Rheumatology Flashcards
Rheumatoid Arthritis
what is it
an autoimmune condition that causes chronic inflammation of the synovial lining of the joints, tendon sheaths and bursa
it is an inflammatory arthritis
symmetrical polyarthritis
Rheumatoid Arthritis
what does symmetrical polyarthritis mean
symmetrical and affects multiple joints
Rheumatoid Arthritis
epidemiology
F>M
middle age
Rheumatoid Arthritis
which gene is often present in RF positive patients
HLA DR4
Rheumatoid Arthritis
which gene is occasionally present in RA patients
HLA DR1
Rheumatoid Arthritis
antibodies
- Rheumatoid Factor (RF)
- Cyclic citrullinated peptide antibodies (anti-CCP antibodies)
Rheumatoid Arthritis
what is RF
an autoantibody that targets the Fc portion of the IgG antibody
which causes activation of the immune system against the patient’s own IgG causing systemic inflammation
RF is most often IgM but can be any class of immunoglobulin
Rheumatoid Arthritis
what are anti-CCP antibodies
Cyclic citrullinated peptide antibodies are autoantibodies that are more sensitive and specific to RA than RF
Rheumatoid Arthritis
Key symptoms
- symmetrical distal polyarthropathy
- joint pain, swelling and stiffness
- worse after rest but improves with activity
Rheumatoid Arthritis
systemic symptoms
- fatigue
- weight loss
- flu like illness
- muscle aches and weakness
Rheumatoid Arthritis
what is Palindromic Rheumatism
self limiting short episodes of inflammatory arthritis with joint pain, stiffness and swelling typically affecting only a few joints
this episode only lasts 1-2d then completely resolves
having positive RF + anti-CCP indicate it will progress to full RA
Rheumatoid Arthritis
which joints are NOT affected
distal interphalangeal joints
Rheumatoid Arthritis
common joints affected
- PIP
- MCP
- wrist + ankle
- Metatarsophalangeal joints
- cervical spine
- large joints also: knee, hips, shoulders
Rheumatoid Arthritis
what is Atlantoaxial Subluxation
occurs in the cervical spine
the axis (C2) and the odontoid peg shift within the atlas (C1)
Rheumatoid Arthritis
what causes Atlantoaxial Subluxation
local synovitis and damage to the ligaments and bursa around the odontoid peg of the axis and the atlas
Rheumatoid Arthritis
why is RA relevant in anaesthetics and intubation
Subluxation can cause spinal compression
MRI scan can visualise changes in these areas as part of pre-op assessment
Rheumatoid Arthritis
what gives the joints a ‘boggy’ feeling
palpation of the synovium in around joints when the disease is active will give this feeling related to inflammation and swelling
Rheumatoid Arthritis
signs in the hands (4)
- Z shaped deformity to the thumb
- Swan neck deformity
- Boutonnieres deformity
- Ulnar deviation of the fingers at the knuckle (MCP joints)
Rheumatoid Arthritis
what is Swan neck deformity
hyperextended PIP with flexed DIP
Rheumatoid Arthritis
what is Boutonnieres deformity
hyperextended DIP and flexed PIP
due to a tear in the central slip of the extensor component of the finger
the flexor digitorum superficialis tendons (lateral tendons that go around the PIP) pull on the distal phalynx without any other supporting structure
