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My MRCP > Rheum: SLE, CTD > Flashcards

Rheum: SLE, CTD Flashcards

1
Q

What is the cause of discoid lupus?

A

Follicular keratin plugs

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2
Q

What is the treatment of discoid lupus?

A

Topical steroids –> hydroxychloroquine

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3
Q

Which antibodies are most specific for SLE?

A

Anti-SM

Anti-dsDNA

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4
Q

Which antibody is most sensitive for SLE

A

ANA

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5
Q

Why are C3 and C4 low during active lupus?

A

Formation of immune complexes causes consumption of complement

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6
Q

What is the pathogenesis of SLE?

A

Dysregulation of IgD

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7
Q

What is the treatment of SLE?

A

Hydroxychloroquine maintenance therapy
Internal organ involvement –> AZA, CYC, ritux
Prednisolone to induce remission

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8
Q

Which inflammatory markers are used to monitor SLE?

A

ESR

As CRP may be normal during active disease; if raised may indicate underlying infection

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9
Q

Apart from ESR, how else is SLE monitored?

A

Low complement

Anti-dsDNA titres

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10
Q

What antibody is associated specifically with neonatal lupus erythematosus?

A

Anti-Ro

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11
Q

What are the complications/features of congenital lupus?

A

Heart block
Skin rashes
Hepatosplenomegaly

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12
Q

What percentage of psoriasis patients develop arthritis?

A

10-20%

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13
Q

What are the 5 types of psoriatic arthritis?

A 
Symmetrical (similar to RA)
Asymmetrical oligo
Sacroilitis
DIP joint disease
Arthritis mutilans
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14
Q

What are other signs of psoriatic arthropathy?

A

Tenosynovitis
Dactylitis
Enthesitis
Nail pitting and onycholysis

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15
Q

What are the X-Ray appearances of psoriatic arthritis?

A

Co-existence of erosive changes and new bone formation
Periostitis
Pencil in cup appearance

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16
Q

What is rheumatoid factor?

A

IgM against IgG

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17
Q

Which HLA is RA associated with?

A

DR4

Especially Felty’s syndrome

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18
Q

What is Felty’s syndrome?

A

RA
Splenomegaly
Neutropaenia

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19
Q

What are the X-Ray features of RA?

A

Loss of joint space
Juxta-articular osteoporosis
Soft tissue swelling
Late - periarticular erosions, subluxation

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20
Q

What percentage of normal people have +ve HLA-B27?

A

10%

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21
Q

What percentage of ankylosing spondylitis patients have +ve HLA-B27?

A

90%

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22
Q

What is Schober’s sign?

A

An increase of less than 5cm on anterior spinal flexion when measuring the distance from 10cm above and 5cm below the left posterior iliac spine

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23
Q

What are the X-Ray features of ankylosing spondylitis?

A

Bamboo spine
Sacroilitis (subchondral erosions, sclerosis of vertebral corners)
Squaring of vertebrae
Syndesmophytes

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24
Q

What is bamboo spine?

