Hip Examination

Intro

• Wash hands, Introduce self, Check patient name and DOB/CHI, Explain procedure and gain consent

Look

• General: age, mobility, mobility aids, trauma etc
• Standing:
  • Posture, asymmetry, muscle wasting, scars, deformity (also look at the back for scoliosis and the gluteal muscles
  • Trendelenburg’s test
    • Look at the patient’s ASIS and ask them to stand on one leg (you may want to be ready to support them)
    • Normally the gluteals will contract so that the contralateral (unsupported) leg rises to balance.  If the contralateral side dips, the abductor muscles are weak on the side they are standing.
• Gait:
  • Speed, limp, arm swing, abnormal gaits e.g. Trendelenburg (wide-based waddle); antalgic
• Lying
  • Look at leg length, comparing symmetry and rotation, scars, sinuses, skin changes etc
  • Measure apparent (xiphisternum/umbilicus to medial malleolus: if unequal, spinal or pelvic problem) and true leg length (from ASIS to medial malleolus: if unequal, true limb shortening e.g. fracture)

Palpate/Feel

• Start on normal side
• Bony landmarks (where possible) e.g. greater trochanter, ASIS, pubic rami
• Temperature and skin etc

Move

• Do all movements actively (except rotation) then passively
• Test leg movement roughly by rolling the legs gently on the bed
• Flexion (130°); (NB Extension is not routinely tested on the couch);
• Test Abduction (45°) and adduction (30°)
  • NB Abduction/adduction is not always tested actively
• Test Internal rotation passively by flexing the hip and knee to 90° and rotating the leg so that the knee points inwards (vice verse for external rotation)
  • Internal rotation lost early in osteoarthritis

NB Some practitioner’s will still perform Thomas’ test for fixed flexion deformity.  However, in Tayside, at least, it is becoming less routine and is no longer part of the hip examination.

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Knee Examination

Intro

• Wash hands, Introduce self, Check patient name and DOB/CHI, Explain procedure and gain consent

Inspection

• General: patient; mobility aids
• Gait: any antalgic gait, limp, locking knee
• Standing: alignment, varus/valgus deformity, swelling, redness, asymmetry
• Lying: skin (scars, swelling, bruising); joints (effusion, nodules, psoriasis/skin changes); alignment and position
• Measure quadriceps bulk (hand’s breadth above the patella) (NB not always performed)

Feel

• Skin- temperature
• Joint
  • with the knee at 90° flexion, feel the patella, along the joint line
  • note any tenderness, synovial thickening etc
  • also feel for any abscesses/popliteal bursitis
• Effusion
  • Patellar tap
    • Empty the suprapatellar pouch with one hand and push down on the patella with one/two finger(s) of the other hand (positive if the patella sinks before hitting the femur)
  • Cross fluctuation may be performed if there is a large effusion
    • Empty the suprapatellar pouch with the other hand below the patella.  Positive if the pressure/ripple is felt with either hand with alternating compressions
  • For smaller effusions (more common) the bulge test is performed
    • ‘Sweep’ the fluid out of the patella bursa, beginning infero-medially and moving superiorly (superomedial aspect); the repeat laterally beginning superiorly and ending inferiorly.  Positive it there is a ‘bulge’ on the medial side after emptying when the lateral side is swept.

Move

• NB Good to compare with the non-affected knee- test active and passive movements (palpating the joint with passive movement)
  • Flexion (normal 140°) and Extension (normal 0°) (also lift the foot off the bed and look for any hyperextension (normal up to 10°))
• Special tests
  • Collateral ligament stress tests
    • Hold the ankle/lower leg in one arm, and, with the knee at 0 and 30° flexion, apply varus (LCL) and valgus (MCL) stresses
    • Excessive movement suggests tear
  • Cruciate ligaments
    • Drawer test
      • With the knee flexed at 90° and the foot on the bed (often useful to sit on the patient’s foot to support it), with your fingers in the popliteal fossa and thumbs on the tibial tuberosity, apply anterior (ACL) and posterior (PCL) forces on the tibia
    • Lachman’s test
      • with the knee slightly flexed, one hand supporting the thigh, pull the calf anterior (ACL laxity)
  • Menisci
    • McMurray’s test
      • Flex the knee as much as possible, externally rotate the foot, apply a lateral force to the knee and extend the leg (med meniscal stress)
      • Flex the knee, internally rotate and apply a medial force and extend the leg (lat meniscal stress)
    • Apley’s manoeuvre (rarely done in practice)
      • With the patient prone (on front); apply downwards force to knee whilst flexed at 90°, and rotate the foot.
  • Patellofemoral apprehension
    • Flex the knee whilst pushing on the patella laterally

