Rheumatology - Misc rheumatological conditions

Question 1

What is carpel tunnel syndrome?

Answer 1

Entrapment of the median nerve as it passes under the flexor retinaculum causes carpel tunnel syndrome.

Although it may occur spontaneously, recognised associations include pregnancy, RA, previous wrist injury trauma, hypothyroidism, acromegaly and amyloidosis.

Bilateral disease is relatively common, especially if there is an underlying systemic disorder, although symptoms are usually worse on one side than the other.

Question 2

What is the presentation of carpel tunnel?

Answer 2

Sensory disturbance (pain and paraesthesia) affecting the radial three and a half digits, worse at night. Some patients report symptoms in the whole hand and extending up the forearm.

Impaired functioning with clumsiness.

Symptoms may be relieved by hanging the arm out of bed at night or shaking the hand.

Question 3

What are the important findings on physical examination in carpel tunnel syndrome?

Answer 3

Physical examination can be normal, especially if symptoms are intermittent. When present sensory disturbance is evident over the thumb, index and middle fingers, while the ring finger shows a loss of sensation over its radial half.

In long standing cases there may be wasting of and weakness of muscles of the thenar eminence. Two additional clinical tests can be performed that reproduce the patients symptoms:
1) Tinels test - percussion over the median nerve as it passes under the flexor retinaculum

2) Phalen’s test - wrist is maintained in a fixed flexion position

Question 4

How should carpel tunnel syndrome be investigated?

Answer 4

Nerve conduction studies - confirm compression of the median nerve at the wrist.

Perform investigation of underlying cause as indicated clinically.

Question 5

How should carpel tunnel syndrome be managed?

Answer 5

Treatment is indicated by the severity of the condition but may involve:

• splinting of the wrists at night
• injection of corticosteroids
• surgical decompression of the flexor retinaculum

Question 6

What is the prognosis of carpel tunnel syndrome?

Answer 6

Complete resolution of symptoms is usually achieved except in the most long standing cases when permanent nerve damage has occurred.

Question 7

What patients are at risk of septic arthritis?

Answer 7

Septic arthritis can occur in patients of any age or gender, but it is more common in the very young, the very old those with pre-existing abnormal/ damaged joints, immunocompromised individuals and IVDUs.

Question 8

How do bacteria infect the joint in septic arthritis?

Answer 8

1) Direct innoculation - e.g. penetrating injury, joint injection or surgery
2) Haematogenous spread during an episode of bacteraemia
3) Spread from neighbouring soft tissues (cellulitis) or bone (osteomyelitis) infection

Question 9

What agents are commonly implicated in septic arthritis?

Answer 9

Staph aureus
Beta haemolytic strep
Gram negative bacilli (e.g. E coli, Pseudomonas)
Neisseria gonorrhoea

Question 10

What is the clinical presentation of septic arthritis?

Answer 10

Sudden painful/ swollen joint in the context of pre-existing infection or in a patient with otherwise quiescent chronic joint disease should be assumed to be septic arthritis until proven otherwise.

Usually a single joint infection is most common (most commonly the knee) but several sites may be involved. Septic joints are very painful and are often held immobile to minimise discomfort. Systemic upset with pyrexia +/- rigors is common, but occasionally the patient may appear well.

Gonococcal infection may present with polyarthralgia and a migratory arthritis, associated with a purpuric rash - clinically apparent genital infection is not always apparent.

Question 11

How should septic arthritis be investigated?

Answer 11

Joint aspiration: joint fluid is turbid and microscopy excludes crystal arthropathy, a Gram stain may confirm the presence of bacteria, and the fluid can be sent for MC&S

FBC: leucocytosis is common and the ESR and CRP are raised

Blood cultures: may confirm a bacteraemia and identify the organism

Radiology: narrowing of the joint space signifies destruction of the cartilage

Question 12

How is septic arthritis managed?

