Flashcards in Rheumatology Deck (37):
A 68-year-old woman complained of pain at the base of her right thumb.
There was tenderness and swelling of the right first carpo-metacarpal joint.
What is the most likely diagnosis?
Osteoarthritis of the first carpometacarpal joint is extremely common and in a 68-year-old lady is the most likely diagnosis.
Swelling is usually bony hard and due to osteophyte formation which can lead to the appearance of squaring of the hand.
de Quervain's tenosynovitis is a common overuse condition which presents with pain at the base of the thumb but is not associated with joint swelling.
This joint can be affected in rheumatoid and psoriatic arthritis but rarely on its own.
A 14-year-old gymnast presents with lower back ache of one year duration. On examination, she has fifth lumbar nerve root pain and hamstring spasm.
Spondylolisthesis is the slipping forward of one vertebra in relation to another.
Isthmic spondylolisthesis is one type (of five) and appears to be a form of repetitive stress fracture; the incidence is much higher in teenage gymnasts and other sports persons. It commonly occurs between the ages of 7 and 10.
The signs and symptoms include
Low back ache
Fifth lumbar nerve root pain
Disturbance in the sagittal profile of the spine with an acute kyphosis
Neurological symptoms in the legs.
On examination, there may be a step in the line of the spinous processes; straight leg raising test may be reduced due to hamstring spasm.
A 32-year-old man presents with an acute low back ache which started on lifting a heavy object at work.
Lumbar disc prolapse is a condition occurring in fit young adults. It occurs acutely when lifting heavy weight or whilst straining. The pain is felt in the lower back, sometimes radiating to the buttock or down the leg.
A 78-year-old lady presents with pain along her lower spinal region. Her urine biochemistry shows detectable amounts of gamma globulins.
Multiple myeloma is a medullary tumour of typical and atypical plasma cells. It commonly affects the pelvic girdle, vertebrae, skull and ribs.
Abnormal plasma proteins are detected in 80% of cases, with Bence-Jones proteinuria being found in half of those affected.
A 27-year-old man presents with morning stiffness and backache. He is positive for HLA-B27 antigen.
Ankylosing spondylosis predominantly affects young men, with a male to female ratio of 6:1. The patients present with
Progressive loss of spinal movements leading to kyphosis
Hyperextension of the neck (question mark posture).
x Rays shows 'bamboo spine' with squaring of the vertebrae. Bloods reveal raised erythrocyte sedimentation rate (ESR), normochromic anaemia and positive HLA-B27 antigen. Early treatment with anti-inflammatories and physiotherapy can reduce disability.
A 22-year-old lady presents with stiffness, swelling, and pain in the joints of her hands. On examination, she has swelling of the metacarpophalangeal joints and of the proximal interphalangeal joints, and has nodules over her left elbow.
Rheumatoid arthritis is a systemic disorder of unknown origin characterised by synovial thickening, joint swelling and inflammation. It is three times more prevalent in women and commonly affects those between 25-45 years of age. Initially the patients present with weakness, fatigue, and anorexia. As the disease progresses, the joint involved becomes stiff, swollen, and hot. This is followed by restricted movements and deformity. Twenty five per cent present with rheumatoid nodules which are common over bony prominences such as the elbow.
A 66-year-old gentleman presents with painful knees and hips, and the pain is relieved by rest. On examination of the knee joint, there is crepitus, mild effusion, and restricted movements. He also has nodules over the distal interphalangeal joints of his fingers.
Osteoarthritis is a degenerative, non-inflammatory arthritis, characterised by gradual, but progressive deterioration of the articular cartilage. Pain is the presenting complaint which is usually relieved by rest. However, joint stiffness occurs with rest which paradoxically improves with activity. The physical signs include crepitus, joint swelling, effusion and restricted movements. Osteophytic overgrowths in the hands are termed as Heberden's nodes when present over the distal interphalangeal joint and Bouchard's nodes when present over the proximal interphalangeal joint.
A 42-year-old male with a 25 year history of type 1 diabetes has a nephropathy and sensory neuropathy of the legs. He is aware of deformity and pain of the left foot with a rocker-bottom appearance.
Charcot's arthropathy is associated with conditions such as diabetes, syringomyelia and syphilis where typically there may be painless destruction of the joint (in this case ankle) due to a combination of avascular necrosis and neuropathy. However, the joint may also be painful.
A 73-year-old male presents with a one day history of pain and tenderness of the left ankle joint. On examination, his left ankle joint is hot, red and exquisitely tender.