Finish

• Thank patient, wash hands,
• Document/explain findings and suggests further investigations/management

Kawasaki Disease

Background

• Idiopathic, self-limiting vasculitis predominantly seen in children under 5.
  • More common in Asian populations
  • Still uncommon (8/100,000)- although potentially serious (see complications)

Features

• Classical
  • Fever >5 days (usually high-grade and paracetamol resistant)
  • Marked irritability
  • Erythema, swelling and desquamation affecting the skin of the extremities
  • Bilateral conjunctivitis
  • Rash
  • Inflammation of the lips, mouth and/or tongue (strawberry tongue)
  • Cervical lymphadenopathy
• Symptoms can gradually improve over several weeks

Investigations

• Clinical diagnosis (often quite apparent) but generic investigations including FBC, LFT and U&Es can be included as part of the workup to exclude infection and other causes)
• Echocardiogram is important to screen for coronary aneurysms, which can increase the risk of myocardial infarction and acute coronary syndromes

Management

• Aspirin (high dose)
  • NB ASPIRIN IS NOT ROUTINELY RECOMMENDED FOR USE IN CHILDREN DUE TO RISK OF REYE’S SYNDROME.  ONLY GIVE ASPIRIN IF DIAGNOSIS IS CLEAR
• IVIg

Systemic Sclerosis (Scleroderma) and CREST

Background

• Generalised disorder of connective tissue affecting the skin, internal organs and vasculature; characterised by sclerodactyly in combination with Raynaud’s and digital ischaemia
• Peak age is 40-60; prevalence 10-20/100,000; much more common in women (4:1)
• Can be classified as diffuse cutaneous (DCSS) (30%) or limited cutaneous (LCSS- restricted to below elbow/knee and face) (70%)
  • LCSS patients often have features grouped into the ‘CREST‘ syndrome
    • Calcinosis
    • Raynaud’s
    • oEsophageal involvement
    • Sclerodactyly
    • Telangiectasia
• Prognosis of DCSS is poor (70% at 5 years from diagnosis)
  • Risk factors include age, diffuse skin disease, proteinuria, high ESR/PV; low TLCO and pulmonary hypertension

Aetiology/Pathophysiology

• Cause is poorly understood- genetic component likely
• Clear evidence of immune dysfunction: T-lymphocytes infiltrate the skin causing abnormal fibroblast activation and increased production of extracellular matrix in the dermis (mainly type I collagen)
  • Results in thickening, tightening and induration of the skin; narrowing of the arterial/arteriolar walls
• There is also endothelial injury which causes vasoconstriction and platelet activation which causes yet further ischaemia and exacerbate fibrosis

Presentation

• Non pitting oedema of the fingers and flexor tendon sheaths, progressing to taut skin (shiny appearance with disappearance of the the skin creases)
  • Thinning of the lips and radial furrowing
• Erythema and tortuous dilation of nail-bed capillaries
• Reynaud’s phenomenon
• Arthralgia, morning stiffness and flexor tenosynovitis
  • Muscle weakness and wasting can occur due to myositis
• Reflux and erosive oesophagitis (lower third oesophageal involvement); dysphagia and odynophagia; upper GI bleeding (watermelon stomach, or antral vascular ectasia- 20%); (pseudo-)obstruction
• Hypertension

Investigations

• Clinical diagnosis but ESR/PV and IgG level are usually high
  • CRP can be normal
• ANA positive in 70%
  • DCSS are topoisomerase 1 antibody positive in 30%
  • LCSS are anticentromere antibody positive in 60%

