11. Joint pain/swelling Flashcards

Question

key features of JIA

Answer 1

joint pain, swelling and stiffness

Answer 2

Systemic JIA Polyarticular JIA Oligoarticular JIA Enthesitis related arthritis Juvenile psoriatic arthritis

Answer 3

Subtle salmon-pink rash High swinging fevers Enlarged lymph nodes Weight loss Joint inflammation and pain Splenomegaly Muscle pain Pleuritis and pericarditis

Answer 4

stills disease

Answer 5

Macrophage activation syndrome (MAS), where there is severe activation of the immune system with a massive inflammatory response. It presents with an acutely unwell child with disseminated intravascular coagulation (DIC), anaemia, thrombocytopenia, bleeding and a non-blanching rash. It is life threatening. A key investigation finding is a low ESR.

Answer 6

Kawasaki disease, Still’s disease, rheumatic fever and leukaemia.

Answer 7

involves idiopathic inflammatory arthritis in 5 joints or more. The inflammatory arthritis tends to be symmetrical and can affect the small joints of the hands and feet, as well as the large joints such as the hips and knees. There are minimal systemic symptoms, but there can be mild fever, anaemia and reduced growth. Systemic symptoms are mild, unlike systemic onset JIA. Polyarticular JIA is the equivalent of rheumatoid arthritis in adults.

Answer 8

It involves 4 joints or less. Usually it only affects a single joint, which is described as a monoarthritis. It tends to affect the larger joints, often the knee or ankle. It occurs more frequently in girls under the age of 6 years.

Answer 9

oligoartiucalr

Answer 10

Enthesitis-related arthritis is more common in male children over 6 years. It can be thought of as the paediatric version of the seronegative spondyloarthropathy group of conditions that affect adults. These conditions are ankylosing spondylitis, psoriatic arthritis, reactive arthritis and inflammatory bowel disease-related arthritis. Patients have inflammatory arthritis in the joints as well as enthesitis. An enthesis (plural: entheses) is the point at which the tendon of a muscle inserts into a bone. Enthesitis is inflammation of this insertion point. Enthesitis can be caused by traumatic stress, such as through repetitive strain during sporting activities, or can be caused by an autoimmune inflammatory process. An MRI scan of the affected joint can demonstrate enthesitis, but cannot distinguish between an enthesitis due to stress or an autoimmune process.

Answer 11

Psoriatic arthritis is an seronegative inflammatory arthritis associated with psoriasis, the skin condition. The pattern of joint involvement varies. Patients can have a symmetrical polyarthritis affecting the small joints similar to rheumatoid, or an asymmetrical arthritis affecting the large joints in the lower limb. Juvenile psoriatic arthritis is associated with several signs on examination: Plaques of psoriasis on the skin Pitting of the nails (nail pitting) Onycholysis, separation of the nail from the nail bed Dactylitis, inflammation of the full finger Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone

Answer 12

1. referral to rheumatology for coordination of care NSAIDS Steroids - either oral, intramuscular or intra-artricular in oligoarthritis Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab

Answer 13

systemic JIA (stills)

Answer 14

enthesitis related JIA

Answer 15

Oligoarticular/ Pauciarticular 'little girl (<6) called ANA'

Answer 16

polyarticualr - older children

Answer 17

PC: young man with lower back pain and stiffness of insidious onset, stiffness worse in the morning and improves with exercise. may experience pain at night which improves on getting up

Answer 18

- reduced lateral flexion - reduced forward flexion - Schober's test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible - reduced chest expansion

Answer 19

1. plain xray of sacroiliac joints 2. if negative and still suspected, MRI

Answer 20

SSS 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

Answer 21

at risk of apical fibrosis - request CXR if suspected Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints. reduced chest expansion o/e

Answer 22

1. NSAIDs 2. Paracetamol/codeine if poorly tolerated 3. Seek specialist advice + Physio + Hydrotherapy + OT

Answer 23

Refer to rheumatology for a spondyloarthritis assessment if a person has low back pain starting before the age of 45 years and lasting longer than 3 months, plus four or more of the following criteria: Low back pain starting before the age of 35 years. Symptoms which wake them during the second half of the night. Buttock pain. Improvement when moving. Improvement within 48 hours of taking a nonsteroidal anti-inflammatory drug (NSAID). Spondyloarthritis in a first-degree relative. Current or past arthritis. Current or past enthesitis. Current or past psoriasis.

