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Rheumatology Flashcards

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1
Q

Pathophysiology of JIA?

A

Inflammation of the synovial lining of the joint, leading to joint destruction through progressive erosion of articular cartilage and bone

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2
Q

what are the 5 main subtypes of JIA?

A 
Oligoarticular 
Polyarticular
Systemic 
Enthesis-related 
Psoriatic
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3
Q

how does oligoarticular JIA present?

A 
1-4 medium and large joints in the first six months
Asymmetrical
F>M
2-3yrs
May have associated uveitis
non-destructive arthritis
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4
Q

how does polyarticular JIA present?

A 
5+ joints in the first six months
2-3yrs, 10-13yrs
symmetrical usually 
may have additional systemic features
destructive arthritis
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5
Q

how does systemic arthritis present?

A 
any number of joints 
daily high fevers
salmon pink rash with the fever
hepatosplenomegaly 
lymphadenopathy 
serositis (pericarditis, pleuritis, peritonitis)
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6
Q

how does psoriatic arthritis present?

A

arthritis plus at least two of:

dactilytis, nail pitting or onycholysis, psoriasis in first degree relative

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7
Q

what is enthesitis-related JIA and how does it present?

A 
arthritis plus inflammation at the site of a tendon/ligament insertion site 
sacroiliac or lumbosacral pain 
HLA B27 positive 
FHx
acute anterior uveitis `
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8
Q

what are some tests for JIA?

A 
usually a clinical diagnosis 
ANA+VE = uveitis more likely 
normocytic anaemia 
raised WBC
ESR/CRP
HLA B27 (classify enthesitis related JIA) 
USS- show joint fluid
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9
Q

what is the management of JIA?

A 
NSAID's
intra-articular steroid injections for affected joints
topical steroids for eye involvement 
methotrexate, adalimumab = first line 
sulfasalazine, etanercept = second line
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10
Q

what are some complications of JIA?

A

joint deformities
uveitis (usually asymptomatic) can lead to blindness, cataracts or glaucoma
osteoporosis
growth restriction
psychosocial, behavioural and educational difficulties

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11
Q

pathophysiology of rheumatoid arthritis?

A

inflammation of the synovium causes a pannus formation (group of granulation tissue), which blocks the normal route of nutrition causing the cartilage to thin and deformities to form

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12
Q

presentation of RA?

A

warm and swollen joints (DIP spared)
stiffness worse in morning or period of inactivity, wears off in 30 mins or less
symmetrical
painful

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13
Q

signs of RA?

A

Boutonniere
Z thumb
Ulnar deviation

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14
Q

investigation of RA?

A 
XRAY LESS: loss of joint space, erosions, soft tissue swelling,  subchondral cyst
RF 
Anti-CCP 
ANA
CRP/ESR
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15
Q

Management of RA?

A

regular exercise + lifestyle advice
first line- DMARD (methotrexate, cyclophosphamide)
second line- TNF alpha inhibitors - infliximab, adalimumab

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16
Q

Pathophsyiology of reactive arthritis?

A

Inflammation following un-related infection through molecular mimicry

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17
Q

which organisms most commonly cause reactive arthritis?

A

GI: campylobacter pylori, salmonella, shigella
GU: chlamydia trachomatis

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18
Q

presentation of reactive arthritis?

A

urethritis/balanitis, conjunctivitis/anterior uveitis, arthritis = triad
+ oral ulcers, plantar fasciitis, dactylitis, keratoderma

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19
Q

investigations for reactive arthritis?

A

stool sample
sexual health screen
bloods- ESR/CRP

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20
Q

management of reactive arthritis?

A

NSAID’s
intra-articular steroid injections
methotrexate and sulfasalazine if persistent

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21
Q

pathophysiology of ankylosing spondylitis?

A

chronic inflammatory disorder associated with HLA-B27 of the sacro-iliac joints and axial skeleton

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22
Q

features of ank spond?

A 
young man with lower back pain and stiffness 
worse in morning 
improves with exercise 
A's: 
apical fibrosis 
anterior uveitis 
aortic regurgitation 
achilles tendonitis 
AV node block 
amyloidosis
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23
Q

what test is used to assess severity of ank spond?