Management

• Symptomatic treatment
  • Reynauds
    • Avoid cold and calcium channel blockers
    • Prostacyclin infusion may help digital ischaemia
  • Oesophageal problems
    • PPI and anti-reflux agents
    • If there is obsructive problems- metoclopramide/domperidone may be helpful
  • Hypertension
    • Treat aggressively with ACE inhibitor
  • Joints
    • Analgesia and/or NSAIDs (although consider renal function)
  • Pulmonary hypertension
    • Bosentan
    • Heart-lung transplant
    • Steroids if there is co-existing myositis/fibrosing alveolitis

Complications

• Frequently causes oesophageal dysmotility leading to recurrent aspiration pneumonia.
• Pulmonary hypertension (common complication of long-standing disease and presents with rapidly progressive dyspnoea, right heart failure and angina), pleuritis, pneumothorax and occasionally pulmonary haemorrhage and fibrosing alveolitis
• Rarely, patients develop malignant hypertension and renal crisis

Giant cell arteritis/Temporal Arteritis

Background/Epidemiology

• GCA is a chronic vaculitis of medium-large sized arteries and arch of the aorta.
  • Commonly associated with polymyalgia rheumatica
• One of the most commoon causes of acute blindness (permanent/irreversible)
  • visual loss occurs in up to 20% of patients with GCA (delayed diagnosis and treatment
  • MEDICAL EMERGENCY- requires urgent referral to ophthalmology or rheumatology

Presentation

• Acute onset, unilateral (often temporal) headache
  • Scalp pain (may cause problems combing hair
• Jaw and tongue claudication (difficulty chewing)
• Symptoms of polymyalgia rheumatica e.g. pain, stiffness, tenderness in procximal muscle groups (50%)
• Constitutional symptoms e.g. fever, weight loss, tiredness
• Visual symptoms
  • Classically ‘curtain’ like loss of visual field (amaurosis fugax), although blurring/double vision also possible
• Other symptoms of claudication (large vessel involvement) e.g. paraesthesia or pain of the hands/feet/calves on exertion; asymmetrical pulses; bruits

Investigations

• A raised ESR/PV is highly sensitive for GCA (95% will have an ESR >50mm/hour)
• Temporal artery biopsy should be performed in patients with clinically suspected GCA within one week
  • NB biopsy is NOT required if patients respond to empirical steroid treatment
• Imaging (only if there are symptoms/signs of large vessel involvement or severe systemic symptoms; or persistently high ESR/PV despite treatment)
  • CT (FDG-PET) or duplex USS (or MRI)

Diagnostic criteria

• Any 3 of
  • Age >50
  • New headache
  • Abnormal temporal artery (e.g. tender, rubbery)
  • Raised ESR (>50) or PV
  • Positive temporal artery biopsy

Management

• Steroids should be started as soon as the diagnosis is suspected
  • Start high-dose (60-80mg) for patients with claudication/visual symptoms/critical ischaemia
  • Medium dose steroids can be used for uncomplicated GCA (40mg)
  • Dose can be reduced after 4 weeks if both symptoms and serum inflammatory markers have normalised
    • Reduce by 10mg every 2 weeks to 20mg/day; then by 2.5mg every 2-4 weeks to 10mg/day; then 1mg every 1-2 months- provided there is no relapse
    • Assess patients at week 1, 3, 6 then every 3 months for first year (test FBC, U&Es, ESR/PRV, CRP, glucose)
    • NB 30-50% of patients can stop taking steroids after 2 years, but many require longer.  Patients should be educated about the long-term use of steroids and risk of adrenal suppression as much as other side-effects
      • If patients do relapse with complications, increase to start dose i.e. 60mg.  If without complications, revert to maintenance dose i.e. lowest dose where symptoms were controlled.
• Also start low dose aspirin, bone protection (bisphosphonates; calcium and vit D supplementation) and a PPI (unless there are contraindications
• Offer patients vaccinations for influenza and pneumococcal vaccination
• Also offer a CXR (or abdo CT) every 2 years for signs of AAA/aortic dissection

Amyloidosis

Background

• Amyloidoses are a group of acquired and hereditary disorders characterised by extracellular deposition of insoluble proteins, which can be localised or systemic
  • Clinical presentation depends on the organs involved

