Conditions Flashcards
What is the pathophysiology behind RA?
It is an autoimmune condition which occurs when there’s antibodies to a portion of the IgG known as RF and another for Anti-citrullinated cyclic peptide. There is a process of citrullination of self antibodies which in turn causes activation of t and b cells and this results in secretion of TNF and proliferation of synoviocytes. Which essentially block the cartilage from nutrients and results in death of the cartilage. Macrophages also cause increased activation of osteoclasts causing bone damage
What is the patient demographic for RA?
- usually female likelihood 3:1 and 30-50
What do you expect to see.on examination of a patient with RA?
- progressive peripheral and symmetrical polyarthritis
-affect MCP and PIP and MTPS. ( OA is of the DIPJ) - morning stiffness of more than 6 weeks
- soft tissue swelling and tenderness
- ulanr deviation
-swan neck deformity and bouternniera - rhuematoid nodules- usually on the elbow
- MAY present with carpal tunnel
What are the investigations associated with RA?
- RF and Anti CCP
- FBC- can present with normocytic anaemia in chronic RA
- WCC to rule out septic arthritis
- X RAY
- inflammatory markers
- Hrct- high Res CT and Pulmonary function tests PFT
Why do patients present with normocytic anaemia in RA??
Because they have chronic inflammation, therefore bone marrow lifespan is reduced
Why do patients present with lung problems in RA?
It’s one of the most common extra articular signs of RA- it occurs because of the formation of Rheumatoid nodules in the lungs
What is the treatment of RA?
DMARD monotherapy- methotrexate and hydroxychloroquine, sulfasalazine
Steroids- PO/IMA OR INTRARTICULAR
- control the symptoms with NSAIDS but give PPI cover
- if RA is persistent then consider biologics- like ANTI- TNF
- consider occupational therapy or physiotherapy
What are the many extra -articular manifestations of RA?
3Cs- carpal tunnel, CVD, Cord compression - via subluxation of the atlas and axial bone
3As- anaemia and amyloidosis and arteritis
3Ps- pericarditis, pleural disease, pulmonary disease,
3Ss- Sjorgens, scleritis and episcleritis, splenic enlargement, ( with neutropenia called Feltys)
What are the X-ray features of RA?
LESS
Loss of joint space
Erosions
Soft tissue swelling
Subluxation
WHAT IS GIANT CELL ARTERITIS?
this is when there is vasculitis of th vessels which originate from the arch of the aorta. It is often called TEMPORAL ARTERITIS
- l
What are the main risk factors associated with GCA?
-most patients are over 60- rare in patients under 50
-Women
-more common in caucasian patients rarer in Afro-american
-Polymyalgia Rheumatica has a strong link to developing GCA
-genetic predisposition if they have HLA-DR4
What is HLA-DR4?
This is a genetic mutation- this is a a human leukocyte antigen which increases the predisposition to RA.
What is Polymyalgia rheumatica?
Inflammatory condition which causes pain, swelling and inflammation in the upper limbs and hips
What are the symptoms of GCA?
-visual disturbances- vision problems- blurring, diplopia and amarousis fugax- visual symptoms tend to come about weeks to months after initial symptom onset
-Jaw claudication and pain on chewing
-Lancinating unilateral boring pain- over one side of the temple
-Scalp tenderness over the temple
-Headache
What is lancinating pain?
-Sharp, stabbing pain
How is a diagnosis of GCA made?
-If there are 2 or more of any of these criteria in someone who is 50+ old:
-new visual problems
=new onset headache
-tenderness on scalp- in the temporal artery region
-Biopsy with necrotizing arteritis
-Raised ESR, CRP AND PV
What is ESR, PV?
-ESR is erethrocyte sedimentation rate- this is a measure of inflammation
-Plasma viscosity- is also increased in inflammation
What is the treatment of GCA?
-Prednisilone- 60-100mg over 2 weeks- before slowly tapering it off -(NEVER STOP IT IMMEDIATELY- HYPOADRENAL SHOCK)
-Methylprednisilone in acute onset of visual symptoms- give it over pulse therapy 1-3 days
-Low dose aspirin to decrease the risk of thrombosis.