Answer 12

Antibiotics should be started following joint aspiration and the ideal agent should cover the most common organisms, i.e. staphylococcus aureus and beta haemolytic strep. Flucloxacillin i.v. (erythromycin or clindamycin if penicillin allergic) and oral fusidic acid 500mg 8 hourly is the preferred combination. Gentamicin is added to immunosuppressed patients to cover gram negatives. Treatment should be modified depending on culture and sensitivity and continue with two antibiotics for 6 weeks (initial 2 weeks IV) and a single antibiotic for a further 6 weeks.

Immobilise joint in early stages, mobilise early to avoid contractures.

NSAIDs for pain relief.

Question 13

What is a frozen shoulder? How does it present?

Answer 13

This is a relatively common and potentially disabling condition affecting 1-2% of the middle aged and elderly population. For reasons that are unclear, the joint capsule becomes adherent to the overlying rotator cuff muscles.

It is typically unilateral and characterised by progressive pain and reduced mobility. Untreated, improvement with time is generally the rule but may take up to 2 years. Intra-articular injection of corticosteroid may help ease the pain and should be combined with regular exercise to restore movement.

Question 14

What is rotator cuff tendonitis?

Answer 14

Repetitive or unaccustomed movements of the shoulders may result in inflammation of one or more of the rotator cuff muscles tendons. Pain during abduction, flexion or rotation of the shoulder is often accompanied by point tenderness. Plain radiographs may show tendon calcification, but ultrasound or MRI will demonstrate oedema with tears within the tendon. Treatment options include NSAIDs, physiotherapy, local injection or corticosteroids and surgery.

Question 15

What is rotator cuff degeneration?

Answer 15

This is commonly seen in elderly patients who have restricted shoulder movements in all directions which limit activities of daily living. Local injection of corticosteroids may tried, but the underlying pathological process tends to be progressive.

Question 16

What back problems are common in younger patients (i.e. 15-30)?

Answer 16

The age of the patient helps in deciding the aetiology of back pain because certain causes are more common in particular age groups.

Mechanical, prolapsed intervertebral disc, ankylosing spondylitis, and spondylolithesis are all common in this age group.

Question 17

How does mechanical back pain present?

Answer 17

Mechanical causes of back pain include lumbar disc prolapse, osteoarthritis, fractures, spondylolithesis and spinal stenosis.

Pain is often of sudden onset and worse in the evening. Morning stiffness is absent and exercise aggravates the pain.

Question 18

What back problems are common in middle aged patients (i.e. 30-50)?

Answer 18

Mechanical
Prolapsed intervertebral disc
Degenerative joint disease
Malignancy
Fractures (all ages) 
Infective lesions (all ages)

Question 19

How does inflammatory causes of back pain present?

Answer 19

The main inflammatory cause of back pain is Ankylosing spondylitis and infection.

Pain is usually of gradual onset and is worse in the morning. Morning stiffness is also present. Exercise relieves the pain.

Question 20

What are the red flag symptoms of back pain?

Answer 20

Age <20 or >50 years
Constant pain without relief
History of TB, HIV, carcinoma, steroid use
Systemically unwell; fever; weight loss
Localised bone tenderness
Bilateral signs in the legs
Neurological deficit involving more than one root level
Bladder, bowel or sexual function deficits

Question 21

What causes of back pain are common in the elderly?

Answer 21

Degenerative joint disease
Osteoporosis
Pagets disease
Malignancy
Myeloma

Question 22

How should back pain be investigated?

Answer 22

The key points are age, speed of onset, the presence of motor and sensory symptoms, involvement of the bladder or bowel and the presence of stiffness and the effect of exercise. Young adults with a history suggestive of mechanical back pain and with no physical signs do not need further investigation.

FBC, ESR and serum biochemistry (calciu, phosphate, ALP) are required only when the pain is likely to be due to malignancy, infection or metabolic bone disease. PSA should be checked if secondary prostatic disease is suspected.

Spinal X rays are only indicated if there are “red flag” symptoms and signs which indicate the high risk of more serious underlying problems.