Classically gout is associated with erythema, inflammation and marked pain of the big toe but it can affect any joint and is usually mono/oligoarticular. The disorder is due to urate crystal deposition and the differential here would rest between septic arthritis. Aspiration of the joint may be expected to reveal negatively birefringent urate crystals. Serum urate concentrations may be unhelpful as these may fall in an acute attack. Rheumatoid arthritis would not be expected to present like this as the condition is usually a symmetrical deforming arthropathy of smaller joints.
A 72-year-old female presents with pain in her feet particularly her left foot. On examination there is tenderness over both first metatarsal phalangeal joints and there is lateral deviation of both big toes.
Hallux valgus deformity sometimes with a bursa (bunion) may become inflamed and painful but is usually nowhere near as painful as gout. Similarly the bilateral nature and the lateral deviation of the big toes are more supportive of the diagnosis being OA.
A baby girl was born by caesarean section for breech presentation. Her mother notices that applying her nappy was difficult. She is late to walk and is noted to have a waddling gait.
Dysplasia of the hip
Developmental dysplasia of the hip (DDH) covers the whole spectrum from mild acetabular dysplasia to complete dislocation of the hip
A 9-year-old boy presents with a three day history of fever and restricted movement in his right hip. His ESR is 110 mm/h, and he has a white cell count of 30 ×109/L. An x ray appears normal.
A septic or pyogenic arthritis is a serious condition. The organism responsible is often Staphylococcus. The child is typically febrile with a hot tender swollen joint. x Ray may be unhelpful although ultrasound may reveal an effusion.
Joint aspiration needs to be carried out to determine the organism. Treatment needs to be started as soon as possible as delay may result in destruction of the joint.
A 6-year-old boy presents with a gradual onset of pain in his right hip and groin. He is apyrexial. His white cell count is 7 ×109/L. His ESR is 5 mm/h and he is generally well. An x ray reveals an abnormality in the femoral head.
Usually Perthes' disease (coxa plana) affects a boy who is small and active, aged 4-9. There is an incidence of 1:9000. Although the condition is bilateral in 10% it is not simultaneous.
rheumatoid arthritis pharmacology complications
Weight gain, diabetes and hypertension.
Methylprednisolone is a corticosteroid and has a cushingoid effect on the body when taken for long periods of time. Weight gain and hypertension are extremely common in patients who take corticosteroids.
rheumatoid arthritis pharmacology complications
Cirrhosis and pulmonary fibrosis.
Methotrexate is a folic acid antagonist and hence a drug which has a low pharmacological index. It can be very toxic and has to be monitored regularly. Side effects include hepatic cirrhosis, folate deficiency and pulmonary fibrosis.
rheumatoid arthritis pharmacology complications
Hydroxychloroquine, also an antimalarial, is proven to work well in the treatment of RA but is associated with retinitis and cataracts.
rheumatoid arthritis pharmacology complications
Sulfasalazine has a vast range of side effects and like methotrexate, must be monitored regularly.
rheumatoid arthritis pharmacology complications
Increased risk of lymphoma with prolonged use.
Azathioprine may be associated with haematological abnormalities and there is a small risk of lymphoma associated with long term use.
The mechanism of action of bisphosphonates involves the inhibition of farnesyl diphosphate synthase within osteoclasts. In doing this they interfere with geranylgeranylation (attachment of the lipid to regulatory proteins), which causes osteoclast inactivation. This leads to reduced bone turnover, increased bone mass and improved mineralisation.
Bisphosphonates licensed for the prevention and treatment of osteoporosis include alendronate, risedronate and ibandronate.
The bisphosphonates zoledronate and pamidronate are used for the treatment of metastatic bone disease and short term management of hypercalcaemia.
A 40-year old lady presents to clinic complaining of an 18 month history of dorsoradial wrist pain. She is a keen tennis player.
On examination she has tenderness localised to the dorsoradial aspect of the wrist and passive motion of the thumb causes crepitus in the same region. Finkelstein's test is positive.
Which of the following is the likely diagnosis?
De Quervain's tenosynovitis is thought to be related to overuse, and is common in golfers and racquet sport players.
Most affected are females 30-50-years-old.
Finkelstein's test (flexion of the thumb into the palm, making a fist over the thumb and ulnar deviation of the wrist causes pain in the first dorsal extensor compartment) is diagnostic.