Why is there an increasxed risk of thrombotic events in patients with GCA?
-Vasculitic conditions is associated with ANCA ,which produces an oxidative burst- which is associated with endothelial dysfunction.
What is the biggest complication of GCA and what happens if it is left untreated?
- Permanent vision loss- (unilateral visual disturbances can- if untreated- cause bilateral blindness)
What is Polymyalgia rheumatica?
-This is when there is inflammation, pain and morning stiffness in the shoulder, neck and hips.
Risk factors for Polymyalgia Rhuematica?
-70 + year old
-Associated with GCA
Symptoms of Polymyalgia Rheumatica?
-Proximal limb pain and stiffness especially in the elderly
-Night time pain
-Morning stiffness
-reduced ROM
-difficulty combing hair or getting out of chairs
-Muscle tenderness
-Normal muscle strength
What are the findings on investigations of PMR?
-ESR,PV and CRP
-temporal artery biopsy (increased risk)
Treatment of PMR?
Usually treat with Prednisolone-15mg daily- slowly taper off- rapid tapering causes symptom relapse- in this case give methotrexate
-Usually should respond to medication immediately- if there is no immediate response reconsider diagnosis. ALWAYS ADJUST MEDS TO SYMPTOMS NOT TO ESR/CRP.
Which other conditions can present with PMR initially ?
-Rheumatoid Arthiritis
-Cancer
why is methylprednisolone given to patients with acute visual problems in GCA?
its stronger than Prednisolone
What does spondyloarthropathies comprise of?
-Ankylosing spondilitis
-reactive arthiritis
-Enteropathic arthiritis
-Psoriatic arthropathies
-HLA-B27+ increased liklihood
What are the similar clinical features of Spondyloarthropathies?
-sacroiliac/axial pain - back and buttock pain
-Arthropathy- joint disease
-Enthesitis- this is tendon inflammation
-Extra-articular features- eye, gut, skin
What is ankylosing spondylitis?
This is when there is inflammation in the spine
Symptomatic presentation of ankylosinf spondylitis?
-Patients- usually male teens or young adults- present with bilateral buttock pain and thoracic (chest) pain and chest wall pain
What are the extra-articular features of ankylosing spondylitis?
Anterior uveitis
Aortic incompetence
AV block
amyloidosis
apical lung fibrosis
What can we find on examination of a patient with ankylosing spondylitis?
-Usually patients have normal examination- its only later that they lose lordosis in their lumbar spine- therefore straightens out and excessive kymphosis in thoracic region- UPSIDE DOWN J.
What are the investigations for Ankylosing Spondylitis?
-Schober’s test- 10cm above the PSIS and 5 cm below- then get them to bend down any distance increase to more than 20cm is normal.
-CRP- this is usually normal
-MRI spine and SI joints
What is the treatment for Ankylosing Spondylitis?
-NSAIDs, Physio, TNF- i and IL-17 inhibitors
What is the typical findings of Psoriatic arthritis?
-(male:female ratio is equal)
-Oligio-arthiritis with dactylitis, which can be symmetrical or asymmetrical (mono arthiritis)
-arthiritis mutilans- this is when the fingers curl and deform is severe very rare forms of psoriatic arthiritis- this is called pencil in a cup deformity
-CRP raised
Treatment for psoriatic arthiritis?
-NSAIDs
-DMARD- disease modifying anti-rheumatic drugs
-TNF-inhibitors
IL-17,IL-12/27
What is reactive arthiritis?
-Asymmetrical arthiritis usually of the lower limbs following either dysentery or cervicitis/urethritis.
What are the symptoms associated with reactive arthritis?
-keratoderma blenorrhagica- mucousy secretions from thickened skin on palms and soles of feet
-Uveitis
-balanitis
-enthisitis
What are the investigations and treatment for Reactive arthritis?
-Aspirate- to look for septic arthiritis
-Inflammatory markers should be raised
Treatment:
treat underlying cause- give NSAIDs and joint infections- symptoms should resolve in two years- if it isn’t resolving consider giving DMARDS- especially in HLA-B27+
What are the types of Enteropathic arthritis?