Bone scans show increased uptake with infection or malignancy.

MRI is useful when neurological symptoms are present. It is useful for detection of discs and cord lesions. CT scans demonstrate bone pathology better.

Question 23

What is acute disc disease?

Answer 23

Prolapse of the intervertebral disc results in acute back pain (lumbago), with or without radiation of the pain to areas supplied by the sciatic nerve. It is a disease of younger people (20-40 years) because the disc degenerates with age and in elderly people is no longer capable of prolapse. In older patients sciatica is more likely to be the result of compression of the nerve root by oesteophytes in the lateral recess of the spinal canal.

Question 24

What are the clinical features of acute disc prolapse?

Answer 24

There is sudden onset of severe back pain, often following a strenuous activity. The pain is often clearly related to position and is aggravated by movement. Muscle spasm leads to sideways tilt when standing. The radiation of the pain and the clinical findings depend on the disc affected.

Question 25

What are the features of an S1 root lesion?

Answer 25

Pain is from the buttock down the back of the thigh and leg to the ankle and foot.

Sensory loss is on the sole of the foot and posterior calf. Plantar flexion of the ankle and toes is lost and the ankle jerk reflex (S1 S2) is affected. There may also be diminished straight leg raise.

Question 26

What are the features of an L5 root lesion?

Answer 26

Pain is from the buttock to the lateral aspect of the leg and dorsum of the foot.

Sensory loss is along the dorsum of the foot and anterolateral aspect of the lower leg.

Dorsiflexion of the foot and toes are affected but there is no reflex lost at this level. There may be diminished straight leg raise as with an S1 lesion.

Question 27

What are the features of an L4 root lesion?

Answer 27

Pain on the lateral aspect of the thigh to the medial side of the calf.

Sensory loss is over the medial aspect of calf and shin. Motor weakness is in dorsiflexion and inversion of ankle; extension of the knee.

Knee jerk reflex is lost.

Question 28

How should acute disc disease be investigated?

Answer 28

Investigations are of very limited value in acute disc disease and X rays are often normal. MRI is usually reserved for patients in whom surgery is being considered.

Question 29

How should acute disc disease be managed?

Answer 29

Treatment is aimed at the relief of symptoms and has little effect on the duration of the disease. In the acute stage, treatment consists of bed rest on a firm mattress, analgesia and occasionally epidural corticosteroid injection in severe disease. Surgery is only considered for severe or increasing neurological impairment - e.g. foot drop or bladder symptoms. Physiotherapy is used in the recovery phase, helping to correct posture and restore movement.

Question 30

What causes chronic disc disease?

Answer 30

Chronic lower back pain is associated with “degenerative” changes in the lower lumbar discs and facet joints. Pain is usually of the mechanical type. Sciatic radiation may occur with pain in the buttocks radiating into the posterior thigh. Usually the pain is long standing and the prospects of cure are limited. Weight loss, NSAIDs and physiotherapy are all useful measures.

Surgery can be considered when pain arises from an identifiable level and has failed to respond to conservative measures, and fusion at this level, with decompression of the nerve roots, can be successful.

Question 31

What is spondylolisthesis?

Answer 31

Spondylolisthesis is characterised by a slipping forward of one vertebra on another, most commonly at L4/5. It arises because of a defect in the pars interarticularis of the vertebra, and may be either congenital or acquired (e.g. trauma). The condition is associated with mechanical back pain that worsens throughout the day. The pain may radiate to one or other leg and there may be signs of nerve root irritation. Small spondylolisthesis can be treated with simple analgesics, large spondylolisthesis causing severe symptoms should be treated with spinal fusion.

Question 32

What is spinal stenosis?

Answer 32

Narrowing of the lower spinal canal compresses the cauda equina, resulting in back and buttock pain, typically coming on after a period of walking and easing with rest. Accordingly it is sometimes called spinal claudication. Causes include disc prolapse, degenerative osteophyte formation, tumour and congenital narrowing of the spinal canal. CT and MRI will demonstrate cord compression and treatment is by surgical decompression.