A

Ossification of outer fibres of annulus fibrosus

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25
What are the complications of ankylosing spondylitis?
``` Apical fibrosis Anterior uveitis Aortic regurgitation Achilles tendonitis AV node block Amyloidosis ```
26
What is the treatment of ankylosing spondylitis?
Exercise and physio NSAIDs DMARDS if peripheral joint involvement TNF inhibitors - do not improve radiological progression
27
What is the most common organism causing septic arthritis?
S.aureus Young sexually active adults - neisseria gonorrhoea
28
What is the cause of reactive arthritis?
Post STI form most common form in men, post-dysenteric form equal sex incidence Shigella flexneri, salmonella typhi/enteritidis, yersinia, campylobacter, chlamydia trachomaatis
29
What is the triad of reactive arthritis?
Urethritis Conjunctivitis/anterior uveitis Arthritis - asymmetrical, lower limbs Also dactylitis, circinate balanitis, keratoderma blenorrrhagica
30
What is the prognosis of reactive arthritis?
Symptoms last 4-6 months | 25% have recurrent episodes, 10% chronic disease
31
In gout, where are the urate crystals deposited?
Superficial portions of the articular cartilage
32
What is the most common cause of gout?
Decreased excretion of uric acid - diuretics, CKD, lead toxicity
33
What are other causes of gout?
Increased production of uric acid - myeloproliferative disorder, cytotoxic drugs, severe psoriasis Lesch-Nyhan syndrome
34
What are the X-Ray features of gout?
``` Joint effusion "Punched out" erosions with sclerotic margins in a juxta-articular distribution, often with overhanging edges Eccentric erosions No periarticular osteopenia Soft tissue tophi ```
35
What is the double contour sign in gout?
Hyperechoic irregular band over the superficial margin of the joint cartilage, produce by deposition of monosodium urate crystals on the surface of the hyaline cartilage
36
Where do calcium pyrophosphate crystals deposit in pseudogout?
The middle layer of the hyaline cartilage, parallel to the bony cortex as a hyper-echoic irregular line embedded in the cartilage (with a normal hyaline cartilage surface)
37
What are risk factors for pseudogout if it develops at a younger age?
``` Haemochromatosis Hyperparathyroidism Low magnesium Low phosphate Acromegaly Wilson's disease ```
38
What is seen on joint aspiration in pseudogout?
Weakly positive birefringent rhomboid shaped crystals
39
What are the 3 types of systemic sclerosis?
Limited cutaneous Diffuse cutaneous Scleroderma
40
What are the features of limited cutaneous systemic sclerosis?
Raynaud's may be first sign Scleroderma of face an distal limbs Subtype: CREST syndrome (calcinosis, Raynaud's, oesophageal dysmotility, sclerodactyly, telangiectasia)
41
What are the features of diffuse cutaneous systemic sclerosis?
Scleroderma of trunk and proximal limbs | Respiratory (ILD and PAH) involvement, renal disease
42
Which antibodies are associated with systemic sclerosis?
Limited cutaneous: ACA Diffuse cutaneous: Anti-Scl-70 Both: ANA in 90%, RF in 30%
43
What are the features of antiphospholipid syndrome?
Venous and arterial thombosis Recurrent foetal loss Thrombocytopenia Livedo reticularis
44
Why is APTT high in APS?
Ex vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade
45
What is the treatment of antiphospholipid syndrome?
Primary thromboprophylaxis - low dose aspirin Secondary thromboprophylaxis - lifelong warfarin, target INR 2-3 Recurrent VTE on warfarin - add low dose aspirin, target INR 3-4
46
What are the features of dermatomyositis?
Heliotrope rash around the eyelids Gottron's papules Fatigue and weakness
47
Which antibodies are associated with dermatomyositis?
ANA most common (60%) | Anti-Mi2 most specific (only seen in 25%)
48
How is dermatomyositis diagnosed?
Elevated CK EMG Muscle biopsy
49
What is Sharp's syndrome?
Mixed connective tissue disease
50
What are the features of mixed connective tissue disease?
Raynaud's Polyarthralgia Myalgia Dactylitis Also photosensitive rash, scleroderma-like changes, oesophageal dysfunction, pleurisy/PAH/ILD, glomerulonephritis
