Rheumatology Flashcards
What are the cardinal features of Scleroderma
excessive collagen production
vascular damage
immune system activation by autoantibody production
What is localised scleroderma?
Causes one of more hard patches of skin - thickened, discoloured, hair loss over patch
What is the pathophysiology behind localised scleroderma
Fibroblasts make too much collagen
Plaque morphea - oval patches
Linear - in some severe cases just beneath the skin, it can cause scarring
Which creams are used in localised scleroderma
Cream containing calcipotriol
Tacromilus ointment
Steroid treatments/ointments
Which autoantibodys are associated with limited systemic scleroderma
Anti-centromere
Where does limited scleroderma affect predominantly
Affects the face and distal limbs predominantly
What is CREST
Calcinosis - calcium deposits usually on fingers
Raynauds Phenomenon - treated by Nifedipine (calcium channel blocker)
oEsophageal dysmotility - collagen deposition and loss of smooth muscle function
Sclerodactyly - thickening of the skin
Telangiectasisa - red spots
Which type of scleroderma has the poorer prognosis
Diffuse
Which antibodies are assciated with diffuse scleroderma
Scl-70 antibodies
Which other body systems are involved in diffuse scleroderma
Heart –> myocardial fibrotic change - secondary to Pulmonary artery hypertension
Blood vessels - Hypertension
Lungs - lung fibrosis –> pulmonary artery hypertension causing
Exertional Dyspneoa
syncope
RV strain
Renal –> due to vascular complications e.g. hypertension, can go into renal crisis with apparently normal BP
MSK - flexion contractures, arthralgia, myalgia and stiffness
Which parts of the skin are involved in diffuse scleroderma
Skin of the trunk
upper arms
thighs
Skin changes within 1 year of Reynauds
What is a renal crisis in scleroderma patients
Characterised by acclerated BP with symptoms - oliguria - headache - fatigue - oedema Investigations show - riase in creatinine - proteinuria - microscopic haematuria
Why is regular BP monitoring important in diffuse scleroderma
Important to monitor it so its known what normal is for the patient as a scleroderma renal crisis can occur in a apparently normal BP but is higher than baseline
How is a scleroderma renal crisis treated
ACE inhibitors
Dialysis if needed
Which investigations would be done in suspected Scleroderma
Bedside: BP, Urine dip - if presenting with renal crisis Bloods FBC U+Es LFTs ESR and CRP Autoantibodies - anti-centromere (limited) Scl-70 (diffuse) Barium swallow Endoscopy - if presenting with dysphagia CXR Lung function tests
Which investigations are done to monitor scleroderma
Regular BP monitoring Renal function BNP Lung function CT chest ECG Endocscopy - dilatation of strictures Cardiac MRI Oesophageal manometry +24hr pH studies
What is psoriatic arthropathy
A condition that affects both the skin and joints
Referred as one of the seronegative spondyloarthropathy - inflammatory arthritis that is negative for RF
Often precedes the development of skin lesions
What are the clinical features of psoriatic arthritis
Oligoarthritis - 2-5 joints affected, usually asymmetrical and weight bearing
Sometimes spinal involvement - typically at the sacrum
Osteolysis - as bone in fingers are lost - telescoping on X ray –> pencil in cup
Plaques on exstensor surfaces - check scalp, natal cleft and umbilicus
Nail changes
- discoloured
- ridging
- onycholysis
- psoriatic nail dystrophy –> pitting, leukonychia
How is psoriatic arthropathy treated
DMARDs - methotrexate and leflunomide
- same as RA
NOT hydroxychloroquine as this exacerbates skin conditions
Anti-TNF alpha
What is rheumatoid arthritis
Systemic inflammatory disorder - affects multiple joints usually 5 or more symmetrically
Autoimmune
causes progressive symmetrical joint destruction esp in hands
Where else in the body can be affected in rheumatoid arthritis
Brain - interleukin 1 and 6 –> fever
Skeletal muscle - protein breakdown
Liver - hepicidin produced due to inflammatory cytokines causing a decrease in iron absorption
Blood vessels - atheroma - strokes, MI and ischaemia
Skin - rheumatoid nodules, necrosis centre surrounded by macrophages (found at pressure points)
Lungs - interstitium –> fibroblasts proliferate causing fibrosis and decrease in gas exchange
PLeural cavity - becomes inflamed and causes pleural effusion (exudative) - SOB
How does RA typically present
Swollen, tender painful joints in hands and feet
Stiffness worse in morning which lasts longer than 30 mins
Systemic symptoms: tiredness, depression
typical patient in a young woman
What is the pathophysiology of RA
Immune cells - activated T cells and plasma cells with antigens reach the joint space
T cells secrete cytokines and recruit macrophages which produce more cytokines and causs granular cells to proliferate
causes thick swollen synovial membrane - which damage cartilage and erodes bone
Joints are red due to increased blood flow
Swollen due to increased blood flow and inflammatory process
painful due to the nerve endings in the joint being irritated by the cytokines
Which specific deformities are associated with RA
Ulnar deviation
Boutonnieres deformity - extensor tendon splits (PIP flexion and DIP hyperextension)
Swan neck deformity - PIP hyperextended and DIP flexion
Bakers cysts - synovial sac bulges into popliteal fossa
Which Score is used in to determine the severity of RA
DAS-28
What is Felty syndrome
Rheumatoid arthritis