Rheumatoid Arthritis
important extra-articular manifestations (4)
- pulmonary fibrosis with pulmonary nodules (Caplan’s syndrome)
- bronchiolitis obliterans: inflammation causing small airway destruction
- Felty’s syndrome: RA, neutropenia, splenomegaly
- Secondary Sjogren’s Syndrome aka sicca syndrome
Rheumatoid Arthritis
other extra-articular manifestations (7)
- anaemia of chronic disease
- CVS disease
- episcleritis + scleritis
- rheumatoid nodules
- lymphadenopathy
- carpal tunnel syndrome
- amyloidosis
Rheumatoid Arthritis
inx
- clinical
- RF
- if RF -ve, check anti-CCP antibodies
- CRP, ESR
- X-ray of hands and feet
- USS: synovitis
Rheumatoid Arthritis
x-ray changes
- joint destruction and deformity
- soft tissue swelling
- periarticular osteopenia
- bony erosions
Rheumatoid Arthritis
when to refer
any adult with persistent synovitis
urgent: if it involves the small joints of the hands or feet, multiple joints or symptoms present >3m
Rheumatoid Arthritis
diagnostic criteria from ACR/ELAR 2010
Patient scored based on:
- joints involved (more + smaller joints score higher)
- RF + CCP
- ESR + CRP
- duration of sx (more or less than 6w)
score ≥6 = dx of RA
Rheumatoid Arthritis
what is the DAS28 score
Disease Activity Score based on the assessment for 28 joints
useful in monitoring disease activity and response to trx
Rheumatoid Arthritis
what are the points given for on a DAS28 score
- swollen joints
- tender joints
- ESR/CRP result
Rheumatoid Arthritis
what is the Health Assessment Questionnaire (HAQ)
measures functional ability
NICE recommend using this at diagnosis to check response to trx
Rheumatoid Arthritis
what factors make a worse prognosis
- younger onset
- male
- more joints and organs affected
- presence of RF and anti-CCP
- erosions seen on x-ray
Rheumatoid Arthritis
what can be used at 1st presentation and during flare ups to quickly settle the disease
- short course of steroids
- NSAIDs + PPI
Rheumatoid Arthritis
NICE guidelines for DMARDs 1st line
monotherapy with:
- methotrexate
- leflunomide
- sulfasalazine
Rheumatoid Arthritis
what DMARD can be considered in mild disease (considered the mildest anti-rheumatic drug)
hydroxychloroquine
Rheumatoid Arthritis
NICE guidelines for DMARDs 2nd line
2 of either:
- methotrexate
- leflunomide
- sulfasalazine
Rheumatoid Arthritis
NICE guidelines for DMARDs 3rd line
Methotrexate
+
biological therapy: usually a TNF inhibitor
Rheumatoid Arthritis
NICE guidelines for DMARDs 4th line
Methotrexate
+
rituximab
Rheumatoid Arthritis
why do pregnant women have an improvement in symptoms
probs due to the higher natural production of steroid hormones
Rheumatoid Arthritis
what DMARDs can be given in pregnancy
Hydroxychloroquine and Sulfasalazine
Rheumatoid Arthritis
what can biological therapies leads to
immunosuppression : prone to serious infections
reactivation of dormant infections: TB + Hep B
Rheumatoid Arthritis
Biological therapies: name some Anti-TNFs
- adalimumab
- infliximab
- etanercept
- golimumab
- certolizumab pegol
Rheumatoid Arthritis
what is rituximab
a biological therapy (Anti-CD20)
Rheumatoid Arthritis
how does methotrexate work?