Question 33

What is a vertebral crush fracture?

Answer 33

This is most commonly seen in middle aged to elderly females with osteoporosis of the thoracic spine. Sudden vertebral collapse results in abrupt onset of severe pain. Long term involvement at several levels may result in kyphosis.

Question 34

How should crush fractures be investigated?

Answer 34

Plain radiographs confirm the extent of vertebral involvement. Further investigation is geared towards excluding other underlying conditions (e.g. malignant infiltration), screening for osteoporosis and identifying potentially reversible causes of the latter. Achieving adequate pain control can be challenging and may require opioid analgesics. Osteoporosis is treated along conventional lines. If there are any neurological symptoms then MRI scanning should be undertaken urgently.

Question 35

What is juvenile - onset systemic Still’s disease?

Answer 35

This is also called juvenile idiopathic arthritis. It is a childhood disease which usually presents with:

• constitutional symptoms
• fever
• skin rashes
• joint pain in 75% of cases at onset which may be monoarticular
• persistent joint swelling (knee, ankle, wrist and small joints of the hands and feet)
• eye changes: cataracts
• lymphadenopathy, splenomegaly and pericarditis
• 1/3 of patients usually present with an insidious history of polyarthritis similar to adult RA, but rheumatoid factor is negative
• growth retardation may result from the primary condition or treatment (e.g. systemic corticosteroids)

Question 36

What is the treatment of choice in Juvenile onset Still’s disease?

Answer 36

Physiotherapy, NSAIDs, and intra-articular corticosteroid injections are used in treatment. Other immunosuppressive agents (e.g. methotrexate) and the TNF alpha inhibitor etanercept may also be required in refractory cases with more extensive joint involvement.

Question 37

What are the clinical features of adult onset Still’s disease?

Answer 37

This is an acute systemic inflammatory disorder of unknown origin. The onset is typically between 16 and 35 years of age, with both sexes affected equally.

In the absence of specific disease markers, the diagnosis is based on clinical features: the presence of 5 or more criteria, including at least 2 major criteria has a diagnostic sensitivity of >95% and specificity of >90%:

1) Major: spiking fever (>39 for >1 week) arthralgia, typical rash, leucocytosis (with >80% neutrophilia)
2) Minor: sore throat, lymphadenopathy and/or splenomegaly, liver dysfunction, negative ANA and RhF screens
3) Exclude: infections, malignancies and other rheumatological conditions

Marked elevation in serum ferritin is a key finding and correlates with disease activity. The ESR and CRP are also normally elevated. Aspirin and other NSAIDs may help as first line therapy, but long term management with corticosteroids is required in at least half of all patients. DMARDs and TNFalpha inhibitors may be required.

Question 38

What is acute rheumatic fever?

Answer 38

This is an acute febrile systemic disorder affecting mainly the heart and joints following a streptococcal infection (group A beta haemolytic) and usually occurs between the ages of 5 and 15 years. The diagnosis is made when there is evidence of previous streptococcal infection plus one major and two minor or two major (Jones) criteria.

Question 39

What is the clinical presentation of acute rheumatic fever?

Answer 39

Symptoms:

• migratory polyarthropathy : common in adults
• carditis: common in children
• arthralgia: exquisitely tender joints and history of streptococcal throat infection in last 10-20 days
• chorea: Sydenhams chorea, usually in children

Signs:

• fever
• flitting arthropathy of large joints (small joints ma be affected in the elderly)
• erythema nodosum and erythema marginatum
• symmetrical subcutaneous nodules: over bony prominences and extensor surfaces in children
• myocarditis: tachycardia, cardiomegaly, heart failure
• endocarditis: any valve may be involved and cause transient murmurs. A transient mitral diastolic murmur (Carey Coombs) is the most common. Mitral systolic and aortic murmurs also occur
• pericarditis: friction rub or small effusion

Question 40

How is acute rheumatic fever investigated?

