Cortex- Rheumatology Flashcards
what are the 4 sub chatagories of inflammatory arthritis
seropistive, seronegative, infectious and crystal induced
what are the seropositive arthrides
RA, lupus, scleroderma, vasculitis, srogrens
what are the seronegative arthrides
AS, psoriatic arthritis, reactive arthritis, IBD arthritis
name the conditions associated with this antigen:
anti-ccp
RA
name the conditions associated with this antigen:
ANA
SLE, sjogrens, systemic sclerosis, MCTD, autoimmune liver disease
name the conditions associated with this antigen:
anti-dsDNA
SLE
name the conditions associated with this antigen:
anti-sm
SLE
name the conditions associated with this antigen:
anti-ro
SLE, sjogrens
name the conditions associated with this antigen:
anti-la
sjogrens
name the conditions associated with this antigen:
anti-centromere
systemic sclerosis (limited)
name the conditions associated with this antigen:
anti-scl-70
systemic sclerosis (diffuse)
name the conditions associated with this antigen:
anti-rnp
SLE, MCTD
name the conditions associated with this antigen:
anti-jo-1
myositis
name the conditions associated with this antigen:
anti-cardiolipin antibody and lupus anti- coagulant
anti-phopholipid syndrome
name the conditions associated with this antigen:
ANCA
small vessel vasculitis (GPA, EGPA, MPA)
what mutation in OA
none found, no pattern of madelian inheritance
what can cause secondary osteoarhritis
injury dislocation, perthes disease, crystal arthropathy, inflammatory arthritis, genu varum or valum
what does LOSS on x ray if OA mean
Loss of joint space
Osteophytes
Sclerosis
Subchondral cysts
what is the main treatment for osteoarthritis
analgesia, mild opiates, physiotherapy, weight loss and exercise, joint replacement
what are the antiflammatory treatments
steroids and NSAIDs
what is the joint involvement pattern of RA
symmetric polyarthopathy, mostly small joints of hands and feet- larger joints as disease progresses
who gets RA
women 2x more likely
peak age 35-50
what is the pathogenesis of RA
immune response against synovium initiated by e.g smoking, infection or trauma
inflammatory pannus forms which then attacks and denudes articular cartilage
=joint destruction, tendon ruptures, joint instability, subluxation
what are the clinical features of RA
symmetrical synovitis (doughy swelling), pain and morning stiffness
rheumatoid nodules
lung involvement- pleural effusions, interstitial fibrosis, pulmonary nodules
cardio morbidity and mortality increased
occular- keratoconjunctivitis, sicca, episcleritis, uveitis, nodular scleritis
what joints are not affect in RA
DIPs
how can RA cause cervical chord compression
cervical spine affected- atlanto-axial subluxation
antibodies used to diagnose RA, which is more specific
RF and anti-CCP (more specific)
what investigations to diagnose RA
antibodies (RF, anti-CCP)
CRP and ESR raised
ultrasound for synovial inflammation
x rays might show peri-articular osteopenia, soft tissue swelling (more helpful in late disease)
when do you aim to start DMARDs
within 3 months of symptom onset
what is the treatment for RA
short term- analgesia, NSAIDs, steroids (IM, IA, oral)
DMARDs- methotrexate first line (also have sulphasalazine, hydroxycholorquine and lefunomide) (regular blood tests required for immune suppression)
last line biologics- anti TNF (all given by injection) (screen for TB)
physio, occupational therapy, podiatrists, orthotists
what are the four components of DAS28 scores
tender joint count, swollen joint count, CRP/ESR, visual analogue score
what are the perimeters of DAS28 scores
DAS 28 < 2.6 Remission
DAS 28 2.7-3.2 Low disease activity
DAS 28 3.3-5.1 Moderate disease activity
DAS 28 >5.1 High disease activity
patients with seronegative inflammatory arthropathies usually have what gene
HLA-B27
who gets AS
males (3:1), typically between 20-40
what is the presentation of AS
spinal pain, stiffness, may also develop knee and hip arthritis
describe the posture in AS
question mark- loss of lumbar lordosis and increased thoracic kyphosis
what test measures lumbar spine flexion
schobers test (measuring 5cm below posterior superior iliac crests and 10 cm above- when bending forward should extend beyond 20cm)
what conditions are associated with AS
anterior uveitis, aortitis, pulmonary fibrosis, amyloidosis
what might x rays show in AS
