis

Answer 34

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

Question 35

What are the clinical features of scleroderma

Answer 35

reynauds, progressive fibrosis of skin, lung, heart, GI tract and kidney. May be diffuse cutaneous, or limited scleroderma

Question 36

What is slcerodactyly

Answer 36

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

Question 37

What is microstomia

Answer 37

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

Question 38

What are the skin manifestations of scleroderma

Answer 38

``` 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 ```

Question 39

What does CREST stand for

Answer 39

Calcinosis, Reynauds, oEsopahgeal disease, sclerodactyly, Telangiectasia

Question 40

What is telangiectasia?

Answer 40

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.

Question 41

Is CREST syndrome referring to limited or diffuse scleroderma?

Answer 41

Limited

Question 42

What are the sgns of diffuse systemic sclerosis

Answer 42

Skin fibrosis of limbs, face, trunk, neck Less common skin tethering to underlying surfaces Prominent swelling and pruritis (itching) Less common telangiectasia and calcinosis

Question 43

Where does limited SS see skin thickening

Answer 43

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

Question 44

How can scleroderma affect the kidneys

Answer 44

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

Question 45

How does systemic sclerosis have pulmonary manifestations

Answer 45

Interstitial lung disease, pulmonary hypertension

Question 46

What are the seroligical tests for SS

Answer 46

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

Question 47

How is systemic sclerosis treated

Answer 47

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