Answer 24

apical fibrosis anterior uveitis CVS risk and heart involvement: aortic regurg, AV node block osteoporosis and fractures cauda equina

Answer 25

hip, knee and ankle

Answer 26

PC: hot red swollen painful joint, stiffness and reduced range of motion, systemic symptoms such as fever and lethargy any hot/red joint

Answer 27

staph aureus Neisseria gonorrhoea (gonococcus) in sexually active teenagers Group A streptococcus (Streptococcus pyogenes) Haemophilus influenza Escherichia coli (E. coli)

Answer 28

always think of gonococcus septic arthritis until proven otherwise Gonorrhoea infection is common and delaying treatment puts the joint in danger. In your exams it might say the gram stain revealed a “gram-negative diplococcus”. The patient may have urinary or genital symptoms to trick you into thinking of reactive arthritis but remember that it is important to exclude gonococcal septic arthritis first as this is the more serious condition.

Answer 29

Have a low suspicion until joint fluid assessed 1. Aspiration for gram stain, crystal microscopy, culture and antibiotic sensitivities 2. If paeds, USS, if shows effusion and with corroborating history, treat as septic arthritis 3. Bloods inflammatory markers, blood culture 4. Empirical IV antibiotics (local guidelines) 5. Continue abx for 3-6 weeks Patients may require surgical drainage and washout of the joint to clear the infection in severe cases.

Answer 30

Flucloxacillin for 6 weeks if pen allergic: clindamycin If MRSA suspected: vancomycin If gonococcal arthritis or gram -ve susepcted : cefotaxime or ceftriaxone

Answer 31

The kocher criteria fever >38.5 degrees C non-weight bearing raised ESR > 40 raised WCC >12 If 0 = very unlikely and can be managed in primary care with close follow up

Answer 32

an infection in the bone and bone marrow. This typically occurs in the metaphysis of the long bones.

Answer 33

staph aureus

Answer 34

Chronic osteomyelitis is a deep seated, slow growing infection with slowly developing symptoms. Acute osteomyelitis presents more quickly with an acutely unwell pt. The infection may be introduced directly into the bone, for example during an open fracture. Alternatively it may have travelled to the bone through the blood, after entering the body through another route, such as the skin or gums.

Answer 35

boys and children under 10 years

Answer 36

Osteomyelitis can present acutely with an unwell child, or more chronically with subtle features. Signs and symptoms are: - Refusing to use the limb or weight bear - Pain - Swelling - Tenderness They may be afebrile, or may have a low grade fever. Children with acute osteomyelitis may have a high fever, particularly if it has spread to the joint causing septic arthritis.

Answer 37

1. xray 2. MRI if xray inconclusive 3. inflammatory markers CRP, ESR, WCC 4. blood culture

Answer 38

Osteomyelitis is treated with antibiotics, usually with surgical debridement Flucloxacillin for 6 weeks + consider fusidic acid or rifmapicin if pen allergic: clindamycin If MRSA suspected: vancomycin

Answer 39

Discitis is an infection in the intervertebral disc space. It can lead to serious complications such as sepsis or an epidural abscess.

Answer 40

Back pain General features pyrexia, rigors sepsis Neurological features e.g. changing lower limb neurology if an epidural abscess develops

Answer 41

staph aureus

Answer 42

Invetsigation Imaging: MRI has the highest sensitivity blood culture CT-guided biopsy may be required to guide antimicrobial treatment transoesophageal/transthoracic echo to assess for endocarditis (seeding) management abx based on cultures/biopsy

Answer 43

An arthritis that develops following an infection where the organism cannot be recovered from the joint.

Answer 44

'Can't see, pee or climb a tree' conjunctivitis , urethritis and arthritis

Answer 45

Chlamydia trachomatis

Answer 46

Shigella flexneri Salmonella typhimurium Salmonella enteritidis Yersinia enterocolitica Campylobacter

Answer 47

symptomatic: analgesia, NSAIDS, intra-articular steroids sulfasalazine and methotrexate are sometimes used for persistent disease symptoms rarely last more than 12 months

Answer 48

SLE is characterised by anti-nuclear antibodies (ANA). These are autoantibodies against proteins within the cell nucleus. These antibodies generate a chronic inflammatory response, leading to the condition’s features.

Answer 49

Fatigue Weight loss Arthralgia (joint pain) Non-erosive arthritis Myalgia (muscle pain) Fever Photosensitive malar rash Lymphadenopathy Splenomegaly Shortness of breath Pleuritic chest pain Mouth ulcers Hair loss Raynaud’s phenomenon Oedema (due to nephritis)

Answer 50

triggered or worsened by sunlight.