Type 1- this is oligoarthritis, with very strong association with the IBD flare-up
-Type 2- This is polyarthritis with less association with IBD flare-up
What are the causes of Enteropathic arthritis?
-Usually the cause of this type of arthritis is associated with IBD
Treatment of enteropathic arthritis?
-DO NOT USE NSAIDS- this will increase the flare ups of IBD
-Use TNF-i
-DMARDS
Inflammatory back pain symptoms?
IPAIN
insidious onsets
Pain at night- usually gets better when getting back up
Age of onset more than 40
Improves with exercise
No improvement with rest
What is amyloidosis?
This is the build up of amyloid in organs-
What are the symptoms of Systemic lupus erethymatosus?
gloves and sweater approach
- raynauds
-oral ulcers
-mouth dryness
-malar rash
-truncal rash
-Photosensitive rash
SOAP BRAIN
Serositis
Oral ulcers
Arthritis
photosensitivity
Blood disorders- thrombocytopenia, lymphopenia
Renal- glonerulonephritis
ANA positive ( and dsDNA positive)
Immunological tests
Neurological disorders- seizures,psychosis
What are the investigations associated with SLE?
ANA, dsDNA Anti-Ro,Anti-La
antiphospholipid syndrome
raised inflammatory markers- ESR,PV and CRP
-low c4,c3
-Renal biopsy
Skinbiopsy
-Urine analysis
Why are Complements low in SLE?
-They are low because in anay inflammatory condition there is an increased consumption of complements- therefore fewer in the blood
Why is there an increased risk of pregnancy loss in Antiphophospholipid syndrome
Because it prevents the zygote from implanting into the endometrium properly- and can affect the growth of the foetus
What is the treatment fo SLE?
-hydroxychloroquine for rash
-Sun screen
-DMARDS
-for flares use prednisilone
-give advice on the CVD risk
Why is there an increased risk of CVD in SLE?
-Because the inside of vessels are damaged by inflammatory processes- causes coronary diseases.
What are the risk factors of LUPUS?
-Being a woman - this makes you 9* more likely to develop LUPUS- increased levels of estrogens increase autoimmunity- estradiol increases the lifespan of autoreactive T and B -cells -therefore increases disease processes
-Being a person of colour- they also have a worse prognosis
-being on isoniazid, minocycline and TNF I
-UV light exposure
Why does UV light increase your risk of SLE?
-it damages the DNA- causes mutations which makes it more immunogenic.
What is Raynauds?
-This is when there’s vasospasms:
-white- stasis of blood
blue- venous stasis
-Red- re- warming hyperaemia
When should you suspect underlying disease in Raynauds?
-Usually in females- young adults and teens- its normal and then after 30 year olds start to suspect underlying disease
What conditions is Raynauds associated with?
-SLE
-Scleroderma
Dermatomyositis
-polymyositis
Sjogren’s
What are the physical causes of SLE?
-vibrating tools
-cryoglobinaemia
-Cervical rib
-bet blockers
What is cervical rib?
this is when theres an abnormal cervical rib- which applies pressure on the vessels, also there is an anomalous rib on the otherside
Treatment of Raynauds phenomenon?
-Calcium channel blockers- this helps vessel vasodilate
-phosphodiesterase-5 inhibitors- this binds onto PDE5 on vessels to help them relax and increase blood flow through pulmonary vessels
What are the complications of Raynauds?
-ischaemia
-ulcers
-infection
Symptoms of raynauds?
-Nail fold capillaries
-symmetrical raynauds/ asymmetrical- and only affecting a few digits if there’s underlying pathology
What is vasculitis?
This is when there is inflammation of vessels which predisposes patients to ischaemia, thrombosis and aneurysm formation
What might you expect in a history of a patient with vasculitis?