51
Which antibodies are positive in mixed connective tissue disease?
Anti-U1 ribonucleotide antibodies | ANA
52
What is Raynaud's disease?
Exaggerated vasoconstrictive response of the digital arteries and cutaneous arteriole to the cold or emotional stress
53
What are the secondary causes of Raynaud's?
``` CTDs Leukaemia Type 1 cryoglobulinaemia Vibrating tools COCP Cervical rib ```
54
Which features of Raynaud's suggest underlying CTDs?
Onset after 40 Unilateral Rash, digital ulcers, calcinosis Autoantibodies
55
What is the management of Raynaud's?
CCB - nifedipine | 2nd line IV prostacyclin infusion (lasts several weeks/months)
56
What is the cause of Marfan's syndrome?
Mutation on FBN1 gene on chromosome 15 that codes for the protein fibrillin 1.
57
What are the eye symptoms of Marfan's?
Supero-temporal ectopia lentis Blue sclera Myopia
58
What is dural ectasia?
Ballooning of the dural sac at the lumbrosacral level 60% Marfan's patients Lower back pain, headaches, bladder and bowel dysfunction
59
What are the cardiac symptoms of Marfan's?
MV prolapse | Aortic dissection
60
What is the management of Marfan's?
Regular ECHOs | BB/ACEIs
61
What is the cause of polymyositis?
T cell mediated cytotoxic process against muscle fibres Idiopathic, CTD, malignancy
62
How is polymyositis diagnosed?
Elevated CK, LDH, aldolase, AST, ALT EMG Muscle biopsy
63
Which antibodies are associated with polymyositis?
Anti-synthetase | Anti-Jo 1 (associated with more severe form associated with lung involvement)
64
What is polyarteritis nodosa?
Vasculitis of medium sized arteries leading to aneurysm formation
65
What is PAN associated with?
Hepatitis B | Middle aged men
66
What are the features of PAN?
``` Fever etc Mononeuritis multiplex Hypertension Livedo reticularis Haematuria and renal failure ```
67
Which antibody is associated with PAN?
pANCA in 20%
68
What is familial mediterranean fever and who gets it?
Autosomal recessive Presents in 2nd decade Turkish, Armenian, Arabic descent
69
What are the features of FMF?
``` Pyrexia Abdo pain from peritonitis Pleurisy Pericarditis Arthritis Erysipeloid rash ```
70
What is the management of FMF?
Colchicine
71
What is the cause and pathophysiology of Langerhans Cell Histiocytosis?
Abnormal proliferation of dendritic cells/histiocytes Formation of multiple granulomas
72
What are the features of Langerhans Cell Histiocytosis?
Bone pain (skull or proximal femur) Rash Recurrent otitis media/mastoiditis
73
What is seen on electron microscopy in LCH?
Tennis racket shape Birbeck granules
74
What are the features of Still's disease?
Arthralgia Swinging pyrexia that peaks early evening Salmon pink maculopapular rash Lymphadenopathy
75
What is the diagnostic criteria of Still's disease?
Yamaguchi
76
How is Still's disease diagnosed?
RF and ANA negative | Elevated ferritin
77
What is osteopetrosis?
Failure of osteoclastic bone resorption --> cortical thickening and sclerosis --> thick and brittle bones Autosomal dominant
78
What are the features of osteopetrosis?
Fractures
79
How is osteopetrosis diagnosed?
XR: symmetrical and diffuse skeletal sclerosis - stone bone Normal Ca, phosphate, ALP, PTH
80
What is the cause of osteogenesis imperfecta?
Autosomal dominant inheritance of mutation in gene coding for type 1 collagen Decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides
81
What are the features of osteogenesis imperfecta?
Fractures Blue sclerae Deafness secondary to otosclerosis Dental imperfections
82
What types of collagen form cell surfaces and hairs, and the basal lamina?
Cell surfaces, hairs - Collagen type 5 - Classic Ehlers-Danlos Basal lamina - Type 4 - Alport's and Goodpasture's syndrome
83
What is the cause of Paget's disease?
Excessive osteoclastic resorption followed by increased osteoclastic activity - uncontrolled bone turnover
84
How is Paget's disease diagnosed?
Normal Ca, PO4, PTH Isolated raised ALP
85
What is the treatment of Paget's disease?
Bisphosphonates
86
What are the features of spinal stenosis?
