Rheumatology Flashcards
what is SLE (systemic lupus erythematosus)
inflammatory autoimmune connective tissue disease
has varying and non-specific symptoms
more common in women and Asians, usually presents in young to middle aged adults but can present later in life
pathophysiology of SLE
anti-nuclear antibodies, antibodies to proteins within the person’s own cell nucleus
results in chronic inflammation, resulting in a shortened life expectancy
presentation of SLE
fatigue
weight loss
arthralgia
myalgia
fever
photosensitive malar rash, livedo reticularis, discoid rash
lymphadenopathy, splenomegaly
SOB
pleuritic chest pain
mouth ulcers
hair loss
Raynaud’s
autoantibodies associated with SLE
anti-ANA (99% of patients will have this, good rule out test, but positive in other conditions as well e.g. autoimmune hepatitis)
anti-dsDNA = specific to SLE
20% of patients have rheumatoid factor positive
diagnosis of SLE
SLICC criteria/ ACR criteria
confirming antinuclear antibodies and a certain number of clinical features suggestive of SLE
babies whose mothers have anti-Ro and anti-La antibodies are at an increased risk of developing what?
neonatal lupus
monitoring SLE investigations
ESR, (CRP during active disease may be normal)
complement levels C3 & 4 decreased
anti-dsDNA titre levels used for monitoring
treatment for SLE
anti-inflammatory medication and immunosuppression
first line:
NSAIDs
Steroids e.g. prednisolone
Hydroxychloroquine (first line for mild SLE)
suncream and sun avoidance
more resistant/severe lupus:
methotrexate
azathioprine
leflunomide
then biological therapies e.g. rituximab, belimumab
what is spared regarding the malar rash in SLE
nasolabial folds
risk factors for osteomalacia
darker skin
reduced sun exposure
chronic kidney disease
malabsorption disorders e.g. IBD
drug induced e.g. anticonvulsants
inherited
pathophysiology of osteomalacia
vitamin D = a hormone created from cholesterol by the skin in response to UV radiation
inadequate vitamin D results in lack of calcium and phosphate in the blood as it is not absorbed from the intestines and kidneys
low calcium causes a secondary hyperparathyroidism
complications of SLE
** all due to chronic inflammation
CVD - hypertension, coronary artery disease resulting in leading cause of death
Infection
Anaemia of chronic disease = chronic normocytic anaemia
Pericarditis
Pleuritis
Interstitial lung disease = pulmonary fibrosis
Lupus nephritis
Neuropsychiatric SLE = optic neuritis, transverse myelitis, psychosis
Recurrent miscarriage
what is osteomalacia
defective bone mineralisation causing soft bones secondary to low vitamin D levels
if this occurs in growing children it is termed rickets
what organ is responsible for metabolising vitamin D to its active form
kidneys
presentation of osteomalacia
fatigue
bone pain
bone/muscle tenderness
muscle weakness
fractures e.g. especially femoral neck
proximal myopathy = waddling gait
investigations for osteomalacia
vitamin D deficiency (<25nmol/L)
low calcium, low phosphate
high ALP
secondary hyperparathyroidism
x-rays may show osteopenia (more radiolucent bones)
management of osteomalacia
supplementary vitamin D (colecalciferol)
** a loading dose often needed initially
calcium supplementation if dietary calcium inadequate
what is polymyalgia rheumatica?
inflammatory condition causing pain and stiffness in the shoulders, pelvic girdle, and neck
strong association with giant cell arteritis
demographics associated with polymyalgia rheumatica
older age >50 yrs
more common in women, Caucasians
features of polymyalgia rheumatica
should be present for at least 2 weeks: usually rapid onset
bilateral shoulder pain that may radiate to the elbow
bilateral pelvic girdle pain
worse with movement
interferes with sleep
stiffness for at least 45 mins in the morning
** weakness is not a symptom
can be associated with fever, night sweats, low mood, anorexia, fatigue
investigations for polymyalgia rheumatica
inflammatory markers e.g. CRP, ESR are raised, but diagnosis mainly based on clinical presentation and response to steroids
treatment for polymyalgia rheumatica
started on 15mg of prednisolone
if 3-4 weeks has given a good response, then start a reducing regime, aim is to get patient off steroids
long term steroids advice: Don’t STOP
don’t stop taking steroids after 3 weeks as will have become steroid dependant and don’t want an adrenal crisis
S = sick day rule, increase steroid dose if become unwell
T = treatment card to alert others
O = osteoporosis, think about prophylaxis with biphosphonates, calcium, vitamin D
P = PPI
what is antiphospholipid syndrome
acquired disorder resulting in a hyper-coagulable state where they have a predisposition to venous and arterial thromboses
associated with recurrent miscarriages and thrombocytopenia
can occur on its own or secondary to an autoimmune condition e.g. SLE
autoantibodies associated with antiphospholipid syndrome
lupus anticoagulant
anticardiolipin antibodies
anti-beta-2-glycoprotein I antibodies
features of antiphospholipid syndrome
VTE = DVT, PE
Arterial thromboses = stroke, MI, renal thrombosis
pregnancy complications = recurrent miscarriage, stillbirth, pre-eclampsia
livedo reticularis
thrombocytopenia
prolonged APTT
Libmann-Sacks endocarditis = non-bacterial endocarditis with growths on the valves, particularly mitral valve
management of antiphospholipid syndrome
primary thromboprophylaxis = low dose aspirin
secondary =
initial venous and arterial events, lifelong warfarin with INR of 2-3
recurrent venous = warfarin INR 3-4, consider adding aspirin
if pregnant = low molecular weight heparin e.g. enoxaparin, and aspirin
What is psoriatic arthritis
Form of inflammatory arthritis, associated with psoriasis
Occurs in 10-20% of patients
Part of the seronegative spondyloarthropathy
What is rheumatoid arthritis
An autoimmune condition that causes chronic inflammation of the synovial lining of the joints, tendon sheaths, and bursa
Form of inflammatory arthritis
Symmetrical polyarthritis
Demographics of RA
More common in women
Develops in middle age, but can present at any age
FHx increases the risk
Patterns of affected joints in PA
Symmetrical poly arthritis = hands, wrists, ankles, and DIP joints
MCP joints are less effected, unlike in RA
Asymmetrical pauciarthritis = affecting mainly the digits and feet (when the arthritis only affects a few joints)
Spondylitic pattern = often seen in men, causing back stiffness, sacroilitis, Atlanta-axial joint involvement
Other areas can be affected = spine, Achilles’ tendon, plantar fascia
Nail signs and other associated signs of PA
Pitting of nails
Psoriatic plaques
Onycholysis
Dactylitis
Enthesitis (point of insertion of tendons onto bones)
Eye disease = conjunctivitis, anterior uveitis
Aortitis
Amyloidosis
What is the varying severity of PA
mild stiffening or soreness
Or the joint can be completely destroyed in arthritis mutilans
What screening tool is recommended for PA
PEST
Psoriatic epidemiological screening tool
What is arthritis mutilans
Most severe form of psoriatic arthritis occurring in the phalanxes
There is osteolysis of the bones around the joint in the digits, leading to progressive shortening of the digit and the skin folds
‘Telescopic finger’
Changes seen on X-ray in psoriatic arthritis
Dactylitis (soft tissue swelling)
Osteolysis
Periostitis
Pencil in cup appearance
Management of PA
NSAIDs for pain
DMARDs e.g methotrexate (treats skin plaques as well), leflunomide, sulfasalazine
Anti-TNF e.g. etanercept, infliximab, adalimumab
Last line = ustekinumab, a monoclonal antibody targeting interleukin 12 and 23
