Multisystem Disorders Flashcards

1
Q

Yellow flags for chronic pain

A

Belief that pain and activity are harmful
Sickness behaviours such as extended rest
Social withdrawal
Emotional problems such as low mood, anxiety or stress
Problems or dissatisfaction at work
Problems with claims for compensation or time off work
Overprotective family or lack of support
Inappropriate expectations of treatment e.g. low active participation in treatment

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2
Q

Amyloidosis

A

Group of disorders characterised by EC deposits of a protein in abnormal fibrillar form, resistant to degradation
NB Amyloid deposition is also a feature of Alzheimer’s disease T2DM and haemodialysis-related amyloidosis

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3
Q

AL amyloid (primary amyloidosis)

A

Proliferation of plasma cell clone leads to amyloidogenic monoclonal immunoglobulins and fibrillar light chain protein deposition, causing organ failure

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4
Q

Features of primary amyloidosis

A

Kidneys: glomerular lesions (proteinuria, nephrotic syndrome)
Heart: restrictive cardiomyopathy, arrhythmias, angina
Nerves: peripheral and autonomic neuropathy, carpal tunnel syndrome
Gut: macroglossia, malabsorption/weight loss, perforation, haemorrhage, obstruction, hepatomegaly
Vascular: purpura, esp periorbital (CHARACTERISTIC feature)

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5
Q

Mx of primary amyloidosis

A

Optimise nutrition

Oral melphalan + prednisolone

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6
Q

Prognosis of amyloidosis

A

Primary: 13 months (17 if melphalan and prednisolone)

In general, 1-2 years

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7
Q

AA amyloid (secondary amyloidosis)

A

Amyloid is derived from serum amyloid A (acute phase protein), reflecting chronic inflammation in RA, IBD, chronic infections

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8
Q

Dx of amyloidosis

A

Biopsy (rectum or SC fat): Congo Red staining

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9
Q

AI connective tissue diseases

A
SLE
Systemic sclerosis
Sjogren's syndrome
Idiopathic inflammatory myopathies (e.g. myositis)
Mixed connective tissue disease
Relapsing polychondritis
Behcet's disease
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10
Q

Systemic sclerosis

A

Features scleroderma (skin fibrosis) and vascular disease

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11
Q

CREST features

A
Calcinosis
Raynaud's
Esophageal and gut dysmotility
Sclerodactyly
Telangiectasia
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12
Q

Mx of systemic sclerosis

A

No cure
Immunosuppressant
Monitor BP and renal function

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13
Q

Mixed connective tissue disease

A

Features of systemic sclerosis, SLE and polymyositis

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14
Q

Disorders which are RhF +ive

A
RA
Sjogren's syndrome
Mixed connective tissue disease
SLE
Can be +ive in normal individuals
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15
Q

Disorders which are anti-CCP +ive

A

RA

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16
Q

Disorders which are ANA +ive

A
SLE
AIH
Sjogren's syndrome
SLE
RA
17
Q

Disorders which are anti-dsDNA +ive

A

SLE (not very sensitive but highly specific)

18
Q

Disorders which are anti-phospholipid Ab +ive

A

Anti-phospholipid syndrome

SLE

19
Q

Disorders which are anti-Ro +ive

A

SLE
Sjogren’s syndrome
Systemic sclerosis

20
Q

Disorders which are anti-La +ive

A

Sjogren’s syndrome

SLE

21
Q

Disorders which are anti-Jo1 and anti-Mi2 +ive

A

Polymyositis

Dermatomyositis

22
Q

Disorders which are AMA +ive

A

PBC
AIH
Idiopathic cirrhosis

23
Q

Disorders which are anti-SMA +ive

A

AIH
PBC
Idiopathic cirrhosis

24
Q

Disorders which are anti-GBM +ive

A

Goodpasture’s disease

25
Q

Disorders which are cytoplasmic (c-)ANCA +ive

A

Wegener’s granulomatosis
Microscopic polyangiitis
Polyarteritis nodosa

26
Q

Disorders which are perinuclear (p-)ANCA +ive

A

Microscopic polyangiitis
Churg-Strauss
Some pulmonary-renal vasculitides

27
Q

Other disorders which may be ANCA +ive

A
IBD
PSC
AIH
RA
SLE
Drugs (e.g. antithyroid, allopurinol, ciprofloxacin)
28
Q

Features of SLE

A
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Non-erosive arthritis
Serositis
Renal disorder: proteinuria, casts
CNS disorder: seizures, psychosis
Haematological disorder: haemolytic anaemia, leukopenia, lymphopenia, thrombocytopaenia
Immunological disorder: anti-dsDNA, anti-Sm, antiphospholipid, ANA
29
Q

Drug-induced SLE

A
Isoniazid
Hydralazine
Procainamide
Quinidine
Chlorpromazine
Minocycline
Phenytoin
30
Q

Antiphospholipid syndrome

A

May be associated with SLE, more commonly a primary disorder

CLOTS: Coagulation defect, Livedo reticularis, Obstetric (e.g. miscarriage), Thrombocytopaenia

31
Q

Mx of antiphospholipid syndrome

A

Low-dose aspirin, or warfarin if recurrent thromboses