Resp:-Dyspnoea,cough
-oral ulcers, nose bleeds, crusts
CNS- Neuropathy, encephalopathy, stroke seizures, tinnitus, vision loss/disturbance
-Malaise- a general uncomfortable feeling
-Fatigue
-CVS- pericarditis, chest pain , haemoptysis
-Raynauds
-Limbs- Myalgia,arthralgia, digital ischaemia/ulcers
-GI- abdo pain
-Renal-Haematuria
What might you see on examination of a patient with vasculitis?
-Resp-Crackles, pleuritic rub
-CVS- murmers,arrythmia
Neurological exams- ocular exam- examine for uveitis, episcleritis and also do cranial nerve exam
-Peripheral nerves exam
-Abdo exam- look for tenderness and organomegaly
-Skin- look for rash, nail fold cappillaries, livedo reticularis, gangrene, nodules
-Vital signs- blood pressure and pulse
What are the types of vasculitis?
-Microscopic- eosinophilic, IgA vasculitis, granulomatosis polyangiitis
-Medium-vessel vasculitis- Polyarteritis Nodosa, Kawasaki disease
-Large-vessel Vasculitis- Takayasu arteritis, Giant cell arteritis
What is kawasaki disease?
it affects children under the age of 5 and it causes lymph node swelling and swelling of all the blood vessels in the body - causing high fever and rash and swollen limbs
What is takayasu arteritis?
it is a type of large vessel vasculitis, and it is relatively rare and causes inflammation in the largest arteries in the body - the aorta and its branches
What is polyarteritis nodosa?
It’s a medium- vessel vasculitis, that is thought to be caused by streptococcus bug.
What are the two broad categories of vasculitis?
Primary- uncommon
Secondary- post infection, drugs, malignancies
What are the investigations we do for vasculitis?
-FBC-
-ANCA,ANA, RF
-U&E’s
-LFTS
-PV, ESR, CRP
C3,C4
-Cryoglobulins
-CK, ECG, Blood cultures
-Serum and urine PCR
-CXR, CT, PET
-URINE DIP AND MICROSCOPY to rule out renal involvement
What is the treatment for vasculitis?
stop the offending medication
1st line- corticosteroids
2nd line- start cytotoxic meds- azathioprine, mycophenolate mofetil and rituximab and IVIG
Which condition mimics the findings of Vasculitis?
Atrial myxoma- which is a tumour on the heart- this can be ruled out with ECHO.
What are dermatomyositis and polymyositis?
This is an idiopathic condition in which there is inflammation of striated muscle- onset is insidious and the peak age of incidence is 40-50years old. Equally likely in women and men.
What are the symptoms of Dermatomyositis and polymyositis?
-Rash
-Breathlessness
-Gradual proximal limb weakness with no pain
-Scleroderma/ SLE
-Mechanics hand
-Heliotropes rash
-Gottrons papules
-peri-orbital oedema
-Photosensitive rash in DM- scalp, neck face can cause hyper/hypo pigmentation
What are gottrons papules?
-These are papules on the back of the hand- linear plaques
Heliotropes rash?
This is a blueish rash around the eyes
What is Mechanics hand?
This is dilated nail fold capillaries and dry cracked palms and fingers
What is the diagnostic requirements for DM AND PM?
- Symmetrical proximal weakness
-Raised serum muscle enzymes
-EMG-electromyography changes- measures muscle responses to nervous stimuli
-Biopsy evidently shows myositis
-Rash typical of dermatomyositis
it is PM If 3/ the first 4
It is DM if 2/ of the first 3
What are the typical findings on investigation for PM/DM?
-can have raised inflammatory markers- but more often than not- normal inflammatory markers.
-Have raised ALT but normal liver enzymes- suggests enzymes being released from muscle breakdown
-Auto-antibodies raised- anti-jo1 anti-Mi2
-Anti-nuclear antibody positive
Myositis visible on MRI
Treatment for PM and DM?
-high dose corticosteroids for a couple of weeks- monitor disease activity via inflammatory markers, EMG and CK
Methotrexate and azathioprine - long term
DM- use Hydroxychloroquine and sun protection
What are the complications associated with DM and Pm?
Patients with DM- are more likely to develop malignancies-
-DM and PM can cause diaphragmatic failure- which causes respiratory failure
-Can cause aspiration pneumonia through oesophageal failure (oesophagus is striated muscle)
What is Sjorgen’s Syndrome?
