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Musculoskeletal > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

what is SLE

A

chronic autoimmune disease which mainly involves the skin, joints, kidneys, blood cells and NS but can affect almost any organ system

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2
Q

what is the pathogenesis of SLE

A

defect in apoptosis that causes increased cell death the defective clearance of apoptotic cell debris allows for persistence of antigen and immune complex production

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3
Q

how do the clinical manifestations occur in SLE

A

because the circulating immune complexes are deposited on blood vessels and on basement membrane of skin and kidneys

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4
Q

what is the prevalence of SLE

A

based on environmental genetics (if mother has SLE, her daughters risk is 1:40 and son risk 1:250)

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5
Q

what is typical SLE patient

A

asian, afro-americans or afro-caribbean women frequently starting at childbearing age

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6
Q

what causes SLE

A

genetics - high concordance in monozygous twins hormonal factors - those with high oestrogen exposure

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7
Q

what are general symptoms of SLE

A

fever, fatigue and weight loss

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8
Q

what are MSK symptoms of SLE

A

non-deforming polyarthritis deforming arthropathy - Jaccoud’s arthritis myopathy - weakness, myalgia, myositis inflammatory arthritis increased prevalence of AVN, usually of femoral head which may relate to steroid use

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9
Q

what are muco-cutaneous symptoms of SLE

A

malar rash photosensitivity discoid lupus subacute cutaneous lupus oral / nasal ulceration alopecia raynauds phenomenon

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10
Q

what are renal symptoms of SLE

A

lupus nephritis proteinuria of >500mg in 24 hours

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11
Q

what are respiratory symptoms of SLE

A

pleurisy pleural effusion pneumonitis PE pulmonary hypertension ILD

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12
Q

what are haematological symptoms of SLE

A

leukopenia lymphadenopathy leucopenia lymphopenia anaemia (may be haemolytic) thrombocytopenia

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13
Q

what are neuropsychiatric symptoms of SLE

A

seizures psychosis / depression headache aseptic meningitis

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14
Q

what are cardiac symptoms of SLE

A

pericarditis pleural effusion pulmonary hypertension sterile endocarditis accelerated ischaemic HD

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15
Q

what are GI symptoms of SLE

A

less common but include autoimmune hepatitis, pancreatitis and mesenteric vasculitis

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16
Q

what is diagnostic criteria of SLE

A

>4 criteria (at least 1 clinical and 1 laboratory criteria) OR biopsy proven lupus nephritis with positive ANA or anti-DNA

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17
Q

what is list of clinical criteria in diagnosis of SLE

A

acute cutaneous lupus chronic cutaneous lupus oral or nasal ulcers non scarring alopecia arthritis serositis renal neurologic haemolytic anaemia leukopenia thrombocytopenia

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18
Q

what is list of immunologic criteria in diagnosis of SLE

A

ANA anti-DNA anti-SM anti phospholipid Ab low complement (C3, C4, CH50) direct coombs test - do not count in presence of haemolytic anaemia

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19
Q

how is SLE treated

A

depends on manifestation avoid sun exposure

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20
Q

how is skin disease and arthralgia in SLE treated

A

hydroxychloroquine topical steroids NSAIDs

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21
Q

how is inflammatory arthritis or organ involvement in SLE treated

A

immunosuppression (azathioprine mycophenolate mofetil) corticosteroids for short period

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22
Q

how is severe organ disease (eg lupus nephritis or CNS lupus) in SLE treated

A

IV steroids cyclophosphamide

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23
Q

how is unresponsive cases of SLE treated

A

IV immunoglobulin and rituximab

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24
Q

how is SLE monitored

A

check anti-dsDNA antibodies and complement levels regularly also important to check urinalysis for blood or protein which may indicate glomerulonephritis

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25
Q

what is Sjorgens syndrome

A

autoimmune condition characterised by lymphocytic infiltrates in exocrine organs

26
Q

Sjorgens can be primary, or secondary to what

A

other autoimmune conditions eg RA and SLE

27
Q

what diseases are associated with sjorgens

A

peripheral neuropathy and ILD. Also risk of lymphoma

28
Q

what are symptoms of sjorgens

A

dryness of eyes and mouth (sicca symptoms) - keratoconjunctivitis sicca arthralgia belpharitis (eyes swollen and red) tooth decay dry cough fatigue vaginal dryness parotid gland swelling

29
Q

how is ocular dryness confirmed for patients with sjorgens

A

schirmers test

30
Q

what antibodies are positive in sjorgens

A

anti-SSA (Ro) anti-SSB (La) Raised IgG

31
Q

sjorgens must have 4 of the american european consensus group to be diagnosed; what are these

A

occular symptoms - dry eyes for 3 months, use of tear substitutes, schirmers test performed without anaesthesia (<5mm in 5 mins) oral symptoms - dry mouth, swollen salivary glands, abnormal salivary scintigraphy or parotid sialography findings (unstimulated salivary flow <1.5mm in 15 mins) positive minor salivary gland biopsy findings positive antibodies