A

Schobers test - line drawin 10cm above and 5cm below the back dimples. distance between the two lines are measured, and if increase by less than 5cm when patient bends forward then suggests ank spond

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24
Q

investigations for ank spond

A

Schobers test
HLA-B27 testing
XRAY (most useful)- sacroiliitis (subchondral erosions), bamboo spine, squaring of lumbar vertebrae, syndesmophytes
Spirometry to assess chest expansion

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25
management of ank spond?
encourage regular exercise NSAIDs first line Anti-TNF - infliximab and adalimumab
26
what is the pathophysiology of gout?
abnormal purine synthesis leads to an increase in uric acid, leading to chronic deposition of monosodium urate crystals. Joint damage is caused by both deposition of the MSU crystals and inflammation.
27
Risk factors for gout?
``` male gender red meat seafood high alcohol intake CKD HTN obesity diuretics ```
28
symptoms of gout?
``` swollen tender erythematous joint comes on over 6-12 hour period most commonly first MTP also affects knee, wrists, ankles, elbows can have fever and malaise ```
29
signs of gout?
``` synovitis, erythematous joint gouty tophi (chronic tophaceous gout) ```
30
investigations of gout?
often clinical diagnosis joint fluid microscopy - under polarizing light microscopy there are needle shaped negatively birefringent crystals serum urate levels serum urea, creatinine, eGFR
31
Management of gout?
NSAID's (naproxen, diclofenac)- acute management of pain Colchicine allopurinol - started after the attack
32
MOA of allopurinol?
xanthine oxidase inhibitor
33
Pathophysiology of pseudogout?
deposition of calcium pyrophosphate cyrstals in hyaline and fibrocartilage
34
risk factors of pseudogout?
``` dehydration intercurrent illness hyperparathyroidism hypothyroidism wilsons dialysis ```
35
symptoms of pseudogout?
erythematous synovitis monoarticular or oligoarticular arhtiritis most commonly knees can affect wrists, shoulders, ankles, hands and feet
36
investigations of pseudogout?
joint aspiration- polarizing light microscopy shows rhomboidal weakly positive birefringent crystals
37
management of pseudogout?
``` no specific treatments treat underlying cause ice packs and rest aspiration of joint NSAIDS intra-articular steroid joint injections systemic steroids colchicine ```
38
pathophysiology of osteoarthritis?
degenerative condition where there is destruction of the articular cartilage, commonly as part of the reparitive process. There is loss of cartilage, remodelling of adjacent bone and associated inflammation.
39
risk factors for ostearthritis?
``` genetic ageing female sex obesity reduced muscle strength joint laxity joint malalignment occupational or recreational stresses on joints ```
40
symptoms of OA?
joint pain - exacerbated by exercise and relieved by rest short lived morning stiffness (<30 mins) reduced function night pain as disease progresses
41
signs of OA?
``` reduced joint movement bouchards nodes heberdens nodes crepitus swelling absence of systemic features ```
42
investigations of OA?
XRAY blood tests - should be normal joint aspiration - to exclude other causes MRI
43
XRAY findings of OA?
LOSS: Loss of joint space Osteophytes Subchondral cysts Subarticular sclerosis
44
management of OA?
lifestyle factors - exercise, weight loss, thermotherapy, advice on footwear, physio input, OT- bracing, joint supports pain relief- NSAID's, paracetamol Intra-articular corticosteroid injections joint surgery- arthroplasty, joint fusion, realignment
45
most common organisms of septic arthritis?
staphylococcus aureus neisseria gonorrhoea streptococcus IV drug users- pseudomonas
46
risk factors for septic arthritis?
``` increasing age DM joint surgery skin infection immunodeficiency ```
47
symptoms of septic arthritis?
``` single swollen joint effusion reduced movement fevers rigors pain children - localising signs usually absent, often presents with fever, joint pain and unillingness to move the joint i.e. limp ```
48
investigations of septic arthritis?
ESR + CRP increased joint aspiration and culture- synovial fluid shows high leukocyte count and grows bacteria At least two blood cultures to exclude bactereamia
49
management of septic arthritis?
