Musculoskeletal and rheumatology Flashcards
What are the causes of a toe-heel gait?
idiopathic, pyramidal dysfunction, foot drop, tight tendoachilles
What are the causes of a broad based gait?
Toddler, cerebellar
What are the causes of a waddling gait?
Proximal muscle weakness at hip girdle
What are the causes of a. circumducting gait
corticospinal tract lesions (internal hip rotation and flexion at the hip and knee and plantar flexion at the ankle)
What is the main cause of fluctuating lower limb tone?
Extrapyramidal lesions
What questions should you ask as part of pGALS?
Any pain or stiffness in joints, muscles or back?
Any difficulty getting yourself dressed without help?
Any difficulty going up and down stairs?
How do you assess posture and gait in pGALS?
Observe standing in 360 degrees
Walk on your tip toes
Walk on your heels
How do you assess the arms in pGALS?
Put your hands out straight in front of you
Turn your hands over and make a fist
Pinch your index finger and thumb together
Touch the tips of your fingers with your thumb
Squeeze the metacarpophalangeal joints for tenderness
Put your hands together palm to palm
Put your hands back to back
Reach up and touch the sky
Look at the ceiling
Put your hands behind your neck
How do you assess the legs in pGALS
Feel for effusion at the knee
Bend and then straighten your knee - feel for crepitus
Passive movement of the hip
What should you assess after the legs and before the neck and spine in pGALS?
TMJ - open your mouth and put three fingers in your mouth
How do you assess the spine in pGALS?
Touch your shoulder with your ear - observe lateral flexion of the cervical spine
Bend forward and touch your toes - observe curve of the spine
What is the salter Harris classification for?
Growth plate fractures
What is a Monteggia fracture?
ulnar shaft fracture with dislocation of radial head
What is a Galeazzi fracture?
radial shaft fracture and disruption of the inferior radioulnar joint
What is Osgood-Schlatter disease?
Osteochondrosis of the patellar tendon insertion at the knee
Who usually gets Osgood-Schlatter disease?
Physically active adolescent males
How does Osgood-Schlatter disease present?
Knee pain after exercise
Localised tenderness
Sometimes swelling over the tibial tuberosity
Hamstring tightness
How is Osgood Schlatter disease managed?
Resolves with reduced activity and quadriceps strengthening, hamstring stretches and occasionally orthotics
What is chondromalacia patellae?
Softening of the articular cartilage of the patella
Who gets chondromalacia patellae?
Most often affects adolescent females
Associated with hypermobility and flat feet
How does chondromalacia patellae present?
Causes pain when the patella is tightly apposed to the femoral condyles e.g. standing up from sitting or walking up stairs
How is chondromalacia patellae managed?
Rest and quad strengthening
What is osteochondritis dissecans?
Segmental avascular necrosis of the subchondral bone
How does osteochondritis dissecans present?
Persistent knee pain in very active adolescents
Localised tenderness over femoral condyles
What causes the pain in osteochondritis dissecans?
separation of bone and cartilage from the medial femoral condyle following avascular necrosis
What is a possible sequelae of osteochondritis dissecans?
Complete separation of articular fragments can cause loose body formation and symptoms of knee locking or giving way
How is osteochondritis dissecans treated?
Rest and quadriceps exercises; surgery occasionally needed
What is the defect in osteogenesis imperfecta?
Type 1 collagen
How many types of osteogenesis imperfecta are there and which is the mildest?
4 types - 1 mildest
What are the features of osteogenesis imperfecta?
Increased fragility of bones, but fracture healing process is unaffected Short stature Scoliosis Defective dentinogenesis Conductive deafness Ligamentous laxity Blue sclerae and tympanic membranes
What is Perthes disease?
Avascular necrosis of the capital femoral epiphysis of the femoral head due to interruption of blood supply, followed by revascularisation and reossification over 18-36 months
Who gets Perthes disease?
Boys age 5-10
How does Perthes disease present?
Insidiously
Limp, hip or knee pain
Bilateral in 10-20%
What investigation findings would you expect in Perthes disease?
XR of both hips including frog views - May show increased density in the femoral head which then becomes fragmented and irregular
How is Perthes disease managed?
Bed rest and traction
If severe, may need to maintain the hip in abduction with plaster or calipers
Femoral or pelvic osteotomy
What is the problem in SUFE?
displacement of the epiphysis of the femoral head postero-inferiorly - Needs prompt treatment to avoid avascular necrosis
Who gets SUFE?