32
Q

how is sjorgens treated

A

symptomatic lubricating eyedrops regular dental care pilocapine can stimulate saliva production (SE: flushing) hydroxychloroquine for arthralgia and fatigue other immunosuppression only useful in organ involvement eg ILD

33
Q

what is systemic sclerosis

A

systemic connective tissue disease where there is vasomotor disturbances (raynauds), fibrosis and atrophy of skin and subcutaneous tissue

34
Q

what is it that causes the skin and internal organ changes in systemic sclerosis

A

collagen deposition calcium deposits in skin

35
Q

the limited symptoms of scleroderma are referred to as a CREST, what does this stand for

A

calcinosis - calcium deposits in skin raynauds - spasm of vessels in response to cold or stress eosophageal dysfunction - acid reflux sclerodactyly - thickening and tightening of skin on fingers and hands telangiectasis - dilation of capillaries causing red mark on surface of skin

36
Q

what is the limited form (lsCC) of systemic sclerosis

A

skin involvement - face, hands, forearms, feet organ involvement tends to occur later anti-centromere antibody association (ACA)

37
Q

what is the diffuse form (dsCC) of systemic sclerosis

A

skin changes more rapid and involve trunk early significant organ involvement anti-Scl-70 antibody association

38
Q

what is the three phases of cutaneous involvement in systemic sclerosis

A

oedematous, indurative, atrophic

39
Q

what are major features of cutaneous involvement in systemic sclerosis

A

centrally located skin sclerosis affects arm, face and/or neck

40
Q

what are the minor features of cutaneous involvement in systemic sclerosis

A

sclerodactyly and atrophy of fingertips bilateral lung fibrosis

41
Q

how is systemic sclerosis diagnosed

A

when patient has 1 major or 2 minor features anti-centromere and anti-scl-70

42
Q

how is systemic sclerosis treated

A

no overall, needs to be tailored to specific issue

43
Q

how would raynauds in systemic sclerosis be treated

A

CCB (amlodipine, nifedipine, verapamil) Iloprost Bosentan

44
Q

how is renal involvement in systemic sclerosis treated

A

ACE inhibitors

45
Q

how is GI involvement in systemic sclerosis treated

A

PPI

46
Q

how is ILD in systemic sclerosis treated

A

immunosuppression usually with cyclophosphamide (chemo)

47
Q

what organs can become involved in systemic sclerosis

A

lung - pulmonary hypertension, fibrosis accelerated hypertension leading to renal crisis gut - dysphagia, malabsorption, bacterial overgrowth inflammatory arthritis and myositis may be seen

48
Q

what is generally responsible for death in systemic sclerosis patients

A

renal and lung changes pulmonary hypertension leads to 12% systemic sclerosis related deaths

49
Q

what is mixed connective tissue disease (MCTD)

A

defined condition which features symptoms also seen in other connective tissue diseases

50
Q

what symptoms may be seen in MCTD

A

raynauds arthralgia arthritis myositis sclerodactyly (thickening of skin on fingers and toes) pulmonary hypertension ILD

51
Q

how is MCTD diagnosed

A

anti-RNP antibodies

52
Q

what should take place after diagnosis of MCTD

A

annual echocardiograms suggested due to risk of pulmonary hypertension screening for ILD with PFTs should also take place

53
Q

how is MCTD treated

A

as with other CTDs - symptomatic eg CCB for raynauds or immunosuppression for significant muscle or lung disease

54
Q

what is anti phospholipid syndrome

A

disorder which manifests clinically as recurrent venous or arterial thrombosis and/or foetal loss

55
Q

although some patients have no associated disease, what diseases can anti phospholipid syndrome be associated with

A

SLE or another rheumatic or autoimmune disorder

56
Q

what are symptoms of anti phospholipid syndrome

A

migraine livedo reticularis (mottled skin) late, spontaneous foetal loss (2nd or 3rd trimester) common recurrent early foetal loss (<10 weeks) also possible

57
Q

how does anti phospholipid syndrome contribute to increased frequency of stroke or MI especially in younger patients

A

stroke may develop in situ thrombosis or embolisation that originates from valvular lesions or Libman-Sacks endocarditis recurrent pulmonary emboli or thrombosis can lead to life threatening pulmonary hypertension

58
Q

how is anti phospholipid syndrome diagnosed

A

may be thrombocytopenia and prolongation of APTT lupus anticoagulant anti-cardiolipin antibodies anti-beta 2 glycoprotein

59
Q

how is antiphospholipid syndrome treated

A

anticoagulation with LMWH patients who are found to have positive antibody but who never had thrombosis do not require anticoagulation

60
Q

why do you not give warfarin in antiphosphlipid syndrome

A

it is teratogenic

61
Q

what is complication of antiphospholipid syndrome

A

catastrophic APS is a rare, serious and fatal manifestation characterised by multi organ infarction over a period of days to weeks

Musculoskeletal (55 decks)

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