Types

• Reactive (AA) amyloidosis
  • Increased production of serum amyloid A as part of prolonged or recurrent acute inflammatory response
    • e.g. Chronic infection (TB, bronchiectasis, chronic abscess, osteomyelitis); Chronic inflammatory disease (chronic untreated/poorly controlled rheumatoid arthritis; familial Mediterranean fever)
  • Most patients present with non-selective proteinuria or nephrotic syndrome
  • May also have hepatosplenomegaly
  • On investigations there may be signs of hepatorenal problems e.g. raised urea and low serum albumin
• Light Chain amyloidosis (AL)
  • Increased production of monoclonal light chains
    • e.g. monoclonal gammopathies e.g. myeloma, benign gammopathies and plasmacytoma
  • Presents with features of restrictive cardiomyopathy, peripheral neuropathy/autonomic neuropathy, carpal tunnel syndrome, proteinuria, spontaneous purpura, amyloid nodules and plaques
    • Macroglossia occurs rarely but is pathognomonic
  • Prognosis is often poor
• Dialysis associated (Aβ2M) amyloidosis
  • Accumulation of circulating β2 microglobulin due to failure of renal catabolism in renal failure
    • associated with dialysis / renal failure
      • often 5-10 years from start of dialysis
  • Presents with carpal tunnel syndrome, chronic arthropathy and pathological fractures due to amyloid bone cysts formation
• Senile systemic amyloidosis
  • Normal deposition of transthyretin protein in tissues in older patients (>70)
    • Normal process of aging
    • Usually asymptomatic
• Hereditary systemic amyloidosis
  • Multiple forms – many mutations of the transthyretin gene; often autosomal dominant
  • Peripheral and autonomic neuropathy, cardiomyopathy (renal involvement is unusual- ~10%)
    • Many asymptomatic

Presentation

• Amyloidosis should be considered in all cases of unexplained nephrotic syndrome, cardiomyopathy and peripheral neuropathy

Investigation/Diagnosis

• Biopsy confirms diagnosis (affected organ, rectum or subcutaneous fat)
  • apple-green birefringent amyloid deposits (Congo red dye)
• Quantitative scintigraphy with radiolabelled SAP can estimate total body load

Management

• Largely supportive; prevent further deposition by treatment of any underlying cause, where possible
  • Liver transplantation may be an option

Henoch-Schonlein Purpura

Background/Epidemiology

• Most common vasculitis in children, although can be found in all ages (75% of cases <10) (incidence ~10-20/100,000)
  • Younger children are less likely to have complications e.g. nephritis of abdomincal complications
• Commonly follows an infective episode (usually Group A β-haemolytic streptococcus) or vaccination

Pathology

• IgA complexes are deposited in small vessels in the skin, gut, kidney (identical to IgA nephropathy- showing focal/segmental proliferative glomerulonephritis) and joints

Clinical Presentation

• Skin- palpable purpuric rash is always present (part of diagnostic criteria)
  • May precede or be preceded by other systemic symptoms
  • Usually symmetrical over the extensor surfaces, lower limbs and buttocks, arms, face and ears but usually spares the trunk
  • Can range from petechiae to large ecchymoses
• GI
  • Colicky abdominal pain occurs in 50-75% of cases
  • Vomiting and GI bleeding (either overt (rare- 2% of cases) or as faecal occult blood)
• Joints
  • Many patients will have joint involvement; which may precede the onset of a rash in up to a quarter of patients
  • Usually arthritis of the large joints of the lower limb (e.g. knees, ankle, hips) although can affect upper limbs
  • Pain, swelling, decreased ROM; rarely causes any permanent damage
• Renal
  • Occurs in between 20 and 60% of patients, can be 4-12 weeks after the initial rash and other symptoms
  • A range of renal symptomology can occur in HSP, including haematuria, proteinuria, nephrotic syndrome, nephritis, renal impairment and hypertension

Diagnosis

• Palpable purpura + one of
  • Diffuse abdominal pain
  • Arthritis or arthralgia
  • Renal involvement (any haematuria and/or proteinuria)
  • Any biopsy showing predominant IgA deposition