1-15 year old obese boys
What are the examination findings in SUFE?
Restricted abduction and internal rotation of the hip
How is SUFE managed?
Usually surgically
What are the main types of JIA?
Oligo Polyarthritis: RF -ve and RF +ve Systemic Enthesitis-related Psoriatic
How is oligoarticular JIA defined?
=4 or fewer joints involved within the first 6 months
Who gets oligoarticular JIA?
females between 12 months and 5 years
What are the common features of oligoarticular JIA?
Chronic uveitis (almost always symptomatic but can cause blindness)
Associated with ANA positivity
Knees most commonly affected
What is the prognosis for oligoarticular JIA?
Exacerbations and remissions but long term prognosis is good unless develops into polyarthritis
Who gets polyarticular RF -ve JIA?
Any age
How is polyarticular JIA defined?
= 5 or more joints during the first 6 months
RF must be positive on at least 2 occasions 3 months apart to be RF positive
What are the features of polyarticular RF-ve JIA?
Involves large and small joints, cervical spine, temporomandibular joints Can be symmetrical or asymmetrical Can remit in late childhood ANA may be positive Requires screening for uveiti
Who gets polyarticular RF positive JIA?
Females
Late childahoodearly teens
What are the features of RF positive JIA?
Symmetrical polyarthritis affecting upper and lower limbs
Also features subcutaneous rheumatoid nodules and early erosive radiological changes
What’s the prognosis for RF positive polyarticular JIA?
Unremitting severe disease and poor functional outcome in over 50%
Who gets systemic JIA?
1-4 year olds most often, equal sex incidence
What are the systemic symptoms of systemic JIA?
Fever - typically single or double quotidian pattern with high spikes daily, at similar times every day
Rash - evanescent, coming and going with fever spikes, discrete salmon pink macules usually around upper trunk and axillae; show Koebner phenomenon, rarely pruritic
Polyarticular arthritis - can be a late feature
What are the non-articular features associated with systemic JIA?
Lymphadenopathy Hepatosplenomegaly Serositis (pericarditis, pleuritis) Inflammatory ascites Haematological changes (anaemia, leucocytosis, macrophage activation syndrome) Occasional myocarditis
Which comes first in systemic JIA - the systemic features or the arthritis?
Systemic features can precede by months
What is the prognosis for systemic JIA?
Approx 50% remit in 2-3 years
Untreated, joint destruction occurs in most cases
Most mortality is in this subgroup - Death is due to infection, myocardial involvement or macrophage activation syndrome
Who gets enthesitis-related arthritis?
Pre teenage males
What are the features of enthesitis related arthritis?
Lower limb involvement, especially hips and sacroiliac joints
Many show features common to spondyloarthropathies e.g. enthesitis, decreased lumbar flexion
Involvement of the spine is uncommon in childhood
Can get uveitis, but is usually symptomatic with red eye with pain and photophobia, in contrast to other forms
Which tests are positive/negative in enthesitis related arthritis?
HLA-B27 in 80%
ANA and RF negative
What are the extra-articular features of enthesitis related arthritis?
Anterior uveitis Aortitis Aortic incompetence Muscle weakness Low grade fever
Who gets psoriatic JIA?
Females, mid childhood
What are the features of psoriatic JIA?
Arthritis is asymmetrical and can be oligoarticular or polyarticular
Dactylitis is seen in younger patients and DIP joint involvement is common
Inflammation can involve tendon sheaths- If severe is called arthritis mutilans, which is very destructive
Often a family history of psoriasis
Nail changes - pitting, onycholysis
Psoriasis
Uveitis
Who gets IBD related arthritis?
> 4 years of age, males and females equal
What are the clinical features of IBD related arthritis?
Usually after onset of bowel symptoms
Usually oligoarticular
What are the 2 forms of IBD related arthritis?
Benign peripheral arthritis coinciding with active bowel disease
In older patients in spondylitic group, joint activity doesn’t necessarily link with bowel activity
What are the non-articular features seen in IBD related arthritis?
Erythema nodosum Pyoderma gangrenosum Mucosal ulcers Fever Weight loss Growth retardation Acute iritis in the spondylitic group
Which tests are positive/negative in IBD related arthritis?