by interfering with the metabolism of folate and suppressing certain components of the immune system
Rheumatoid Arthritis
how often is methotrexate taken and by which route
injection or tablet
once a week
Rheumatoid Arthritis
what is prescribed alongside methotrexate and when should it be taken
Folic acid 5mg
once a week
taken on a different day to methotrexate
Rheumatoid Arthritis
notable SEs of methotrexate (5)
- pulmonary fibrosis
- Teratogenic: avoid prior to conception in mothers and fathers
- mouth ulcers and mucositis
- liver toxicity
- bone marrow suppression + leukopenia (low WCC)
Rheumatoid Arthritis
how does Leflunomide work
an immunosuppressant medication
interferes with the production of pyrimidine (an important component of RNA + DNA)
Rheumatoid Arthritis
side effects of Leflunomide (2)
- Peripheral neuropathy
- increased BP
Rheumatoid Arthritis
how does Sulfasalzine work
immunosuppressive + anti-inflammatory medication
mechanism not clear but may be related to folate metabolism
Rheumatoid Arthritis
what needs to be prescribed alongside Sulfasalzine
folic acid
Rheumatoid Arthritis
SEs of Sulfasalzine (2)
- temporary male infertility (reduced sperm count)
- bone marrow suppression
Rheumatoid Arthritis
how does hydroxychloroquine work
traditionally an anti-malarial med
acts as an immunosuppressive medication by interfering with Toll-like receptors
disrupting antigen presentation and increasing the pH in the lysosomes of immune cells
Rheumatoid Arthritis
SEs of hydroxychloroquine (4)
- nightmares
- reduced visual acuity (macular toxicity)
- liver toxicity
- skin pigmentation
Rheumatoid Arthritis
how do anti-TNF drugs work
tumour necrosis factor is a cytokine involved in stimulating inflammation
blocking TNF reduces inflammation
Rheumatoid Arthritis
what is the difference between Adalimumab, infliximab, golimumab and certolizumab pegol
and Etanercept
the first 4 are monoclonal antibodies to TNF
Etanercept is a protein that binds TNF to the Fc portion of IgG and thereby reduces its activity
Rheumatoid Arthritis
SEs of Anti-TNF drugs
- Vulnerability to severe infections and sepsis
- Reactivation of TB and hepatitis B
Rheumatoid Arthritis
how does Rituximab work
a monoclonal antibody that targets the CD20 protein on the surface of B cells
This causes destruction of B cells
used for immunosuppression for autoimmune conditions
Rheumatoid Arthritis
SEs for Rituximab
- Night sweats
- thrombocytopenia
Myositis
what is Polymyositis
autoimmune condition of chronic inflammation of muscles
Myositis
what is dermatomyositis
an autoimmune connective tissue disorder where there is chronic inflammation of the skin and muscles
Myositis
what is the key inx to diagnose it
creatine kinase blood test
Myositis
what is a normal CK
<300 U/L
Myositis
what is the CK in polymyositis and dermatomyositis
usually >1000
Myositis
what are other causes of raised CK levels
- rhabdomyolysis
- AKI
- MI
- statins
- strenuous exercise
Myositis
what causes a raised CK
inflammation in the muscles cells leads to the release of CK (an enzyme found in muscle cells)
Myositis
what makes Polymyositis or dermatomyositis a paraneoplastic syndrome
they can be caused by an underlying malignancy:
- lung
- breast
- ovarian
- gastric
Myositis
presentation (4)
- muscle pain, fatigue, weakness
- bilateral and typically proximal muscles
- mostly shoulder and pelvic girdle affected
- develops over weeks
Myositis
difference in presentation between Polymyositis or dermatomyositis
Polymyositis occurs without any skin features
Dermatomyositis has skin features
dermatomyositis skin features
- Gottron lesions on the knuckles, elbows and knees
- photosensitive erythematous rash on the back shoulders and neck
- purple rash on the face and eyelids
- periorbital oedema (swelling around the eyes)
- subcutaneous calcinosis (Ca deposits in the subcut tissue)
Dermatomyositis
what are Gottron lesions
scaly erythematous patches found on the knuckles elbows and knees
Polymyositis autoantibodies
Anti-Jo-1 antibodies
dermatomyositis autoantibodies
Anti-Mi-2 antibodies
Anti-nuclear antibodies
Anti-Jo-1 antibodies (also in polymyositis)
Myositis
dx
- definitive: muscle biopsy
- clinical presentation
- elevated CK
- autoantibodies
- electromyography (EMG)
Myositis
mnx
refer to rheumatologist