Investigations

• Majority of cases are clinical diagnoses
• FBC, U&Es
• Urinalysis
• Renal biopsy may be indicated in patients with renal failure and nephritic syndrome

Management

• Most patients will just require supportive treatment
• Steroids may be useful in patients with severe renal involvement and severe abdominal pain
  • Patients should be closely monitored for renal involvement and followed up for up to year for this

Ehlers-Danlos Syndrome

Background/Epidemiology

• ED are actually a group of rare inherited conditions characterised by abnormal proteins which are important for the structural integrity of skin, ligaments, cartilage and blood vessels.
• Occurs in ~1 in 5000 births

Different types of ED

Classic

• Caused by faulty collagen V (COL5A1 or COL5A2 genes) and is an autosomal dominant condition
• Classical features of EDS
  • Joint hypermobility and loose joints which can lead to dislocations and subluxations
  • Stretchy (hyperextensible), smooth and fragile skin which can split and bruise easily
  • Wounds can be slow to heal and leave distinctive widened scars
  • Other subsequent problems may include hernias, prolapse and cervical insufficiency
• Patients should have an echocardiogram to look for heart abnormalities
• Careful care of wounds (particularly e.g. in surgery) should be considered

Hypermobile type

• Most common and often not diagnosed.  It is uncertain as to whether this is a distinct condition from joint hypermobility syndrome, although the diagnosis of HEDS is usually made if there are other systemic features (see below).  It is more common than other types but is also more commonly undiagnosed
• The exact gene causing HEDS is unknown in most cases, although an autosomal dominant mode of inheritance is common
• Features include
  • Joint hypermobility and loose joints that can dislocate/sublux easily; also joint pain and fatigue
  • Easy bruising but no scarring
  • GI dysfunction
  • POTS (Postural orthostatic tachycardia syndrome); mild mitral valve prolapse/regurgitation
  • Uterine, rectal or bladder prolapse; urinary dysfunction
  • Varicose veins
  • Often patients are tall and may have blue sclerae
• Often just advice about contact sport etc and risk of dislocations etc is all that is required

Vascular type

• Caused by defect in collagen III (COL3A1- autosomal dominant) and is very rare
• Features include
  • Fragile blood vessels cause easy bruising, but also more serious complications e.g. rupture of more major blood vessels
  • There is also an increase risk of rupture of hollow organs e.g. GI tract; and gum problems (bleeding gums)
  • Thin skin often makes blood vessels more prominent.  This can appear as premature aging, particularly over the hands and feet.
  • There are also classical facial features (‘madonna face’- thin nose/lips, large eyes, small earlobes and fine hair)
  • Joint hypermobility, slow wound healing and joint contractures are also seen
  • Also consider this diagnosis where there is a history of bleeding but all blood tests are normal
• Patients should be followed-up for any signs/symptoms of major arterial bleed or bowel perforation.  Unfortunately, this can often be fatal by mid-adolescence to late adulthood.  (average lifespan- 48 years)
  • Uterine rupture is also common in pregnancy, and women need to be counselled prior to conception and during pregnancy as to the risks and management options

Other types include Kyphoscoliotic EDS; Tenascin-X deficient EDS; Arthrochalasia EDS; Dermatosparaxis EDS.

ANCA-associated vasculitis

Background

• Vasculitis, in general, can be classified by the size of vessel affected and by associated with anti-neutrophil cytoplasmic antibody (ANCA)
• ANCA-associated vasculitides share some common features (aside from presence of ANCA)
  • often involve small (occasionally medium) sized arteries
  • glomerulonephritis is a common feature
  • they usually respond well to immunosuppression
• Rare diseases (combined incidence ~10-20/1000000)
• The aetiology of these conditions is poorly understood (unlikely to be immune-complex mediated unlike pure small-artery vasculitis)