slcerosis, fusion of sacroiliac joints, syndesmophytes (bony spurs which can bridge the intervertebral disc resulting in fusion= bamboo spine)
what early features of AS can MRI detect
bone marrow oedema, enthesitis of spinal ligaments
what is the treatment for AS
physio, exercise, NSAIDs, anti TNF
DMARDS HAVE NO EFFECT on spinal disease but may help peripheral joint inflammation
what pattern is psoriatic arthritis
asymmetrical oligoarthritis
what symptoms do PA patients usually have
nail changes- pitting, onycholysis
what is arthitis mutlians
aggressive form of PA
what is the treatment for PA
similar to RA, with DMARDs, usually methotrexate. Anti-TNF therapy is available for those who do not respond to standard treatment
what is enteropathic arthritis
inflammatory arthritis involving the peripheral joints and sometimes spine, occurring in patients with inflammatory bowel disease
what pattern in enteropathic arthritis
large joint asymmetrical oligoarthritis
what infections most commonly cause reactive arthritis
gentiourinary (chlamydia, neisseria) and GI (salmonella and campylobacter)
what is reiters syndrome
traid of symptoms in reactive arthritis- urethritis, uveitis/ conjunctivitis and arthritis
what is the treatment for reactive arthritis
underlying infection treated, symptomatic relief (IA or IM steroids)
occasionally DMARDs in chronic cases
what is the pathogenesis of SLE
mediated by circulating immune complexes that are formed in the small blood vessels and lead to complement activation and inflammation
antibody-antigen complexes are deposited on the basement membranes of the skin and kidney
what renal symptoms in SLE
lupus nephritis
what resp symptoms in SLE
pleurisy, pleural effusion, pneumonitis, PE, PHT, ILD
what haematological symptoms in SLE
leukopenia, lymphopenia, anaemia, thrombocytopenia
what neuropsychiatric symptoms in SLE
seizures, psychosis, headache, aseptic meningitis
what cardio symptoms in SLE
pericarditis, pericardial effusion, PHT, sterile endocarditis, accelerated ischemic heart disease
what immunology for SLE
ANA- not specific
anti dsDNA- specific, varies with disease activity
Anti-sm specific, low sensitivity
Anti-ro, anti- RNP- seen in other conditions
C3/C4 level- low when disease activity high
what is urinalysis for in SLE
evidence of glomerulonephritis
what imaging for SLE
to look for evidence of organ involvement eg CT chest for interstitial lung disease, MRI brain for cerebral vasculitis, echo for pericardial effusion
management for SLE skin disease and arthralgia
hydroxychloroquine, topical steroids and NSAIDs
management for SLE with inflammatory arthritis/ organ involvement
immunosuppression, corticosteroids
what is sjogrens characterised by
lymphocytic infiltrates in exocrine organs- typically causes dryness of the eyes, mouth (sicca symptoms)
other symptoms include arthralgia, fatigue, vaginal dryness, parotid gland swelling
what can sjogrens occur secondary to
RA and SLE
what complications can occure in sjogrens
ILD, peripheral neuropathy, increased risk of lymphoma
how do you diagnose sjogrens
confirmation of ocular dryness (schirmers test), positive anti-ro and anti-la antibodies, typical features on a lip gland biopsy
what is the treatment for sjogrens
symptomatic (lubricants)
regular dental care
Hydroxychloroquine - arthralgia and fatigue
immunosuppresion in organ involvement
what are characteristics of systemic sclerosis
vasomotor disturbances (raynauds)
fibrosis
subsequent atrophy of skin and subcutaneous tissue
excessive collagen deposition causes skin and internal organ changes
calcinosis (subcutaneous calcium deposits in digits)
sclerodactyly
what are the phases of cutaneous involvment in systemic sclerosis
- oedematous
- indurative
- atrophic
skin becomes thickened and tight
what are signs of systemic sclerosis on the face
beaking of nose
tightening of skin around mouth
telangiectasia
describe organ involvement in systemic sclerosis
Pulmonary hypertension, pulmonary fibrosis and accelerated hypertension leading to renal crisis are important organ manifestations.
Gut involvement may lead to dysphagia, malabsorption and bacterial overgrowth of the small bowel.
Inflammatory arthritis and myositis may be seen.
describe diffuse ans limited systemic sclerosis
Limited: skin involved tends to be confined to face, hands and forearms and feet. Organ involvement tends to occur later. Anti-centromere antibody association.