- physio, occupational therapy
1st line: corticosteroids
- immunosuppressants (azithioprine)
- IV immunoglobulins
- Biological therapy (infliximab or etanercept)
Osteomalacia
what is it
osteo: bone
malacia: soft
condition where there is defective bone mineralisation causing soft bones
results from insufficient vit D
Osteomalacia
what is rickets
osteomalacia in children prior to their growth plates closing
Osteomalacia
why is vit D deficiency common in CKD
the kidneys are essential in metabolising vit D to its active form
Osteomalacia
what is vit D essential for
- calcium and phosphate absorption from the intestines and kidneys
- bone turnover
Osteomalacia
what causes defective bone mineralisation
inadequate levels of vit D –> low ca and phosphate in blood
ca and phosphate are required for construction of bone
Osteomalacia
why does low calcium cause secondary hyperparathyroidism
parathyroid gland tries to raise ca levels by secreting parathyroid hormone
which increases reabsorption of ca from the bones
Osteomalacia
symptoms
may not have any
- fatigue
- bone pain
- muscle weakness
- muscle aches
- pathological or abnormal fractures
Osteomalacia
what are looser zones
fragility fractures that go partially through the bone
Osteomalacia
RFs
RFs for Vit D deficiency
- darker skin
- low exposure to sunlight
- live in colder climates
- spend majority of time indoors
Osteomalacia
what is the lab inx for vit D
serum 25-hydroxyvitamin D
Osteomalacia
interpretation of Serum 25-hydroxyvitamin D <25 nmol/L
vitamin D deficiency
Osteomalacia
interpretation of Serum 25-hydroxyvitamin D 25 – 50 nmol/L
vitamin D insufficiency
Osteomalacia
interpretation of Serum 25-hydroxyvitamin D 75 nmol/L or above
optimal
Osteomalacia
inx results
- low serum ca
- low serum phosphate
- Serum alkaline phosphatase may be high
- PTH may be high
- X-Ray: may show osteopenia (more radiolucent bones)
- DEXA scan shows low bone mineral density
Osteomalacia
trx
Colecalciferol with one of the following regimes:
- 50,000 IU once weekly for 6 weeks
- 20,000 IU twice weekly for 7 weeks
- 4000 IU daily for 10 weeks
A maintenance supplementary dose of 800 IU or more per day should be continued for life after the initial treatment.
Osteomalacia
trx for pts with only vitamin D insufficiency
started on the maintenance dose without the initial treatment regime
SLE
what is it
Systemic Lupus Erythematosus
an inflammatory autoimmune connective tissue disease
SLE
whom is it more common in
women and Asians
SLE
when does it present
in young to middle aged adults
but can present later in life
SLE
what are the leading causes of death
CVD and infection
SLE
pathophysiology
inflammatory response to anti-nuclear antibodies
SLE
symptoms
- photosensitive malar rash. Butterfly shaped rash across nose and cheek bones that gets worse with sunlight
- hair loss
- SOB
- splenomegaly
- joint pain
- lymphadenopathy
- myalgia
- fatigue
- weight loss
SLE
which antibodies are associated with it
anti-nuclear antibodies (ANA)
anti-double stranded DNA (anti-dsDNA)
SLE
inx
- autoantibodies (ANA, anti-dsDNA)
- FBC: normocytic anaemia
- C3+4: low
- CRP+ESR: high
- Immunoglobulins: raised
- Urinalysis: proteinuria in lupus nephritis
- renal biopsy: lupus nephritis
SLE
what are ANA
anti-nuclear antibodies
antibodies against normal proteins in the cell nucleus
not specific
SLE
what are anti-dsDNA
specific to SLE. Useful for monitoring disease activity
SLE
which type of ANA is highly specific to SLE but not very sensitive
Anti-Smith
which type of ANA is associated with systemic sclerosis
Anti-Scl-70
which type of ANA is associated with Sjogren’s syndrome
Anti-Ro and Anti-La
which type of ANA is associated with limited cutaneous systemic sclerosis
Anti-centromere antibodies
SLE
what can occur secondary to SLE
Antiphospholipid antibodies and antiphospholipid syndrome
associated with an increased risk of VTE
SLE
what do you use to establish a diagnosis
the SLICC Criteria or the ACR Criteria
involves confirming the presence of ANA and establishing a certain number of clinical features
SLE
complications: why does it cause CVD
chronic inflammation in the blood vessels –> HTN + coronary artery disease
SLE
complications: why is infection more common
part of the disease process and secondary to immunosuppressants.