treat immediately - do not wait for synovial fluid gram stain or cultures to return IV antibiotics to cover both S.aurues and Strep- Flucloxacillin 4-6 weeks Vancomycin if MRSA suspected Cefotaxime if neisseria gonorrhea surgical drainage if infected joint does not respond to medical therapy
50
pathophysiology of psoriatic arthritis?
exact cause unknown | HLA-B27 thought to mediate the inflammation of the joints and the skin
51
presentation of psoriatic arthritis?
presents with either psoarisis or arthritis first nail pitting onikolysis psoriatic plaques
52
pathophysiology of raynauds disease?
vascular and neural abnormalities lead to intense vasoconstriction, followed by cyanosis and rapid reperfusion
53
symptoms of raynauds?
``` initial white phase (vasoconstriction) blue phase (cyanosis) red phase (rapid reperfusion) may have accompanying- tingling, numbness, burning ```
54
management of raynauds?
avoid cold provocation - always wear gloves stop smoking vasodilators - Ca channel blocker such as nifedipine
55
what is polymyositis/dermatomyositis?
both are idiopathic inflammatory myopathies- a group of chronic autoimmune conditions that primarily affect the proximal muscles and are characterised by inflammation of the muscles
56
presentation of polymyositis?
steady progression of weeks to months diffuse weakness in proximal muscles to distal muscles difficulty rising from low chair, climbing steps etc. fatigue myalgia not usually painful muscle atrophy
57
investigations of polymyositis?
creatinine kinase - vastly elevated anti-Jo-1 antibodies LDH elevated
58
symptoms of dermatomyositis?
same pattern of weakness as polymyositis, but with additional features: rash - blue discolouration of upper eyelids, flat red rash involving face and upper trunk and raised purple scaly patches over extnsor surfaces of joints and fingers systemic features- fever, arthalgia, malaise, weight loss possible cardiac disease gastrointestinal ulcers
59
investigations of dermatomyositis?
creatinine kinase not so reliable ANA positive anti-Mi-2 anitbodies muscle bopisy
60
management of dermatomyositis/polymyositis?
physio/OT- encourage exercise etc. 1st line- topical or systemic corticosteroids 2nd line - immunosupressive drugs - azathioprine + cyclophosphamide 2rd line- TNF alpha antagonist
61
what is osteomyelitis?
infection of the bone marrow which may spread to the bone cortex and periosteum via the Haversian canals. This results in inflammatory destruction of bone, necrosis and sequestration.
62
what is sequestration?
when dead bone becomes detached from healthy bone
63
what is the most common site of osteomyelitis in children?
distal femur and proximal tibia
64
what are the two main subtypes of osteomyelitis?
haematogenous - haematological bacterial seeding from a remote source direct - direct infection of bone following a surgical procedure or trauma
65
what are the most common pathogens of osteomyelitis?
``` S. aureus (including MRSA) Haemophilus influenza strep spp. E coli pseudomonas ```
66
risk factors for osetomyelitis?
``` trauma prosthetics diabetes peripheral arterial disease alcoholism IVDU immunosuppression - HIV/AIDS sickle cell disease ```
67
presentation of osteomyelitis?
fever pain localised over bone, exacerbated my movement immobile limb may be effusion over joint and local areas erythema
68
what is Potts disease?
vertebral osteomyelitis resulting from haematogenous spread of tuberculosis - there is damage to the bodies of two neighbouring vertebra this can lead to vertebral collapse
69
investigations for osteomyelitis?
FBC- rasied inflammatory markers blood clutlures bone cultures are gold standard MRI
70
management of osteomyelitis?
local bone and soft tissue debridement stabilisation of bone local antibiotic therapy - flucloxacillin or vancomycin if MRSA reconstruction of soft tissue
71
what is polyarteritis nodosa?
necrotising arteritis of medium or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries or venules
72
symptoms of polyarteritis nodosa?
``` fever malaise myalgia abdominal pain haematuria, proteinuria (renal) hep B infection ```
73
management of polyarteritis nodosa?
corticosteroid therapy addition of cyclophosphamide or azathioprine if relapse IVIG and apsirin in childhood PAN
74
symptoms of bechets disease?