HLA-B27 in many
RF and ANA negative
How does juvenile sarcoidosis present?
Painless swelling of joints Marked tenosynovitis Rash Panuveitis Renal disease
What test is often raised in juvenile sarcoidosis?
ACE - in 50%
What is rheumatic fever?
Inflammatory reaction in joints, skin, heart and CNS following group A haemolytic strep infection
Who gets rheumatic fever?
Girls, >3 years
What are the criteria called for diagnosis of rheumatic fever?
Revised jones criteria
What are the major criteria for diagnosis of rheumatic fever?
Carditis Polyarthritis Subcutaneous nodules Chorea Erythema marginatum
What are the minor criteria for diagnosis of rheumatic fever?
Fever Arthralgia Previous rheumatic fever and rheumatic heart disease Raised acute phase reactants Prolonged P-R
How long does rheumatic fever usually last?
6 weeks
How is rheumatic fever managed
Bed rest
Penicillin
Salicylates
Corticosteroids with significant carditis
Prophylactic oral or IM penicillin after an attack required into adult life
What is dermatomyositis?
small-vessel vasculopathy involving arterioles and capillaries
What are the diagnostic criteria for dermatomyositis called?
Bohan and peter
What are the criteria for diagnosis of dermatomyositis?
Characteristic rash
Plus 3/4 of:
Symmetrical proximal muscle weakness
Elevated muscle-derived enzymes: E.g. CK, aldolase, LDH, transaminases
Muscle histopathology confirming the typical pattern of myofibre atrophy and drop out necrosis with fibre regeneration, and chronic inflammatory infiltrate involving blood vessels
EMG changes of inflammatory myopathy: Fasciculations at rest, bizarre high-frequency discharges
What are the characteristic rashes of dermatomyositis?
Gottron's papules Heliotrope rash Scaly red rash on sunexposed areas Shawl sign Rash on extensor tendons - can be mistaken for psoriasis
What are some general, non-diagnostic features of dermatomyositis?
Generalised tissue oedema Skin ulceration Fever Hypernasal voice GI involvement Cardiopulmonary involvement including myocarditis and dilated cardiomyopathy Pulmonary fibrosis Respiratory muscle weakness CNS vasculitis Ophthalmological complications e.g. retinopathy, glaucoma, cataracts Renal failure Raynaud's Lipodystrophy
Which tests can be done in dermatomyositis?
Muscle enzymes
ESR
CRP
Autoantibodies ANA Anti-ds-DNA Anti-Sm Anti-RNP Anti-Jo1 Myositis specific antibodies
Imaging Pulmonary function tests ECG Nail fold capillaroscopy \+/- biopsy
What is the principal problem in SLE?
dysregulation of the innate and adaptive immune system including production of autoantibodies and hypergammaglobulinaemia
Which investigations can be sent in SLE?
ANA Anti-ds-DNA Anti-U1 RNP Antibodies Anti-cardiolipin Complement
What is Behcet?
Clinical triad of:
Recurrent oral aphthous ulcers
Recurrent genital ulcers
Uveitis - severe and can lead to glaucoma and blindness
Who gets Behcet?
Male predominance, Japan, Middle East and Mediterranean
What are some other features of Behcet?
Arthritis
Rash - skin hypersensitivity
Bowel involvement
Meningoencephalitis, brainstem lesions and dementia
How is Behcet treated?
Steroids
Thalidomide
Anti TNF
What are the clinical features of Sjogren syndrome?
Dry eyes (keratoconjunctivitis sicca)
Dry mouth and carious teeth
Parotitis
What causes Sjogren?
May occur alone or in association with other rheumatological conditions
Occasional complication of renal disease or lymphoreticular malignancy
What are the two kinds of scleroderma?
Localised and diffuse/CREST
What are the features of localised scleroderma?
Morphea: thickened shiny pale skin then darkens as resolves with loss of subcutaneous tissue, Can be single or multiple
Linear: thickened plaque which causes loss of subcutaneous tissues and contractures over joints and loss of bone growth:
Face, forehead and scalp (en coup de sabre)
Limb (en bande)
What are the features of CREST?
Calcinosis cutis Raynaud phenomenon Oesophageal hypomobility Sclerodactyly Telangiectasia Systemic problems include respiratory, gastrointestinal, and renal disease
What is Henoch-Schonlein purpura?