SLE
complications: why is anaemia of chronic disease more common
It affects the bone marrow causing a chronic normocytic anaemia
can also get low WCC, neutrophils and platelets
SLE
complications: why does lupus nephritis occur
inflammation in the kidney. it can progress to end-stage renal failure
SLE
1st line trx
anti-inflammatory and immunosuppression
- NSAIDs
- steroids (prednisolone)
- ## hydroxychloroquine
SLE
what is 1st line trx for mild SLE
hydroxychloroquine
SLE
other commonly used immunosuppressants in resistant or more severe lupus
Methotrexate
Mycophenolate mofetil
Azathioprine
Tacrolimus
Leflunomide
Ciclosporin
SLE
what is considered for patients with severe disease or where patients have not responded to other treatments.
Biological therapies:
- Rituximab
- Belimumab
SLE
how does Belimumab work
monoclonal antibody that targets B-cell activating factor
what needs to be tested before giving azathioprine
Thiopurine methyltransferase enzyme activity
why does the activity of thiopurine methyltransferase need to be tested before starting azathioprine
metabolism + elimination of azathioprine and its metabolites involve xanthine oxidase and thiopurine methyltransferase (TPMT)
azathioprine should not be prescribed to patients with absent TPMT activity
Psoriatic Arthritis
what is it
an inflammatory arthritis associated with psoriasis
Psoriatic Arthritis
what is arthritis mutilans
most severe form of psoriatic arthritis
osteolysis (destruction) of the bones around the joints in the digits.
This leads to progressive shortening of the digit.
The skin then folds as the digit shortens giving an appearance that is often called a “telescopic finger”.
Psoriatic Arthritis
what group of conditions is it part of
seronegative spondyloarthroapathy
Psoriatic Arthritis
name 3 patterns of it
- Symmetrical polyarthritis
- Asymmetrical pauciarthritis
- Spondylitic pattern
Psoriatic Arthritis
describe the symmetrical polyarthritis pattern
- similar to RA presentation
- more common in women
- hands, wrists, ankles, DIP joints are affected
- MCP joints are less commonly affected (unlike rheumatoid)
Psoriatic Arthritis
describe the asymmetrical pauciarthritis pattern
- affects mainly digits and feet
- pauciarthritis: only affects a few joints
Psoriatic Arthritis
describe the spondylitic pattern
- more common in men
- back stiffness
- sacroilitis
- atlanto-axial joint involvement
Psoriatic Arthritis
what other areas can be affected (not the patterns)
- spine
- achilles tendon
- plantar fascia
Psoriatic Arthritis
signs (5)
- plaques of psoriasis on skin
- pitting of the nails
- onycholysis
- dactylitis
- enthesitis
Psoriatic Arthritis
what is onycholysis
separation of the nail from the nail bed
Psoriatic Arthritis
what is dactylitis
inflammation of the full finger
Psoriatic Arthritis
what is enthesitis
inflammation of the entheses (points of insertion of tendons into bone)
Psoriatic Arthritis
other associations (3)
- eye disease: conjunctivtiis + anterior uveitis)
- aortitis (inflammation of the aorta)
- amyloidosis
Psoriatic Arthritis
what screening tool is recommended by NICE to use in patients with psoriasis
Psoriasis Epidemiological Screening Tool (PEST)
to screen for psoriatic arthritis
Psoriatic Arthritis
what does the Psoriasis Epidemiological Screening Tool (PEST) involve
questions asking about joint pain, swelling, a history of arthritis and nail pitting.
A high score triggers a referral to a rheumatologist.
Psoriatic Arthritis
x-ray changes (6)
1) pencil in cup appearance
2) periostitis
3) ankylosis
4) osteolysis
5) dactylitis
Psoriatic Arthritis
x-ray changes: what is pencil in cup
central erosions of the bone beside the joints
this causes the appearance of one bone in the joint being hollow and looking like a cup
whilst the other is narrow and sits in the cup.