Answer 51

Autoantibodies ANA - 85% will have positive - other things can make it positive Anti-dsDNA - 50% will have positive - specific Full blood count may show anaemia of chronic disease, low white cell count and low platelets CRP and ESR may be raised with active inflammation C3 and C4 levels may be decreased in active disease Urinalysis and urine protein:creatinine ratio shows proteinuria in lupus nephritis Renal biopsy may be used to investigate for lupus nephritis

Answer 52

general: - Infection (from disease and immunosupp drugs) - Anaemia of chronic disease cardio: - CVS disease - pericarditis resp: - pleuritis renal: - lupus nephritis neuro: - neuropsychiatiric (optic neuritis, transverse myelitis, psychosis) complication: - recurrent miscarriage - VTE - due to antiphospholipid secondary to SLE

Answer 53

First-line options include: Hydroxychloroquine NSAIDs Steroids (e.g., prednisolone) Treatment options for resistant or more severe SLE include: DMARDs (e.g., methotrexate, mycophenolate mofetil or cyclophosphamide) Biologic therapies Biological therapies include: Rituximab (a monoclonal antibody that targets the CD20 protein on the surface of B cells) Belimumab (a monoclonal antibody that targets B-cell activating factor)

Answer 54

seronegative spondyloarthropathy group

Answer 55

There are 5 recognised patterns. Asymmetrical oligoarthritis and symmetrical polyarthritis are the most common. Asymmetrical oligoarthritis affects 1-4 joints at any given time, often on only one side of the body. Symmetrical polyarthritis presents similarly to rheumatoid arthritis. More than four joints are affected, such as the hands, wrists and ankles.

Answer 56

Psoriatic arthritis tends to affect the distal interphalangeal (DIP) joints and axial skeleton, whereas rheumatoid arthritis tends not to affect these joints. This can help you distinguish them.

Answer 57

Plaques of psoriasis on the skin Nail pitting (tiny indents in the fingernails and toenails) Onycholysis (separation of the nail from the nail bed) Dactylitis (inflammation of the entire finger) Enthesitis (inflammation of the entheses, which are the points of insertion of tendons into bone)

Answer 58

Arthritis mutilans is the most severe form of psoriatic arthritis. It affects the phalanges (the bones of the fingers and toes). There is osteolysis (destruction) of the bones around the joints, leading to progressive shortening of the digits. The skin folds as the digit shortens, giving an appearance described as a telescoping digit.

Answer 59

DOPA Dactylitis (inflammation of the whole digit, seen as soft tissue swelling) Osteolysis (destruction of bone) Periostitis (inflammation of the periosteum, causing a thickened and irregular outline of the bone) Ankylosis (fixation or fusion of the bones at the joint) The classic x-ray finding in the digits is a “pencil-in-cup” appearance. There is erosion of the bones at the joint. There is central erosion on one side of the joint, giving a cup-like appearance. The other bone becomes pointed and looks like a pencil in the cup. This appearance is associated with arthritis mutilans.

Answer 60

There is a crossover between the systemic treatments used to treat the skin condition and psoriatic arthritis. Treatment is coordinated between dermatologists, rheumatologists and other multidisciplinary team members. Depending on the severity, treatment may involve: Non-steroidal anti-inflammatory drugs (NSAIDs) Steroids DMARDs (e.g., methotrexate, leflunomide or sulfasalazine) Anti-TNF medications (etanercept, infliximab or adalimumab) Ustekinumab is a monoclonal antibody that targets interleukin 12 and 23

Answer 61

Rickets is a condition affecting children where there is defective bone mineralisation causing “soft” and deformed bones. In adults the same process leads to a condition called osteomalacia. Osteo– means bone and –malacia means soft. Causes Rickets is caused by a deficiency in vitamin D or calcium.

