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Rheumatology

QuestionAnswer
Abatacept fusion protein that modulates a key signal required for activation of T lymphocytes leads to decreased T-cell proliferation and cytokine production given as an infusion not currently recommend by NICE
Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an autosomal recessive disorder which typically presents by the second decade. It is more common in people of Turkish, Armenian and Arabic descent Features - attacks typically last 1-3 days pyrexia abdomi
Rituximab anti-CD20 monoclonal antibody, results in B-cell depletion two 1g intravenous infusions are given two weeks apart infusion reactions are common
Polyarteritis nodosa (PAN) is a vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation. PAN is more common in middle-aged men and is associated with hepatitis B infection. Features fever, malaise, arthralgia weight loss hypertension
TNF-inhibitors the current indication for a TNF-inhibitor is an inadequate response to at least two DMARDs including methotrexate etanercept: recombinant human protein, acts as a decoy receptor for TNF-α, subcutaneous administration, can cause demyelination, risks inclu
Denosumab a human monoclonal antibody that inhibits osteoclast formation, function and survival. It is given as a subcutaneous injection, at a dose of 60mg, every 6 months. A larger dose of denosumab (120mg) may also be given every 4 weeks for the prevention of
Raloxifene selective oestrogen receptor modulator (SERM) has been shown to prevent bone loss and to reduce the risk of vertebral fractures, but has not yet been shown to reduce the risk of non-vertebral fractures has been shown to increase bone density in the spine
Strontium ranelate 'dual action bone agent' - increases deposition of new bone by osteoblasts (promotes differentiation of pre-osteoblast to osteoblast) and reduces the resorption of bone by inhibiting osteoclasts any history of cardiovascular disease or significant risk o
Teriparatide recombinant form of parathyroid hormone very effective at increasing bone mineral density
Allopurinol is particularly recommended if: → >= 2 attacks in 12 months → tophi → renal disease → uric acid renal stones → prophylaxis if on cytotoxics or diuretics
PMR ttt explanation aerobic exercise: has the strongest evidence base cognitive behavioural therapy medication: pregabalin, duloxetine, amitriptyline
Dermatomyocytis Investigations the majority of patients (around 80%) are ANA positive around 30% of patients have antibodies to aminoacyl-tRNA synthetases (anti-synthetase antibodies), including: antibodies against histidine-tRNA ligase (also called Jo-1) antibodies to signal recognit
Dermatomyocytis features Skin features photosensitive macular rash over back and shoulder heliotrope rash in the periorbital region Gottron's papules - roughened red papules over extensor surfaces of fingers nail fold capillary dilatation Other features proximal muscle weakness
Immunology of SLE 99% are ANA positive 20% are rheumatoid factor positive anti-dsDNA: highly specific (> 99%), but less sensitive (70%) anti-Smith: most specific (> 99%), sensitivity (30%) also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
Drug causes of SLE Most common causes procainamide hydralazine Less common causes isoniazid minocycline phenytoin
X ray findings in Ankylosing Spondylitis sacroiliitis: subchondral erosions, sclerosis squaring of lumbar vertebrae 'bamboo spine' (late & uncommon) syndesmophytes: due to ossification of outer fibers of annulus fibrosus chest x-ray: apical fibrosis
Complications of RA respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced ca
Indications for urate-lowering therapy → >= 2 attacks in 12 months → tophi → renal disease → uric acid renal stones → prophylaxis if on cytotoxics or diuretics
RA x rays Early x-ray findings loss of joint space juxta-articular osteoporosis soft-tissue swelling Late x-ray findings periarticular erosions subluxation
deafness (cranial nerve entrapment) bone sarcoma (1% if affected for > 10 years) fractures skull thickening high-output cardiac failure
Azathioprine Azathioprine is metabolised to the active compound mercaptopurine, a purine analogue that inhibits purine synthesis. A thiopurine methyltransferase (TPMT) test may be needed to look for individuals prone to azathioprine toxicity. Adverse effects include