Gradual onset uni/bilateral leg pain, with or without back pain Numbness and weakness Worse on walking, resolves when sitting/leaning forwards/crouching down Normal examination
87
What is osteomalacia?
Softening of the bones secondary to low vitamin D
88
What are the symptoms of osteomalacia?
Bone pain Fractures Proximal myopathy
89
How is osteomalacia diagnosed?
High ALP Low calcium and phosphate Translucent bands (Looser's zones) on X-Ray
90
What are the complications of Paget's disease?
``` Cranial nerve entrapment Bone sarcoma Fractures Skull thickening High output cardiac failure ```
91
What are the risk factors for avascular necrosis of the hip?
Long term steroid use Chemotherapy Alcohol excess Trauma
92
What is seen on X-Ray in avascular necrosis of the hip?
Osteopenia Microfractures Crescent sign (collapse of the articular surface)
93
How do bisphosphonates work?
Analogues of pyrophosphate, a molecular which decreases demineralization in bone Inhibits osteoclasts by reducing recruitment and promoting apoptosis
94
What are 3 side effects of bisphosphonates?
Oesophagitis Osteonecrosis of the jaw atypical stress fractures of the proximal femoral shaft
95
What type of retinopathy is seen in hydroxychloroquine use?
Bull's eye
96
How does sulfasalazine work?
Prodrug for 5-ASA | Decreases neutrophil chemotaxis, suppresses proliferation of lymphocytes and pro-inflammatory cytokines
97
In which patients should use of sulfasalazine be avoided?
G6P deficiency | Allergy to aspirin/sulphonamides (cross-sensitivity)
98
What are 6 side effects of sulfasalazine?
``` Oligospermia Stevens-Johnson syndrome Pneumonitis Myelosuppression Heinz body/megaloblastic anaemia Coloured tears ```
99
What are the side effects of leflunomide?
``` Diarrhoea Hypertension WL Peripheral neuropathy Myelosuppression Pneumonitis ```
100
What are the rules when stopping leflunomide?
Wash out period of a year | Requires co-administration of cholestyramine
101
In what condition are anti-yo antibodies seen?
Patients with breast cancer and paraneoplastic syndrome | Associated with cerebellar degeneration and ataxia
102
What is DeQuervain's tenosynovitis?
Sheath containing the extensor pollicis brevis and abductor pollicis longus tendons is inflamed
103
What are the features of De Quervain's tenosynovitis?
Pain on radial side of wrist Tenderness over radial styloid process Painful abduction of thumb against resistance Finkelstein's test - examiner pulls thumb in ulnar deviation and longitudinal traction - causes pain over radial styloid process
104
Which nerve is compressed in carpal tunnel syndrome?
Median
105
What are 6 causes of carpal tunnel syndrome?
``` Pregnancy Lunate fracture RA Hypothyroid Acromegaly Idiopathic ```
106
What is seen on examination in Carpal Tunnel syndrome?
Tinel's and Phalen's sign Weakness of thumb abduction (abductor pollicis longus) Wasting of thenar eminence
107
What is surgical decompression of Carpal tunnel syndrome?
Division of flexor retinaculum
108
What are the general features of prolapsed disc?
Dermatomal leg pain associated with neurological deficits Leg pain > back pain Pain worse when standing
109
Which vertebral disc is prolapsed when a positive femoral stretch test is seen, with weak quadriceps, reduced knee reflex, and sensory loss over anterior knee?
L3 or L4
110
What is seen in L5 disc prolapse?
Sensory loss over dorsum of foot Weakness in foot and big toe dorsiflexion Reflexes intact Positive sciatic nerve stretch test
111
What is seen in S1 disc prolapse?
Positive sciatic nerve stretch test Weakness in plantar flexion of foot Reduced ankle reflex Sensory loss over posterolateral aspect of leg and lateral aspect of foot
112
What level of rheumatoid factor and ANA titre is significant?
>1:160 | >1:80
113
What skin lesions are seen in Behcet's disease?
Erythema nodosum Acneiform Migratory thrombophlebitis Vasculitic
114
What is reflex sympathetic dystrophy?
Complex regional pain syndrome Disproportionate pain secondary to injury/surgery Skin changes from autonomic dysfunction

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