Answer 40

Raised or rising antistreptolysin or DNAase titre: evidence of preceding group A streptococcal infection.

Throat swab: haemolytic strep may be identified.

Leucocytosis and hypochromic normocytic anaemia, ESR and CRP elevated.

ECG: may show first degree heart block or other rhythm disorders.

CXR: progressive cardiac enlargement.

Question 41

How should rheumatic fever be managed?

Answer 41

Anti-inflammatory therapy: aspirin or other NSAIDs can be tried.

Corticosteroids may be required, especially if there is evidence of cardiac involvement.

Antistreptococcal therapy: i.v. benzylpenicillin during the acute phase and oral phenoxymethylpenicillin continued in those with cardiac involvement for at least 5 years and preferably until 20 years of age to prevent recurrence. Erythromycin can be used for penicillin allergic patients.

Neurolpetics may help with chorea.

Question 42

What agents are commonly associated with post infectious arthralgia?

Answer 42

Low-grade, poly arthralgia frequently follows some infections, e.g. glandular fever, Rubella, Mycoplasma, and viral hepatitis and may persist for months or years. The association of erythema chronicum migrans is seen in Lyme disease following tick borne infection with the spirochete Borrelia burgdorferi.

Question 43

What is fibromyalgia?

Answer 43

The lack of evidence of underlying musculoskeletal pathology means fibromyalgia is often considered in the same category as so called other “functional syndromes”. It often co-exists with depression and other psychiatric disorders.

Some have proposed that it be considered first and foremost as a disorder of bodily perception, in which pain is perceived centrally even in the absence of a peripheral cause – added to which, affected individuals often exhibit features of somatisation, i.e. the expression of psychological distress in physical terms. Factors which are recognised to exacerbate fibromyalgia include sleep deprivation and depression.

Question 44

What is the clinical presentation of fibromyalgia?

Answer 44

Classically, patients present with widespread pain and disability, together with marked generalised tiredness/fatigue. Review of the past medical history may reveal other unexplained physical symptoms (e.g. headache, dizziness, breathlessness, chest pain, gynaecological symptoms).

Question 45

How should fibromyalgia be investigated?

Answer 45

The lack of a specific diagnostic test means that fibromyalgia is often a diagnosis of exclusion, based on clinical features. However, it is reasonable to ar- range a simple panel of tests to exclude other causes including: full blood count, ESR, CRP, renal and liver biochemistry, CK, thyroid function tests, antinuclear antibodies, myeloma screen (in those >50 years of age) and a plain chest X-ray (especially in smokers). It is desirable to avoid over-investigation, which can reinforce the patient’s belief that there is an under- lying physical cause.

Question 46

What red flag symptoms should you be aware of in patients with fibromyalgia?

Answer 46

Beware the so-called ‘red flag’ markers for patients presenting with widespread pain, i.e. age >50 years, recent onset, associated with weight loss or fever, past history of malignancy or immunosuppressive therapy, areas of focal pain, abnormal physical signs other than tenderness, abnormalities in screening investigations – if any of these are present, then a diagnosis of fibromyalgia should not be made and other causes pursued.

Question 47

Outline an approach to the treatment of fibromyalgia

Answer 47

Once the clinician is satisfied that there is no underlying physical disorder, it is important to openly discuss the diagnosis with the patient – while some argue that the label ‘fibromyalgia’ is unhelpful and may lead to stigmatisation, others find it helpful in that it allows a ‘diagnosis’ to be made and a manage- ment strategy planned – understanding the individual patient’s needs is a key part of effective management. Options include:

. Cognitive behavioural therapy ` antidepressants.
. Low-dose tricyclic antidepressants (e.g. amitriptyline) to improve sleep quality and help with pain
management.
. Programmes to improve physical fitness.
. Avoidance of factors that precipitate exacerbations. . Involvement of the liaison psychiatry and pain man-
agement teams.
. In addition, it is important to consider the issues of
the ‘sick role’ and ‘secondary gain’ as tackling these may help to improve outlook