Autoimmune rheumatic diseases

Question 1

SLE patho and hallmarks

Answer 1

more women, 20-40 years, higher African-Caribbean, ANA, complement activation

Question 2

SLE clinical features most common

Answer 2

joint involvement MOST COMMON feature –> arthralgia, symmetrical in small joints, erosion and deformity rare
Skin –> malar rash, photosensitivity, raynaud’s, vasculitic lesions, purpura, livedo reticularis, alopecia

Question 3

SLE clinical features additional

Answer 3

Lungs –> pleural effusion, pneumonitis, collapse lung
Heart –> pericarditis, ischaemic heart disease, stroke
Kidneys –> nephritis, proteinuria
Nervous system –> depression, epilepsy, migraine, polyneuropathy, cerebral lupus
Eyes –> retinal vasculitis, conjunctivitis, optic neuritis, episcleritis
GI –> mouth ulcers

Question 4

Management SLE

Answer 4

NSAIDs, hydroxychloroquine

Corticosteroids in severe disease

Question 5

Anitphospholipid syndrome (APS): patho + clinical features

Answer 5

antiphospholipid antibodies (not all with antibodies get APS)
thrombosis & frequent miscarriages, ischaemic strokes, often in SLE patients

Question 6

APS management

Answer 6

long-term anticoagulation with warfarin

pregnant women get oral aspirin + low-molecular weight heparin

Question 7

Systemic sclerosis (scleroderma) (SSc): patho / hallmarks

Answer 7

more females, 30-50 years, raynauds in 100% of cases
widespread vascular damage (small vasculitis) –> vasoconstriction, increased permeability, ischaemia, fibrosis lower dermis + internal organs

Question 8

SSc clinical symptoms limited cutaneous scleroderma (70%)

Answer 8

raynauds before skin involvement
skin limited to face, hands, feet, forearms
characteristic ‘beak’-like nose + small mouth
GI involvement + pulmonary hypertension

Question 9

SSc clinical symptoms diffuse cutaneous scleroderma (30%)

Answer 9

oedematous onset
skin thickening + atrophy
GI, renal, lung involvement, myocardial fibrosis

Question 10

Antibodies SSc

Answer 10

Limited = ACA
Diffused = anti-topoisomerase 1 antibodies = anti-Scl-70
RF positive + ANA positive often

Question 11

Management SSc

Answer 11

No cure, organ-based symptomatic relieve

Question 12

Adult polymyositis symptoms

Answer 12

women, shoulder & pelvic girdle muscles wasted, disease progresses: pharyngeal, laryngeal, respiratory muscles involved

Question 13

Adult dermatomyositis

Answer 13

muscle weakness, myalgia, polyarthritis, raynaud’s, rash eyelids (purple discoloration + oedema), rash fingers over knockles (purple-red vasculitic patches)

Question 14

Antisynthetase syndrome

Answer 14

people with PM or DM antibodies against tRNA synthetase enzymes –> more risk to develop pulmonary interstitial fibrosis, dysphagia, raynaud’s, arthritis, hardening & fissuring skin fingers

Question 15

Treatment polymyositis / dermatomyositis

Answer 15

prednisolone, rituximab in autoantibody cases

Question 16

Sjögren syndrome symptoms

Answer 16

dryness eyes, mouth, skin, vagina, enlargement salivary & parotid glands

Question 17

Sjögren syndrome diagnosis tests

Answer 17

Schirmer tear test –> wetting < 10 mm in 5 min = defective tear production
Rose Bengal staining –> staining eyes shows punctate or filamentary keratitis

Question 18

Sjögren syndrome management

Answer 18

artificial tears

saliva replacement solutions

Question 19

undifferentiated ARD

Answer 19

patients with evidence of autoimmunity and some clinical features but not enough to make definit diagnosis