Diffuse: skin changes develop more rapidly and can involve the trunk. Early significant organ involvement. Anti-Scl-70 antibody association.
what conditions comprised the symptoms seen in MCTD
Raynauds phenomenon
Arthralgia/arthritis
Myositis
Sclerodactyly
Pulmonary hypertension
Interstitial lung disease (ILD)
what antibody in MCTD
anti-RNP
what management for MCTD
risk of pulmonary hypertension, regular echocardiograms (annually) are suggested. Screening for ILD with pulmonary function tests should also take place
Calcium channel blockers may be helpful for Raynauds.
If there is significant muscle or lung disease then immunosuppression may be required
what is the treatment for systemic sclerosis
Raynauds/digital ulcers: calcium channel blockers, Iloprost, bosentan
Renal involvement: ACE inhibitors
GI involvement: proton pump inhibitors for reflux
Interstitial lung disease: immunosuppression, usually with cyclophosphamide
what can anti-phospholipid syndrome occur in association with
SLE/ rheumatic/ autoimmune disorder
what is libman sacks
sterile lesions in endocarditis (seen in APS)
what is a common cutaneous finding of APS
livedo reticularis
what investigations into APS
may be thrombocytopenia and prolongation of APTT.
Lupus anticoagulant, anti-cardiolipin antibodies and anti-beta 2 glycoprotein may be positive.
what treatment for APS
anti-coagulation for those with an episode of thrombosis. In patients with recurrent pregnancy loss low molecular weight heparin is used during pregnancy (warfarin is teratogenic).
Patients who are found to have positive antibodies but who have never had had an episode of thrombosis do not require anti-coagulation.
what is uric acid
the final compound in the breakdown of purines in DNA metabolism
what can cause hyperuricaemia
renal underexcretion (which can be exacerbated by diuretics or renal failure) or due to excessive intake of alcohol, red meat and seafood
what can cause the precipitation of urate crystals
dehydration, trauma or surgery
what is podagra
classic site of gout- 1st MTP
how does gout present
intensely painful, red, hot, swollen joint- lasts 7-10 days in untreated
what are gouty trophi
painless white accumulations of uric acid
what can chronic gout result in
destructive erosive arthritis
what is seen in gout in polarised microscopy
negative birefringence
what is the treatment for gout
acute attacks;
NSAIDs,
corticosteroids,
opioid analgesics and colchicine for patients who cannot tolerate NSAIDs.
For sufferers of recurrent attacks or those with joint destruction or tophi, allopurinol or other urate lowering therapies can prevent attacks but they should not be started until an acute attack has settled as they can potentiate a further flare
what crystals in pseudogout
calcium pyrophosphate
what is chondrocalcinosis
when calcium pyrophosphate deposition occurs in cartilage and other soft tissues in the absence of acute inflammation
what is CPPD
Calcium Pyrophosphate Deposition disease (pseudogout and chondrocalcinosis)
what joints does CPPD affect
knee, wrist, ankle
what is the treatment for CPPD
Treatment of acute attacks includes NSAIDs, corticosteroids (systemic and intra‐articular) and occasionally colchicine
nothing for prophylaxis
who does Polymyalgia rheumatica affect
over 50s
what is Polymyalgia rheumatica characterised by
proximal myalgia of the hip and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour. Symptoms tend to improve as the day goes on and with movement.
what biochem signs is Polymyalgia rheumatica always associated with
raised CRP, PV, ESR
what treatment can be used as a diagnostic tool for Polymyalgia rheumatica
Symptoms respond very dramatically to low dose steroids (prednisolone 15mg daily)
what is the most common form of systemic vascultitis in adults
giant cell arteritis
describe GCA histologically
transmural inflammation of the intima, media, and adventitia of affected arteries, as well as patchy infiltration by lymphocytes, macrophages, and multinucleated giant cells. Vessel wall thickening can result in arterial luminal narrowing, resulting in subsequent distal ischemia
what are the symptoms of GCA
visual disturbances, headache, jaw claudication and scalp tenderness. Constitutional manifestations, such as fatigue, malaise, and fever
describe the headache in GCA and the clinical signs of this
continuous and located in the temporal or occipital areas. Focal tenderness on direct palpation is typically present. The patient may note scalp tenderness with hair combing.