recurrent oral ulceration (aphthous, and herpetiform) genital ulcers eye or skin lesions self-limiting mono or oligo arthritis accompanies GI symptoms - diarrhoea, abdominal pain, pulmonary and renal lesions
75
investigations for bechets?
skin prick test | ESR and CRP are high
76
treatment for bechets?
MDT approach | corticosteroids, colchicine, immunosuppresion agents, anti-TNF agents
77
what is the pathophysiology of SLE?
autoimmune connective tissue disorder where anti-nuclear antibodies are produced to proteins within the cell nucleus, causing widespread inflammation.
78
symptoms of SLE?
``` fatigue weight loss arthalgia myalgia fever photosensitive malar rash - butterfly shape across nose and cheek bones lymphadenopathy splenomegaly SOB mouth ulcers hair loss raynauds ```
79
investigations of SLE?
autoantibodies- ANA, anti-dsDNA (highly specific), anti-phospholipid FBC- normocytic anaemia of chronic disease C3+C4 - decreased CRP/ESR- increased renal biopsy urine protein:creatinine ratio
80
what are some complications of SLE?
``` pericarditis pleuritis infection anaemia of chronic disease lupus nephritis neuropsychiatric SLE (optic neuritis) venous thromboembolism ```
81
management of SLE?
first line- NSAIDs, steroids, hydroxychloroquine second line- methotrexate, MMF, azathioprine, tacrolimus third line- biological therapies i.e. rituximab
82
What is sjogrens syndrome?
autoimmune condition that affects the exocrine glands leading to dysfunctional mucous membranes
83
symptoms of sjogrens syndrome?
dry eyes dry mouth dry genitals (i.e. vagina)
84
what are the two types of sjogrens?
primary- condition occurs in isolation | secondary- condition occurs related to SLE or RA
85
investigations for sjogrens?
anti-bodies: anti-Ro and anti-La | Schirmer test
86
management of sjogrens?
``` artificial tears artificial saliva vaginal lubricants nsaids rituximab- biologics hydroxychloroquine to halt the progression of the disease ```
87
complications of sjogrens?
eye - conjunctivitis, corneal ulcers oral- dental cavities, candida vaginal - candidiasis, sexual dysfunction
88
pathophysiology of marfan's syndrome?
inherited connective tissue disorder caused by mutations in the genes encoding for fibrillin 1
89
pattern of inheritance for marfans syndrome?
autosomal dominant with almost complete penetrance
90
signs of marfans syndrome?
skin - striae CVS- aortic regurg/dilation, mitral valve prolapse lungs- pneumothorax skeletal- ductal ectasia, hypermobility, pectus excavatum, finger contractures, tall and thin with disproportionately long arms and legs compared to trunk facial- long face, high arched palatate
91
investigations of marfans?
ECHO (annaully) CVS CT(every 5 years) MRI of spine pelvic xray
92
management of marfans?
``` psychological support advised to avoid exertion at maximal capacity or contact sports prophylactic BB valve replacement if needed MDT input ```
93
what is ehlers-danlos syndrome?
group of inheritable connective tissue disorders that affect joint and skin mobility.
94
features of ED syndrome?
skin: increased elasticity and fragility (easy bruising and scarring) joints: hypermobile, spontaneous dislocations CVS: palpitations, dizziness intestinal: IBS, abdominal pain hearing: tinnitus urogynae: prolapse, obstetric complications systemic: tiredness, sleep disturbance pshyological: anxiety, depression
95
investigations of ED?
genetic testing | assay of enzyme deficiencies
96
management ED ?
physiotherapy psychological support pain relief celiprolol - beta-1 adrenoreceptor
97
what is systemic sclerosis/scleroderma?
autoimmune inflammatory and fibrotic connective tissue disease
98
what are the two types of systemic sclerosis?
limited cutaneous systemic sclerosis - CREST | diffuse cutaneous systemic sclerosis
99
what are the features of limited cutaneous systemic sclerosis?
``` C-calcinosis R-raynauds E-eosophageal dysmotility S- sclerodactyly T- telangiectasia ```
100
what are the features of diffuse cutaneous systemic sclerosis?
features of limited cutaneous systemic sclerosis plus internal organ involvement: CVS- HTN and coronary artery disease lung- pulmonary HTN + fibrosis kidney- glomerulonephritis
101
investigations of systemic sclerosis?
ANA anti-centromere antibodies - limited anti-Scl-70 - diffuse nail capillaroscopy
102
management of systemic sclerosis?