Ganglia are multilocular cystic swellings. They are common over the dorsum of the hand and the wrist. These swellings are fluctuant and they transilluminate as they contain synovial fluid. The ganglion may disappear with pressure or a blow. Recurrence is usual.
Histology shows a compressed collagen sheath filled with a mucoid substance.
Dupuytren's contracture is more common in Caucasians; it has a possible genetic link, the majority of the patients having a positive family history.
It is associated with epilepsy (and barbiturate medication), diabetes mellitus, alcoholism and trauma. The ring finger is the most commonly affected finger, followed by the little finger; however, any digit may be affected. The disease (contracture) is caused due to thickening and contracture of the palmar fascia: nodules and bands (cords) develop with progressive contracture to metacarpophalangeal or proximal interphalangeal joints.
The other clinical features include: palmar nodule, skin pits, knuckle pads, and eventually contracting cords.
A 47-year-old lady presents with an inability actively to extend the distal interphalangeal joint of her left ring finger. A click is felt on passive extension.
Stenosing tenosynovitis (trigger finger) is caused by size disparity between the tendon and its sheath that results from inflammation and swelling or intratendinous nodules. The disorder may involve any digit but occurs most often in the thumb or the ring finger.
The patient is unable actively to extend the finger from the fully flexed position. There is often an audible 'click' as the finger is passively extended (the tendon is released from the tendon sheath to which it may be adhered).
Chronic leg ulceration is associated with
Sickle cell anaemia
At cold temperatures cryoglobulins precipitate causing complement activation. This causes a vasculitis. The inflammation results in vascular purpura and livedo (marbling appearance). This is described as livedoid vasculitis. The classical triad is a palpable peripheral vasculitis, arthralgia and muscle weakness.
Ulceration is rare in hereditary spherocytosis but should be considered in young patients with no identified risk factors. Erythematous lesions may develop after episodes of haemolysis.
In rheumatoid arthritis the incidence of leg ulceration is about 8%. It is the most common cause of leg ulceration after chronic venous disease and peripheral arterial disease.
Leg ulceration develops in 25-75% of patients with sickle cell anaemia. There may be ischaemia due to thrombosis of small vessels. Ulceration may start with minor trauma. The ulcers are very difficult and slow to heal. Up to 97% will recur in a year.
On plain x ray may show the capital femoral epiphysis to be smaller, denser and flatter
Perthes' disease is due to avascular necrosis of the proximal femoral epiphysis. It presents with hip pain and a limp, usually before the age of 10 years.
The male to female ratio is 4:1.
x Ray shows the capital femoral epiphysis to be small, dense and flattened. A bone scan shows a cold spot within the epiphysis.
Amyloidosis is a pathological process characterised by the accumulation of extracellular fibrils.
The fibrillar insoluble protein is always associated with a non-fibrillar component, amyloid-P component. Amyloid-P component is derived from the acute phase protein serum amyloid-P.
In haematoxylin and eosin (H&E) stained paraffin-embedded tissue sections, deposits of amyloid appear as homogeneous, eosinophilic material in the interstitium and walls of blood vessels, and bind the Congo red dye, and its characteristic apple green birefringence under polarised microscopy.
Amyloid causes renal failure due to its deposition within the kidney.
Known precipitants of gout include
Low dose aspirin
The latter should therefore not be given during acute attacks.
Corticosteroids are like high dose aspirin or non-steroidal anti-inflammatory drugs (NSAIDs) a treatment for acute gout.
Can lead to destruction of synovial gliding surfaces
Infections of the finger flexor tendons are serious with the potential for substantial permanent functional impairment. The majority of these infections result from penetrating wounds and Stapylococcus aureus infection.
The four cardinal signs of tendon sheath infection are
Partially flexed posture of the finger
Fusiform swelling of the finger
Tenderness along the entire flexor tendon sheath
Severe pain from passive extension of the finger.
The tenosynovitis can rapidly destroy the synovial gliding surfaces and result in healing with restrictive adhesions. A fulminating infection can cause tendon necrosis.
Early tenosynovitis can be treated with intravenous antibiotics, elevation and splinting. However, if no definite signs of improvement are seen after 24 hours, surgical exploration of drainage must be considered since the consequences of a fulminating flexor tenosynovitis are so severe and permanent.