Splenomegaly
Agranulocytosis
increased risk of infections - life threatening
How is rheumatoid arthritis investigated
Bedside: BP Bloods - FBC - U+Es - LFTs - CRP - Rheumatoid factor - Anti CCP - May test for other Rheumatological conditions such as SLE e.g anti dsDNA
Imaging
Xray - different to OA
What are the features of RA on XRay
Narrowing of joint space Bony erosions - periarticular erosions Soft tissue swelling Decreased bone density subluxation
How is newly diagnosed RA treated
2 DMARDs - MTX and other
Short course of prednisolone
Which drugs are used to treat RA
DMARDS
- Methotrexate –> pulmonary toxicity, weekly dose
- Hydroxychloroquine - can result in severe and permanent retinopathy, also exacerbates skin conditions
- Sulfasalazine - can be used during pregnancy, give folate
Biologics
e.g. Infliximab TNF inhibitor
Which drugs are used in acute flares of RA
NSAIDs
Glucocorticoids - short term use
When are Biologics used
if there is an inadequate response to at least 2 DMARDs including Methotrexate
How else is RA managed other than DMARDs
Physiotherapy
Analgesia
Surgery
What are some of the ocular manifestations of RA
Keratoconjunctivitis sicca - dryness of the conjunctiva and cornea
Episcleritis
Scleritis - erythema and pain
Corneal ulceration
Keratitis
Iatrogenic: Steroid induced Cataracts and chloroquine retinopathy
What are the risk factors for RA
Smoking
Family history
What are the differentials for RA
Psoriatic Arthropathy
Reactive arthritis - would only affect one or two joints, post infection, uvetitis and urethritis
SLE - other systemic symptoms
Polymyalgia rheumatica - stiffness in proximal muscles rather than joints, rapid onset
Gout - usually seen in first metatarsal
Osteoarthritis - usually weight bearing joints and stiffness wears off <30mins and worse at end of day
What are the side effects of Methotrexate
Anti-folate Myelosuppression Liver cirrhosis Pneumonitis Lung fibrosis
What are the side effects of Sulfalazine
Rashes
Oligospermia
Heinz body anaemia
Interstitial lung disease
What are the side effects of leflunomide
Liver impairment
Interstitial lung disease
Hypertension
What are the side effects of Hydroxychloroquine
Retinopathy
Corneal deposits
Skin condition exacerbation
What is SLE
An inflammatory autoimmune disease in which any tissue can be targeted by autoantibodies
It has a relapsing and remitting course
What are the clinical features of SLE
MD SOAP N HAIR
Malar rash
Discoid rash
Serositis - pleuritis and pericarditis
Oral ulcer
Arthritis
Photosensitivity
Neurological abnormality –> psychosis and seizures
Haematological abnormalities –> Anaemia, thrombocytopenia and leukopenia
ANA positive, Anti ds DNA positive, anti smith - last two are more specific
Immunological abnormality
Renal involvement - abnormal urine protein,, diffuse proliferation, glomerulonephritis
Describe the malar rash seen in patients with SLE
It is across the cheeks but spares the naso-labial folds and appears after sun exposure
What is the pathophysiology behind SLE
Damage done to cells so parts of the nucleus (histones, DNA, proteins) go into circulation
B cells then create antibodies against nuclear antigens
Antinuclear antibodies formed
genetic factors then cause patients body to recognise the ANA as foreign and when they deposit there is an inflammatory reaction
Complement system activated
Type 3 hypersensitivity reaction occurs
Which drugs can cause drug induced SLE
Hydralazine
Isoniazid
Procainimide
Anti-histone antibodies seen
How is SLE managed
Lifestyle: avoid UV exposure Patient education minimise cardiovascular risk factors Pharmacological - Hydroxychloroquine - reduces flares and improves fatigue - Paracetamol/NSAIDs for analgesia - Steroids - in the short term to treat flares - Methotrexate for chronic arthritis - Aziothioprine - moderate disease CNS or renal involvement requires aggressive treatment - high dose steroids - azathioprine or mycophenalate - cyclophosphomide - ciclosporin (may require multiple agents)
Biologics
- used for refractory disease
What are the complications of SLE
Cardiovascular disease
Infection
Antiphospholipid Syndrome
Osteoporosis
How does polymyalgia rheumatica present
Proximal muscle aching and stiffness in the morning
bilateral shoulder pain with bilateral arm weakness - may be difficult to raise arms to brush hair
May have pain in neck or pelvis girdle also
Rapid onset
Typically > 60 years old
mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats
flu like symptoms
What is the pathophysiology of polymyalgia rheumatica
Histology shows vasculitis with giant cells, characteristically skips certain sections of affected artery whilst damaging others
Muscle bed arteries mostly affected
Which investigations are done in polymyalgia rheumatica
Bloods
- FBC - reduced CD8+ T cells
- U+Es
- LFTs
- ESR >40mm/hr
- CK - normal
Imaging
rule out OA
What are the differentials for Polymyalgia rheumatica
Inflammatory disorders: Rheumatoid arthritis Ankylosing Spondylitis Psoriatic arthritis SLE Scleroderma Vaculitis Dermatomyositis polymyositis
Non-inflammatory disorders: OA Drug induced myalgia e.g. statins Rotator cuff disease Infections Malignancy
What is the treatment in CREST syndrome
supportive
endothelin receptor antagonists
phosphodiesterase 5 inhibitors
ACE inhibitors in renal crisis
Which antibodies is CREST syndrome associated with
Anticentromere antibodies
What is the colour change in Reynauds syndrome
White to blue to red