``` steroids immunosuppressants analgesia for pain avoid smoking gentle skin stretching regular emollients avoid cold triggers for raynauds physiotherapy for healthy joints occupational therapy ```
103
what is osteoporosis?
condition where there is a reduction in the density of the bones, making them more prone to fracutres
104
risk factors for osteoporosis?
``` older age female reduced mobility low BMI RA alcohol and smoking long term corticosteroid use post-menopausal women ```
105
what tool is used to assess someones osteoporosis risk?
FRAX tool - gives a prediction of the risk of a fragility fracture in the next 10 years
106
investigations for someone with suspected osteoporosis?
DEXA scan - to measure BMD (T score less than -2.5 indicates osteoporosis)
107
management of osteoporosis?
``` lifestyle - adequate calcium and vit D inake, stop smoking etc. bisphosphonates - first line second lines: denoxumab (monoclonal antibodiy) strontium ranelate raloxifene hormone replacement therapy ```
108
MOA of bisphosphonates?
reduce osteoclast activity, resulting in reduced reabsorption of bone
109
side effects of bisphosphonates?
reflux and oesophageal erosions atypical fractures osteonecrosis of the jaw and external auditory canal
110
features of vit D deficiency?
``` fatigue bone pain muscle weakness muscle aches pathological or abnormal fractures ```
111
investigations of vit D deficiency?
``` serum 25-hydroxyvitamin D low serum calcium low serum phosphate high alkaline phosphatase PTH high XRAY- osteopenia DEXA shows low bone mineral density ```
112
treatment of vit D deficiency?
colecalciferol supplement (maintenance dose 800 IU)
113
what is pagets disease of bone?
excessive bone turnover due to excessive activity of both osteoblasts and osteoclasts, leading to patchy areas of high density bone and low density. this results in enlarged and misshapen bones
114
presentation of pagets disease?
bone pain bone deformity pathological fractures hearing loss (can affect the bones of the ear)
115
investigations of pagets disease?
XRAY: bone enlargement and deformity, osteoporosis circumscripta, cotton wool appearance of skull raised ALP normal calcium and phosphate
116
management of pagets disease?
bisphosphonates NSAIDs for bone pain calcium and vit D supplementation surgery rarely
117
two main complications of pagets bone disease?
``` osteogenic sarcoma - type of bone cancer with very poor prognosis spinal stenosis (leading to SCC) ```
118
what are some extra-articular features of rheumatoid arthritis
``` amyloidosis scleritis, episcleritis raynauds carpal tunnel pulmonary fibrosis vasculitis ```
119
what is given with methotrexate and why?
folic acid - as it is depleted by the methorexate which could lead to anaemia
120
who is methotrexate contrainidcated in?
liver disease- as liver hepatotoxicity | prengnancy- teratogenic
121
what is seen on xray of gout?
normal joint space soft tissue swelling periarticular erosions
122
what are some common triggers of acute gout?
trauma alcohol surgery starting diuretics
123
what is the pathophysiology of antiphospholipid syndrome?
autoimmune condition where antiphospholipid antibodies react against proteins that bind to anionic phospholipids on plasma membranes
124
what are the features of antiphospholipid syndrome?
``` recurrent miscarriage DVT/arterial blood clots coagulation defects livedo reticularis low platelets ``` CLOT: cloagulation defects, livedo reticularis, Obstetric problems, Thrombocytopenia
125
investigations of antiphospholipid syndrome?
anticardiolipin antibodies | lupus anticoagulants
126
management of antiphospholipid syndrome?
warfarin heparin aspirin clopidogrel
127
what conditions have raynauds as a feature?
``` SLE RA dermatomyositis/polymyositis ED polycythemia ```
128
what are some ANCA -ve vasculitis?
HSP | good pastures disease
129
what is an example of a large vasculitis?
temporal arteritis | takayasus
130
what are some examples of medium vasculitis?
polyarteritis nodosa | kawasaki disease
131
what are some systemic conditions in which vasculitis is a feature of the disease?
infective endocarditis rheumatoid arthritis SLE IBD
132
what are some causes of mononeuritis multiplex?
``` HIV/AIDS rheumatoid arthritis diabetes sarcoidosis polyarteritis nodosa ```

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