This is a chronic inflammatory disorder- which causes loss of function of lacrimal and salivary glands- can be primary - unlikely or secondary to rheumatic disease- most patients are women
What are the symptoms for Sjorgens?
MAD FRED
Myalgia
Athralgia
Dry eyes
Fatigue
Raynauds
Enlarged parotids
Dry mouth
Post patients have xerostomia and xeropthalmia and fatigue
What would you find on investigation of a patient with Sjorgens?
Anti-Ro, Anti-La,RF or Anti- dsDNA
Schirmers test- measure volume of tears
Salivary gland biopsy- classic lymphocytic infiltration
What is the treatment for Sjorgens?
Vaginal lubricant
Artificial tears
Pastilles or artificial saliva
emollients
avoid smokey dry areas
-Immunosuppressants/steroids
What is Sjorgens associated with?
-Associated with Billiary cirrhosis
-Autoimmune conditions such as- coeliacs, autoimmune thyroid diseased
What complications can Sjorgens cause?
-Increased risk of malignancies - B-cell lymphoma
-In pregnant women with anti-Ro or anti-La- they will present with increased risk of foetal loss- because antibodies can result in heart block of baby and it can cause the baby to develop SLE
What is hypermobility disorder?
This is when there is pain in patients joints which can move more than normal- the
origin of pain is thought to be because of microtrauma.
What are the risk factors for hypermobility?
-Women- asian
-Familial
-Presents in children or young adulthood and may have had previous subluxations and dislocations
What are the symptoms of Hypermobility syndrome?
-Marfanoid Habitus
-Arachnodactyly
-Drooping eyelids
-Myopia- long-sighted
-Hernia and uterine/ rectal prolapses
-Abnormal skin- this is either papyraceous scars( thin scars on joints), thin striae
-Soft tissue rheumatism
What is the treatment of Hms?
-Working on posture balance
-Splinting
-Strengthening exercises to reduce risk of subluxation
-Paracetamol
What scoring tool is used to diagnose hypermobility?
-Beightons score
- can they bend thumb back
-Can they bend pinkie back
-Can their elbows click out
-Can their knees click back
-Can their hands touch their floor with knees straight
What is Hypermobility syndrome associated with?
Marfans and Ehlers Danlos Syndrome
EDS- 13 types differentiation requires genetic testing
What is the most common complication of Marfans?
Cardiac problems- risk of aortic dissection and regurg
-This is because of defective fibrillin gene
What is Fibromyalgia?
It is condition in which there is chronic widespread pain in all 4 quadrants of the body. Allodynia- this is a heightened response to stimuli
What are the symptoms of Fibrofog?
-Jaw pain and claudication
-Headaches
-unrefreshed sleep
-Numbness
-IBS
-poor concentration - fibrofog
-joint stiffness-
-Fatigue
-depression and anxiety
What are the risk factors for fibromyalgia?
-Affects 5% of the population,women are 9 times more likely to get fibromyalgia than men
-age of onset 40-50
-often after a chronic condition
What is the treatment for Fibromyalgia?
Amitrypytline
Pregabalin
What are tests done to rule out other diagnosises in fibromyalgia?
TFT
U+E
CRP
FBC
ESR
Ca2+
CK
What is systemic sclerosis?
This is an autoimmune condition in which there is abnormal connective tissue growth and vascular damage
What are the two types of systemic sclerosis?
-Diffuse scleroderma:
rarer - but there is a higher risk of mortality- sudden onset skin involvement proximal to the elbow and knees
-Limited scleroderma:- this is when patients have had pre-existing raynauds for 10 years and have developed scleroderma- thickened skin and PA hypertension
CREST syndromw
Calcinosis cutis
Raynauds phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
What are the risk factors for Scleroderma?
being a woman
What are the investigations in systemic sclerosis?
-Anti-centromere antibody for limited
-For diffuse RNA polymerase SCL-70 topoisomerase
-Positive ANA- but its not diagnostic
-X-RAY of hands- calcinosis
-CXR, HRCT ,ECHO -pulmonary
What is the treatment for Systemic sclerosis?
