Multisystem Disorders

Question 1

Yellow flags for chronic pain

Answer 1

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

Question 2

Amyloidosis

Answer 2

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

Question 3

AL amyloid (primary amyloidosis)

Answer 3

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

Question 4

Features of primary amyloidosis

Answer 4

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)

Question 5

Mx of primary amyloidosis

Answer 5

Optimise nutrition

Oral melphalan + prednisolone

Question 6

Prognosis of amyloidosis

Answer 6

Primary: 13 months (17 if melphalan and prednisolone)

In general, 1-2 years

Question 7

AA amyloid (secondary amyloidosis)

Answer 7

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

Question 8

Dx of amyloidosis

Answer 8

Biopsy (rectum or SC fat): Congo Red staining

Question 9

AI connective tissue diseases

Answer 9

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

Question 10

Systemic sclerosis

Answer 10

Features scleroderma (skin fibrosis) and vascular disease

Question 11

CREST features

Answer 11

Calcinosis
Raynaud's
Esophageal and gut dysmotility
Sclerodactyly
Telangiectasia

Question 12

Mx of systemic sclerosis

Answer 12

No cure
Immunosuppressant
Monitor BP and renal function

Question 13

Mixed connective tissue disease

Answer 13

Features of systemic sclerosis, SLE and polymyositis

Question 14

Disorders which are RhF +ive

Answer 14

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

Question 15

Disorders which are anti-CCP +ive

Answer 15

RA

Question 16

Disorders which are ANA +ive

Answer 16

SLE
AIH
Sjogren's syndrome
SLE
RA

Question 17

Disorders which are anti-dsDNA +ive

Answer 17

SLE (not very sensitive but highly specific)

Question 18

Disorders which are anti-phospholipid Ab +ive

Answer 18

Anti-phospholipid syndrome

SLE

Question 19

Disorders which are anti-Ro +ive

Answer 19

SLE
Sjogren’s syndrome
Systemic sclerosis

Question 20

Disorders which are anti-La +ive

Answer 20

Sjogren’s syndrome

SLE

Question 21

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

Answer 21

Polymyositis

Dermatomyositis

Question 22

Disorders which are AMA +ive

Answer 22

PBC
AIH
Idiopathic cirrhosis

Question 23

Disorders which are anti-SMA +ive

Answer 23

AIH
PBC
Idiopathic cirrhosis

Question 24

Disorders which are anti-GBM +ive

Answer 24

Goodpasture’s disease

Question 25

Disorders which are cytoplasmic (c-)ANCA +ive

Answer 25

Wegener's granulomatosis Microscopic polyangiitis Polyarteritis nodosa

Question 26

Disorders which are perinuclear (p-)ANCA +ive

Answer 26

Microscopic polyangiitis Churg-Strauss Some pulmonary-renal vasculitides

Question 27

Other disorders which may be ANCA +ive

Answer 27

``` IBD PSC AIH RA SLE Drugs (e.g. antithyroid, allopurinol, ciprofloxacin) ```

Question 28

Features of SLE

Answer 28

``` 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 ```

Question 29

Drug-induced SLE

Answer 29

``` Isoniazid Hydralazine Procainamide Quinidine Chlorpromazine Minocycline Phenytoin ```

Question 30

Antiphospholipid syndrome

Answer 30

May be associated with SLE, more commonly a primary disorder | CLOTS: Coagulation defect, Livedo reticularis, Obstetric (e.g. miscarriage), Thrombocytopaenia

Question 31

Mx of antiphospholipid syndrome

Answer 31

Low-dose aspirin, or warfarin if recurrent thromboses