Investigations of dermatomyocytis the majority of patients (around 80%) are ANA positive around 30% of patients have antibodies to aminoacyl-tRNA synthetases (anti-synthetase antibodies), including: antibodies against histidine-tRNA ligase (also called Jo-1) antibodies to signal recogni
Inflammatory infiltrates and inclusions within muscle fibres = inclusion body myositis
Endomysial lymphocytic infiltrates that invade nonnecrotic muscle fibres = polymyositis Perimysial inflammation of lymphocytes and parafascicular atrophy = Endomysial lymphocytic infiltrates that invade nonnecrotic muscle fibres = . polymyositis
Perimysial inflammation of lymphocytes and parafascicular atrophy = dermatomyositis
Investigations of Polymyocitis? elevated creatine kinase other muscle enzymes (lactate dehydrogenase (LD), aldolase, AST and ALT) are also elevated in 85-95% of patients EMG muscle biopsy anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud's an
X-ray changes of osteoarthritis decrease of joint space subchondral sclerosis subchondral cysts osteophytes forming at joint margins
Indications to treat Paget disease Indications for treatment include bone pain, skull or long bone deformity, fracture, periarticular Paget's
Adult Onset Stills disease features Features arthralgia elevated serum ferritin rash: salmon-pink, maculopapular pyrexia: typically rises in the late afternoon/early evening in a daily pattern and accompanies a worsening of joint symptoms and rash lymphadenopathy rheumatoid factor (RF) and
Adult Onset Stills disease diagnosis Yamaguchi criteria
Paget ttt bisphosphonate (either oral risedronate or IV zoledronate) calcitonin is less commonly used now
Adverse effects of Azathioprine include bone marrow depression nausea/vomiting pancreatitis increased risk of non-melanoma skin cancer
Ehler-Danlos syndrome Ehler-Danlos syndrome is an autosomal dominant connective tissue disorder that mostly affects type III collagen. This results in the tissue being more elastic than normal leading to joint hypermobility and increased elasticity of the skin. Features and
McArdle's disease Overview autosomal recessive type V glycogen storage disease caused by myophosphorylase deficiency this causes decreased muscle glycogenolysis Features muscle pain and stiffness following exercise muscle cramps myoglobinuria low lactate levels during exe
Features of Behcet classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis thrombophlebitis and deep vein thrombosis arthritis neurological involvement (e.g. aseptic meningitis) GI: abdo pain, diarrhoea, colitis erythema nodosum
Radiological features of gout include: joint effusion is an early sign well-defined 'punched-out' erosions with sclerotic margins ina juxta-articular distribution, often with overhanging edges relative preservation of joint space until late disease eccentric erosions no periarticular osteopen
Polymyositis Overview inflammatory disorder causing symmetrical, proximal muscle weakness thought to be a T-cell mediated cytotoxic process directed against muscle fibres may be idiopathic or associated with connective tissue disorders associated with malignancy dermatomyosit
Medications that may worsen osteoporosis (other than glucocorticoids): SSRIs antiepileptics proton pump inhibitors glitazones long term heparin therapy aromatase inhibitors e.g. anastrozole
Risk Factors of Osteoporosis history of glucocorticoid use rheumatoid arthritis alcohol excess history of parental hip fracture low body mass index current smoking Other risk factors sedentary lifestyle premature menopause Caucasians and Asians endocrine disorders: hyperthyroidism,
Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an autosomal recessive disorder which typically presents by the second decade. It is more common in people of Turkish, Armenian and Arabic descent Features - attacks typically last 1-3 days pyrexia abdomi
Investigation of Sjogren rheumatoid factor (RF) positive in nearly 100% of patients ANA positive in 70% anti-Ro (SSA) antibodies in 70% of patients with PSS anti-La (SSB) antibodies in 30% of patients with PSS Schirmer's test: filter paper near conjunctival sac to measure tear f
the 'A's of Ankylosing Spondylitis Apical fibrosis Anterior uveitis Aortic regurgitation Achilles tendonitis AV node block Amyloidosis and cauda equina syndrome peripheral arthritis (25%, more common if female)
Langerhans cell histiocytosis Langerhans cell histiocytosis is a rare condition associated with the abnormal proliferation of histiocytes. It typically presents in childhood with bony lesions. Features bone pain, typically in the skull or proximal femur cutaneous nodules recurrent
Created by: Dr.Selma