Answer 62

Darker skin Breastfed babies as formula feed is fortified with vitamin D Inadequate sun exposure Chronic kidney disease Liver disease Malabsorption

Answer 63

Lethargy Bone pain/aching Swollen wrists Bone deformity Poor growth Dental problems Muscle weakness Pathological or abnormal fractures

Answer 64

in toddlers genu varum (bow legs) in older children - genu valgum (knock knees) Rachitic rosary, where the ends of the ribs expand at the costochondral junctions, causing lumps along the chest Craniotabes, which is a soft skull, with delayed closure of the sutures and frontal bossing Delayed teeth with under-development of the enamel

Answer 65

1. Serum 25-hydroxyvitamin D is the laboratory investigation for vitamin D. A result of less than 25 nmol/L establishes a diagnosis vitamin D deficiency, which can lead to rickets. + Xray is required to diagnose rickets. X-rays may also show osteopenia (more radiolucent bones). Other investigation results include: Serum calcium may be low Serum phosphate may be low Serum alkaline phosphatase may be high Parathyroid hormone may be high NICE clinical knowledge summaries suggest additional investigations to look for other pathology: Full blood count and ferritin, for iron deficiency anaemia Inflammatory markers such as ESR and CRP, for inflammatory conditions Kidney function tests, for kidney disease Liver function tests, for liver pathology Thyroid function tests, for hypothyroidism Malabsorption screen such as anti-TTG antibodies, for coeliac disease Autoimmune and rheumatoid tests, for inflammatory autoimmune conditions

Answer 66

Referal to paeds Children with vitamin D deficiency can be treated with vitamin D (ergocalciferol). The doses for treatment of vitamin D deficiency depend on the age (see the BNF). The dose for children between 6 months and 12 years is 6,000 IU per day for 8 – 12 weeks.

Answer 67

Patients with vitamin D deficiency and osteomalacia may not have any symptoms. Typical symptoms include: Fatigue Bone pain Muscle weakness Muscle aches Pathological or abnormal fractures Looser zones are fragility fractures that go partially through the bone.

Answer 68

Investigations Serum 25-hydroxyvitamin D is the laboratory investigation for vitamin D: Less than 25 nmol/L – vitamin D deficiency 25 to 50 nmol/L – vitamin D insufficiency Other laboratory investigation results include: Low serum calcium Low serum phosphate High serum alkaline phosphatase High parathyroid hormone (secondary hyperparathyroidism) Imaging investigations include: X-rays may show osteopenia (more radiolucent bones) DEXA scan shows low bone mineral density

Answer 69

Treatment is with colecalciferol (vitamin D₃). There are various loading regimes (e.g., for patients with symptoms) suggested by the NICE clinical knowledge summaries (2022), for example: 50,000 IU once weekly for 6 weeks 4000 IU daily for 10 weeks A maintenance dose of 800-2000 IU per day is continued following the loading regime (or as the initial treatment in patients that do not require rapid treatment. NICE CKS (2022) recommend checking the serum calcium within a month of the loading regime. It may be: Low in calcium deficiency High in primary hyperparathyroidism (previously masked by the vitamin D deficiency) High in other conditions that cause hypercalcaemia, such as cancer, sarcoidosis or tuberculosis

Answer 70

Gout is a type of crystal arthropathy associated with chronically high uric acid levels. Urate crystals are deposited in the joint causing it to become hot, swollen and painful.

Answer 71

PC: single acute got, swollen and painful joint Typical joints: Base of the big toe (metatarsophalangeal joint) Wrists Base of thumb (carpometacarpal joints) Can also affect larger joints such as knee and ankle

Answer 72

Gouty tophi are subcutaneous deposits of uric acid typically affecting the small joints and connective tissues of the hands, elbows and ears. The DIP joints are most affected in the hands.

Answer 73

Typically the space between the joint is maintained Lytic lesions in the bone Punched out erosions Erosions can have sclerotic borders with overhanging edges

Answer 74

Plan 1. Rule out septic arthritis Investigation Can be diagnosed clinically or with joint aspiration 1. Joint aspiration = no bacterial growth, needle shaped crystals, negatively birefringent of polarised light, monosodium urate crystals Management During acute flare: 1. NSAIDs eg ibuprofen 2. Colchicine 3. Steroids Prophylaxis (initiate after attack has settled) 1. Allopurinol (xanthine oxidase inhibitor (reduces uric acid levels) + Lifestyle changes: losing weight, staying hydrated, minimising consumption of alcohol and purine-based foods (such as meat and seafood), and reducing high fructose corn syrup Do not initiate allopurinol prophylaxis until after the acute attack is settled. Once treatment of allopurinol has been started then it can be continued during an acute attack.

Answer 75

no bacterial growth, needle shaped crystals, negatively birefringent of polarised light, monosodium urate crystals

Answer 76

Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium. For this reason, it is now more correctly termed acute calcium pyrophosphate crystal deposition disease. This usually asymptomatic condition of the elderly is due to deposition of calcium pyrophosphate dihydrate (CPPD) crystals in large joints, most commonly the knee. The crystals are initially deposited in the cartilage - chondrocalcinosis - where they are associated with degenerative changes. The shedding of the crystals into the joint space results in an acute synovitis and a clinical picture that is similar to that seen in gout.