Classification 

• ANCA-associated vasculitis
  • Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e. capillaries, venules, arterioles and small arteries); associated with Myeloperoxidase (MPO-) ANCA or PR3-ANCA (although not all patients have ANCA i.e. ANCA-negative)
• Granulomatosis with polyangitis (GPA) (previously Wegener’s granulomatosis)
  • Necrotising granulomatous inflammation usually involving the upper and lower respiratory tract; with necrotising vasculiti affecting small-medium vessels
    • Necrotising glomerulonephritis is common
• Microscopic polyangitis (MPA)
  • Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels
  • Necrotising glomerulonephritis is very common
  • Granulomatous inflammation is absent
  • shares many features of GPA but lacks granulomatous features such as URT features characteristic of GPA
  • As GPA- note that MPA tends to be MPO-ANCA and GPA tends to be PR3-ANCA
• Eosinophilic granulomatosis with polyangitis (EGPA) (or Churg-Strauss syndrome)
  • Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract; and necrotising vasculitis mainly affecting small-medium vessels
  • Associated with late-onset asthma and eosinophilia
  • ANCA is more frequent when glomerulonephritis is present, although renal involvement, in general, is less common than in GPA and MPA

Clinical features

• Prodromal ‘flu-like’ illness is common; lasting weeks-months
• • Fever, polymyalgia, polyarthralgia, headache, malaise, anorexia, weight loss
• Upper-airways disease most common presenting feature
  • Nasal obstruction, nasal ulcers, septal perforation, sinusitis; serosanguinous discharge (crusting) or epistaxis
  • Destruction of the septum can result in ‘saddle-nose’ deformity
• Pulmonary involvement
  • Cough, haemoptysis, pleuritis
• Skin (50%)
  • ulcers, palpable purpura (commonly of the lower extremities), papules, nodules
• Ocular disease (50%)
  • associated with keratitis, conjunctivitis, scleritis, episcleritis, uveitis, retinal vessel occlusion and optic neuritis
  • visual loss occurs in <8% of patients
• Musculoskeletal
  • Arthralgia/myalgia are common, as is synovitis
• Nervous
  • Mononeuritis multiplex
• Renal
  • Although not a presentation- pauci-immune focal segmental necrotising glomerulonephritis

Investigations

• FBC, U&Es (particularly renal function), LFTs
  • ANCA screen
  • CRP/ESR/PV
• Urinalysis- haematuria, proteinuria
• CXR- pulmonary infiltrates and lymphadenopathy
• Tissue biopsy

Management

• Cyclosporin or rituximab and high dose steroids initially
  • Can step down to azathioprine and steroids

Polymyalgia Rheumatica

Background/Epidemiology

• Inflammatory condition of unknown cause characterised by aching morning stiffness of the neck, shoulder and pelvic girdle
• Fairly common (incidence ~85/100,000 / year); particularly in the elderly (average age of onset ~70 years and rarely diagnosed <50 years); more common in women (2-3:1)
• Tends to be associated with Giant Cell Arteritis in between 20-40% (debate as to whether part of the same condition)
  • This can be an emergency as it can be sight limiting

Presentation

• Suspect PMR in anybody >50, presenting with >2 weeks of
  • Bilateral shoulder, neck and/or pelvic girdle/hip pain; worse with movement; may trouble sleep; worse in the morning
    • May radiate to the elbows (75-90%); knees
  • Stiffness
    • should last at least 45 minutes after waking or periods of rest
    • may cause difficulty getting out of bed
• Additional symptoms include
  • Low-grade fever, fatigue, anorexia, weight loss, depression
    • Occur in up to half
  • Arm tenderness (bilateral)
  • Carpal tunnel syndrome
  • Peripheral arthritis (predominantly knees and wrists)
  • Peripheral oedema
• THERE IS NO WEAKNESS IN PMR (cf polymyositis)

Investigations

• FBC- normochromic, normocytic anaemia
• ESR/PV- raised
• CRP- may be raised
• It is prudent to check Renal function, LFTs, bone profile etc prior to starting steroids

Management

• NB Prior to starting steroid, make sure that the patient does not have any features of GCA (as this may require higher doses and more urgent treatment)
• Low dose steroids typically have a dramatic effect
  • 10-15mg daily initially will usually work overnight
    • Reduce the dose gradually
    • Patients may relapse but will again respond quickly to steroid treatment
    • Protect against bone loss as appropriate