Small vessel vasculitis that can be precipitated by infection
Which infection classically causes HSP?
Haemolytic strep
Who gets HSP?
Boys >3 years
What are the clinical features of HSP?
Petechiae
Rash: urticarial lesions evolving into purpuric macules, usually on legs, feet and buttocks
Cutaneous nodules over the elbows and knees
Localised areas of subcutaneous oedema that affect the forehead, spine, genitalia, hands and feet
Arthritis: transient, involving large joints
GI involvement: colicky abdominal pain and/or GI bleeding
Renal involvement: nephritis, occasionally nephrosis
Which marker is often raised in HSP?
Serum IgA
What is the prognosis/management of HSP?
Episodes usually self limiting but can occur
Long term morbidity is related to renal involvement
Severe disease may need corticosteroids
What will you find in an FBC in Kawasaki disease?
polymorph leucocytosis, raised platelets
What are the diagnostic features of Kawasaki?
≥5 days fever Bilateral conjunctival injection Changes in lips and oral cavity Changes in extremities: reddening and oedema of palms and soles followed by desquamation Polymorphous erythematous rash Cervical lymphadenopathy
What are the non-diagnostic features that can be associated with Kawasaki?
Irritability Pericarditis Valvular dysfunction Coronary artery disease Arthritis and/or arthralgia GI symptoms Urethritis Aseptic meningitis Iritis
What is the usual course of Kawasaki?
Acute and convalescent stage lasts up to 10 weeks
What are the possible complications of Kawasaki?
Coronary aneurysms or widening in 20%
Death due to coronary vasculitis causing MI or aneurysm rupture occurs in about 1%
How is Kawasaki managed?
Supportive care Careful observation Salicylates IV gamma globulin Steroids Anti-TNF
What is polyarteritis nodosa?
Small to medium vessel vasculitis
Who gets polyarteritis nodosa?
3-16 years
What are the clinical features of polyarteritis nodosa?
Fever Abdo pain Arthralgia/myalgia Rash: -Petechiae/purpura in generalised form -Tender subcutaneous nodules and livedo reticularis in the cutaneous form Hypertension Renal involvement Neurological disease
What would be the investigation findings in polyarteritis nodosa?
High ESR Low Hb Leucocytosis ASOT elevated in some Focal necrosis in small and medium sized arteries
What is the prognosis in polyarteritis nodosa?
Cutaneous form usually benign but can relapse
Prognosis is worse in generalised form depending on organ involvement
How is polyarteritis nodosa managed?
High dose steroids
Immunosuppression
Penicillin prophylaxis if streptococcal aetiology proven
What is takayasu disease?
aka giant cell arthritis: a panarteritis of the aorta and its large branches leading to thrombosis, stenosis or occlusion
Who gets takayasu disease?
Primarily affects young adult women, more common in East Asian and Black people
What are the clinical features of takayasu disease?
Claudication Myalgia Hypertension Malaise Fever
How is takayasu disease managed?
Steroids +/- cytotoxic and reconstructive surgery when the disease is inactive
What is Churg-Strauss syndrome?
Systemic necrotising vasculitis of small arteries and veins, accompanying asthma and eosinophilia
What are the clinical features of Churg-Strauss syndrome?
Lungs: asthma and transient pulmonary infiltrates
Rash: palpable purpuras and tender subcutaneous nodules
Peripheral neuropathy
Renal involvement
What is Wegener granulomatosis?
Necrotising granulomatous vasculitis of the upper and lower respiratory tracts, accompanied by glomerulonephritis
What are the clinical features of Wegener granulomatosis?
Pulmonary granulomas Destructive granulomas of the ears, nose and sinuses Rash Glomerulonephritis Eye lesions
Which antibodies are positive in Wegener granulomatosis?
Anti neutrophil cytosolic antibodies
How is Wegener granulomatosis treated?
Steroids and cyclophosphamide
What are the features of Poland syndrome?
Absent or hypoplastic pectoral muscle Shoulder abnormalities Limb abnormalities Hypoplasia or aplasia of the breast or nipple Aplasia of ribs Syndactyly/bradydactyly Absent fingers Short middle phalanges
Which side is commoner in Poland syndrome?
Right
What is associated with left sided Poland syndrome?
Dextrocardia
Which conditions are associated with Poland syndrome?
Moebius
Klippel Fiel
Non Hodgkins lymphoma
Acute leukaemia