-there is no cure
-Sildenafil, calcium anatagonist for raynauds
-for thickened skin mycophenolate and methotrexate
-ACEi prevent hypertensive crisis and decrease renal mortality
-PPI for any bowel related symptoms
-Prednisolone for flare ups
What is it called when Systemic sclerosis affects one joint only?
Morphoea
What rules out the diagnosis of systemic scleroderma?
-Pulmonary hypertension
-Raynauds
-hypertensive crises
What are patients with diffuse systemic sclerosis at risk of?
-Scleroderma renal crises- can result in accelerated hypertension and renal failure
What is osteoarthiritis?
it is a condition in which there is degeneration of bone and cartilage
What are the three main cause of OA?
1.poor loading on cartilage-
2.normal loading on abnormal cartilage
3.Normal loading- but presence of subchondral bone causes cartilage to break
What are the two key features of OA?
-Loss of elasticity
-Cellularity and proteoglycan content are decreased
What are the risk factors for OA?
-Old age >65
-Women are more symptomatic than men
-trauma
-Obesity
-genetic- for knee and hip OA
Symptoms of OA?
-feeling like the knee will give way
-Inactivity and gelling feeling in the knee
-initially the pain is intermittent but eventually becomes constant
What are the radiological signs of OA?
Loss of joint space
Osteophytes
Subchondral sclerosis
Subchondral cysts
What is the management of OA?
-lifestyle management - weight loss
insoles and a walking stick
-Treatment:
Paracetamol- offered short term
NSAIDs
Topical rubefacients and Capsaicin
-surgery- remember that younger patients are more likely to require revision of surgery again.
What does Nodal OA suggest?
Nodal OA- is very common in women especially In heberdens and boutenniers nodes and also it can be linked to the development of knee OA.
What is osteoporosis ?
It is when there is erosion of the bone - which increases the risk of fractures.
What are the risk factors?
Low weight <58kg and BMI<21
old age >65
woman
Cigarette smoking
excessive drinking
caucasian or south asian
calcium deficiency vit D def
Iatrogenic- corticosteroids
What are the investigaations for Osteoporosis?
-DEXA
-T score if their T score is between -1 to -2.5 indicates osteoporosis
-look at their Z score- which compares their BMD to people of the same age and weight- if it is less than -2 then abnormal
-rib fractures, vertebral fractures and compression fractures without any cause- should spark curiosity
What is the treatment for OSTEOPENIA?
-usually just lifestyle management:
-weight bearing exercises
-Vit D
-reducing alcohol and smoking
-Calcium supplements
What is the treatment for Osteoporosis?
you should give Vit D sand Calcium
1st line-bisphosphonates
2nd line- denosumab
What are secondary causes of osteoporosis?
-IBD- coeliacs,
eating disorder
-hyperparathyroidism
-multiple myeloma
What condition should you suspect in a male patient with osteoporosis?
-underlying disease of hypogonadism- treat with testosterone.
What is Gout?
it is when there is hyperurecaemia which causes inflammatory arhtiritis usually in the metatarsophalangeal joint- PODAGRA
What are the risk factors of Gout?
- male
->40 y/o
-Increased uric acid intake
-fructose intake
-diuretics - increase uric acid
-CCF
-Hypertension
-DM
-Renal disease
-Smoking
What is the general prevention for Gout?
-Maintain optimal weight
-exercise
-reduce alcohol consumption
-smoking cessation
-NSAIDS IS FIRST LINE
-oral steroids
Colchicine
What is the treatment for chronic cases of gout?
-Xanthine oxidase inhibitor- Allopurinol- febuxostat
-Benzobromarone
- the aim is to decrease uric acid levels <360
FOR FLARES- TREATMENT SHOULD BE WITHIN 24 HOURS.
What investigations should you do for gout?
-Aspirate the joint- should find monosodium crystals
-Bifringement of urate crystals - NEGATIVE
What is Pseudogout?
This is when gout is caused by calcium pyrophosphate crystals in older women with OA.