The other systemic causes of avasular necrosis are
Steroid therapy (for example, following renal transplantation)
Cushing's disease and
The local causes which predispose to this condition include
Rheumatoid arthritis (affecting that particular bone)
Trauma (as in fracture neck of femur; fractures of the scaphiod)
Severe osteoarthritis and
Is associated with plantar fasciitis
The prevalence of ankylosing spondylitis is approximately 1 in 2000.
They are more common in men and the M:F ratio is approximately 6:1 at 16-years-old and 2:1 at 30-years-old.
There is often a family history: HLA B27 positive in more than 95% of cases and HLA CW6 positive in the remaining.
Chest pain and carditis are recognised complications of this condition.
The other features associated with ankylosing spondylitis are
Decrease in thoracic excursion
Periositis of calcaneum and ischial tuberosities
It is associated with aortic regurgitation rather than myocardial complications.
Can cause Volkmann's ischaemic contracture
Compartment syndrome is a devastating early complication usually seen after fractures, which must be recognised and treated early.
A rise in compartmental tissue pressure may be triggered by a number of factors, including soft tissue injury.
If a sustained rise in tissue pressure above the arterial perfusion pressure occurs, muscles and nerves will be deprived of essential blood supply and ischaemic muscle further releases factors (such as free radicals) which increase capillary permeability, worsening the situation.
The main early feature is excessive pain which is made worse by passive stretch of the suspected group of muscles.
Compartment syndrome can occur in the presence of normal peripheral pulses, since it is the microvasculature which is initially affected.
Sensory loss may be detected early but motor weakness is a late sign.
Fasciotomy should be undertaken if the difference between the diastolic and the measured compartment pressure is less than 30 mmHg.
If not recognised and treated early, it could lead to Volkmann's ischaemic contracture.
Angiotensin causes vasoconstriction and is incriminated in the chronic vasoconstriction and endothelial dysfunction associated with hypertension.
Serotonin (also known as 5-hydroxytryptamine) is a vasoactive amine, as is histamine, which may be released from mast cells, basophils and platelets, and result in vasodilatation and increased vascular permeability.
In which of the following is the risk of fractures due to osteoporosis not increased?
Osteoporosis is caused due to a reduction in bone mass (osteopenia) for the patient's age and sex, and there is usually a negative calcium balance.
In later life osteoporosis can cause fractures, even after trivial trauma. The bone is fully mineralised but it is porous and weak.
The condition affects at least a quarter of all women over the age of 60 years and is usually associated with postmenopausal changes.Caucasian women with a premature menopause (and amenorrhoea) and a familial tendency are more likely to develop osteoporosis.
The other risk factors include
Endocrine diseases such as thyrotoxicosis
Hyperparathyroidism and hypopituitarism
Primary biliary cirrhosis
Prolonged bed rest
Past low-trauma fractures and
Gout, caused by hyperuricaemia as the result of an abnormality in purine metabolism, usually affects the small joints.
The underlying cause may be an over-production or under-excretion of uric acid. Monosodium urate monohydrate crystals are deposited within the synovium which becomes inflamed.
Crystals may be deposited within joints, tendons or bursae. Deposits over the achilles tendon, elbow, pinnae and fingers are known as tophi and may ulcerate or destroy bone and cartilage.
The serum uric acid is elevated above 420 µmol/l and typical birefringent crystals are present in the synovial fluid or on biopsy of a tophus (demonstration of rhomboid crystals with weak birefringence is characteristic of pseudogout).
Gout is treated by rest and large doses of nonsteroidal anti-inflammatory drugs (NSAIDs) (such as indomethacin). Colchicine is now rarely used because of its side effects.
Surgical washout of the joint is not usually required.
Modification of the patient's lifestyle in combination with a xanthine oxidase inhibitor (allopurinol) or a uricosuric agent such as probenecid, provided the renal function is normal, should prevent recurrences.
Preventing infection in joint replacement
Joint replacement surgery should not be considered in patients with open wounds.
Cessation of smoking at least two weeks before surgery reduces prosthesis and wound infection rates.
Hair should be removed from the operative field with clippers immediately before surgery, shaving the day before surgery causes an increased release of skin commensals.
Laminar flow air hoods reduce the number of bacteria in the air above the wound.
The antibiotic impregnated cement reduces the rate of deep infection.
Affects adults with pre-existing joint disease
It most commonly affects the hip in infants and the knee in older children.
Ten per cent of patients present with multiple joints involved.
Septic arthritis is more common in patients with rheumatoid and osteoarthritis.
Organisms in the joint fluid cause an influx of neutrophils causing the release of proteolytic enzymes which attack the cartilage.