The temporal artery may be thickened and prominent and tender to touch.
what is the definitive test for GCA
arterial biopsy
what do biopsys find in GCA
mononuclear infiltration or granulomatous inflammation, usually with multinucleated giant cells.
what is the treatment for GCA
40mg prednisolone, 60mg if vision impaired ASAP
tapered over 18 months-2 years
what is polymyositis
idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness in upper and lower extremities
who gets Polymyositis and dermatomyositis
women 2:1
over 20s, peak 45-60
what is the pathogenesis of Polymyositis
T-cell–mediated cytotoxic process directed against unidentified muscle antigens. CD8 T cells, along with macrophages, initially surround healthy nonnecrotic muscle fibers and eventually invade and destroy them
what antibodies in Polymyositis and dermatomyositis
ANA, anti-RNP antibody (shred with other autoimmune)
unique- anti-Jo-1, anti-SRP antibodies
what are the complications of polymositis
Dysphagia secondary to oropharyngeal and esophageal involvement
ILD
what investigations into PM
Inflammatory markers are often raised.
Serum creatine kinase (CK) level is usually raised, often more than 10 times the normal level.
Autoantibodies include ANA, anti-Jo-1 and anti-SRP.
MRI may be useful to localize the extent of muscle involvement.
electromicrograph
muscle biopsy- fibres in stages of inflammation, necrosis, and regeneration.
what is the management for PM and DM
prednisolone (initially around 40mg) combined with immunosuppressive drugs such as methotrexate or azathioprine.
what are the cutaneous features of DM
V shaped rash over chest
gottrons papules
heliotrope rash
describe large vessel vasculitis
primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches
what are the two types of large vessel vasculitis
temporal (giant cell) arteritis and Takayasu arteritis (TA)
what happens if larger vessel vasculitis is left untreated
vascular stenosis and aneurysms- reduced pulses and bruits
what investigations in vasculitis
raised ESR, PV, CRP
MR angiography good for stenosis/ thickened vessels
what is the treatment for large vessel vasculitis
corticosteroids, starting at 40-60mg prednisolone and gradually reducing. Steroid sparing agents such as methotrexate and azathioprine may be added
what are common features of small vessel vasculitis
Fever and weight loss
A raised, non blanching purpuric rash
Arthralgia/arthritis
Mononeuritis multiplex
Glomerulonephritis
Lung opacities on x-ray
what are the four types of ANCA associated vasculitis
Granulomatosis with polyangiitis (GPA) or Wegeners
Eosinophilic granulomatosis with polyangiitis (EGPA)
Microscopic polyangiitis
churg strauss syndrome
what symptoms are common in GPA
ENT symptoms are common in GPA and include nose bleeds, deafness, recurrent sinusitis and nasal crusting (over time there can be collapse of the nose). Respiratory symptoms such as haemoptysis and cavitating lesions on x-ray
what antibodies are associated with GPA
cANCA and PR£
what symptoms are associated with EGPA
late onset asthma, rhinitis and a raised peripheral blood eosinophil count.
Neurological symptoms such as mono neuritis multiplex are common
what is the most common complication in microscopic polyangitis
glomerulonephritis, which occurs in up to 90% of patients.
what investigations in AAV
ESR, PV and CRP and raised
Anaemia of chronic disease is common.
U+E looking for renal involvement
Anti-neutrophil cytoplasmic antibody (ANCA)
Urinalysis (looking for renal vasculitis)
CXR
Biopsy of an affected area e.g. skin or kidney
treatment for AAV
IV steroids and cyclophosphamide due to their aggressive disease course
what is henloch-schonlein purpura
acute immunoglobulin A (IgA)–mediated disorder characterized by a generalised vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system
who does Henoch-Schönlein purpura affect and how
children. Commonly a history of an upper respiratory tract infection predates the symptoms by a few weeks.
purpuric rash over the buttocks and lower limbs, abdominal pain and vomiting and joint pain
what is the treatment form Henoch schonlein purpura
self limiting condition not requiring specific treatment. It usually settles over the course of weeks to months.
what is the least likely cause of chest pain in SLE out of ;
a. Pleurisy
b. Pulmonary embolus
c. Pneumothorax
d. Pericarditis
e. Myocardial infarction
pneumothorax
what antibody is most specific to SLE
anti-DNA binding, anti dsDNA
cane sacrolitis be a feature of crohns related arthritis
yes
is aortic valve incompetence a common manifestation of the spondyloarthritides
yes
should allopurinol be stopped during acute flares of gout
no