Psoriatic Arthritis
x-ray changes: what is periostitis
inflammation of the periosteum causing a thickened and irregular outline of the bone
Psoriatic Arthritis
x-ray changes: what is ankylosis
bones joining together causing joint stiffening
Psoriatic Arthritis
x-ray changes: what is osteolysis
destruction of bone
Psoriatic Arthritis
x-ray changes: what is dactylitis
inflammation of the whole digit
appears on the xray as soft tissue swelling
Psoriatic Arthritis
mnx
- NSAIDs for pain
- DMARDS (methotrexate, leflunomide or sulfasalazine)
- Anti-TNF medications (etanercept, infliximab or adalimumab)
- Ustekinumab (last line)
Psoriatic Arthritis
what is last line mnx and what is it
Ustekinumab: a monoclonal antibody that targets interleukin 12 and 23
Gout
what are gouty tophi
subcutaneous deposits of uric acid typically effecting the small joints and connective tissues of the hands, elbows and ears
Gout
which joint in the hands is most effected by gouty tophi
the DIP joints
Gout
presentation
single acute hot swollen and painful joint
Gout
RFs (7)
- male
- obese
- high purine diet (meat+seafood)
- alcohol
- diuretics
- existing CVD or kidney disease
- FH
Gout
which typical joints are affected
- Base of the big toe (metatarsophalangeal joint)
- Wrists
- Base of thumb (carpometacarpal joints)
Gout
Dx
clinically or by aspiration of fluid from the joint
Gout
what will aspirated fluid show
- no bacterial growth
- needle shaped crystals
- negatively birefringent of polarised light
- monosodium urate crystals
Gout
what will x-ray show
- joint space maintained
- lytic lesions
- punched out erosions:
sclerotic borders
overhanging edges
Gout
acute flare mnx
1st line: NSAIDs
2nd line: colchicine
3rd line: steroids
Gout
when is colchicine used
during an acute flare when NSAIDs are inappropriate: renal impairment or significant heart disease
Gout
common SE of colchicine
diarrhoea (dose dependent)
Gout
prophylaxis mnx
allopurinol
lifestyle changes
Gout
what is allopurinol
a xanthine oxidase inhibitor used for the prophylaxis of gout: it reduces uric acid levels
Antiphospholipid Syndrome
what is it
a disorder associated with antiphospholipid antibodies where the blood becomes prone to clotting. The patient is in a hyper-coagulable state.
Antiphospholipid Syndrome
main associations
- thrombosis
- recurrent miscarriages
Antiphospholipid Syndrome
can occur secondary to which condition
SLE
Antiphospholipid Syndrome
which antiphospholipid antibodies is it associated with
- lupus anticoagulant
- Anticardiolipin antibodies
- Anti-beta-2 glycoprotein I antibodies
Antiphospholipid Syndrome
what do the antiphospholipid antibodies do
interfere with coagulation and create a hypercoagulable state where the blood is more prone to clotting.
Antiphospholipid Syndrome
what is the rash that you get called
livedo reticularis
Antiphospholipid Syndrome
what is livedo reticularis
a purple lace like rash that gives a mottled appearance to the skin
Antiphospholipid Syndrome
what is Libmann-Sacks endocarditis
a type of non-bacterial endocarditis where there are growths (vegetations) on the valves of the heart
mitral valve is most commonly affected
associated with SLE and antiphospholipid syndrome.
Antiphospholipid Syndrome
blood results
thombocytopenia (low platelets)
Antiphospholipid Syndrome
dx
hx of thrombosis or pregnancy complication
AND persistent antibodies:
- lupus anticoagulant
- anticardiolipin antibodies
- anti-beta-2 glycoprotein I antibodies
Antiphospholipid Syndrome
mnx if not pregnant
- long term warfarin (INR 2-3)
Antiphospholipid Syndrome
mnx if pregnant
LMWH (enoxaparin) + Aspirin
Pseudogout
which crystals are present
calcium pyrophosphate crystals