Answer 77

Patients who develop pseudogout at a younger age (e.g. < 60 years) usually have some underlying risk factor, such as: haemochromatosis hyperparathyroidism low magnesium, low phosphate acromegaly, Wilson's disease

Answer 78

strongly associated with increasing age

Answer 79

joint aspiration: weakly-positively birefringent rhomboid-shaped crystals

Answer 80

aspiration of joint fluid, to exclude septic arthritis NSAIDs or intra-articular, intra-muscular or oral steroids as for gout

Answer 81

age - gout over 40, pseudogout over 60 size of joint - gout smaller joints, pseudo larger joints

Answer 82

Paget’s disease of bone involves excessive bone turnover (reabsorption and formation) due to increased osteoclast and osteoblast activity. The excessive turnover is not coordinated, leading to patchy areas of high density (sclerosis) and low density (lysis). The result is enlarged and misshapen bones, structural problems and an increased risk of pathological fractures. It particularly affects the axial skeleton (the bones of the head and spine). The cause is unknown. It typically affects older adults.

Answer 83

Patients may be asymptomatic (diagnosed incidentally on an x-ray), or present with: Bone pain Bone deformity Fractures Hearing loss

Answer 84

Bone enlargement and deformity Osteoporosis circumscripta (well-defined osteolytic lesions that appear less dense compared with normal bone) Cotton wool appearance of the skull (poorly defined patchy areas of increased and decreased density) V-shaped osteolytic defects in the long bones

Answer 85

X-ray Raised alkaline phosphatase Normal calcium Normal phosphate

Answer 86

Bisphosphonates are the main treatment. They are generally very effective. They interfere with osteoclast activity and restore normal bone metabolism. They improve symptoms and prevent further abnormal bone changes. Other measures include: Calcitonin is a treatment option where bisphosphonates are unsuitable Analgesia (e.g., NSAIDs) for bone pain Calcium and vitamin D supplementation, if necessary Surgery is rarely required to treat fractures, severe deformities and arthritis Monitoring involves checking the serum alkaline phosphatase (ALP) and reviewing symptoms. Effective treatment should normalise the ALP and eliminate symptoms.

Answer 87

Hearing loss (if it affects the bones of the ear) Heart failure (due to hypervascularity of the abnormal bone) Osteosarcoma Spinal stenosis and spinal cord compression

Answer 88

In osteoporosis, bone mass decreases, but the ratio of bone mineral to bone matrix is normal. In osteomalacia, the ratio of bone mineral to bone matrix is low.

Answer 89

Osteoporosis involves a significant reduction in bone density. Osteopenia refers to a less severe decrease in bone density. Reduced bone density makes the bones weaker and prone to fractures.

Answer 90

Normal more than -1 osteopenia -1 to -2.5 osteoporosis less than -2.5 severe osteoporosis less than - 2.5 + a fracture

Answer 91

Bone mineral density (BMD) is measured using a DEXA scan (dual-energy x-ray absorptiometry). DEXA scans are a type of x-ray that measures how much radiation is absorbed by the bones, indicating how dense the bone is. The bone mineral density can be measured anywhere on the skeleton, but the femoral neck reading is most important. Bone density can be represented as a Z-score or T-score. The Z-score is the number of standard deviations the patient is from the average for their age, sex and ethnicity. The T-score is the number of standard deviations the patient is from an average healthy young adult. The T-score is used to make the diagnosis.

Answer 92

The T-score is the number of standard deviations the patient is from an average healthy young adult. A T-score of -1 means the bone mineral density is 1 standard deviation below the average for healthy young adults.

Answer 93

Older age Post-menopausal women Reduced mobility and activity Low BMI (under 19 kg/m2) Low calcium or vitamin D intake Alcohol and smoking Personal or family history of fractures Chronic diseases (e.g., chronic kidney disease, hyperthyroidism and rheumatoid arthritis) Long-term corticosteroids (e.g., 7.5mg or more of prednisolone daily for longer than 3 months) Certain medications (e.g., SSRIs, PPIs, anti-epileptics and anti-oestrogens)

Answer 94

Tamoxifen is a selective oestrogen receptor modulator (SERM) used to treat breast cancer. It blocks oestrogen receptors in breast tissue but stimulates oestrogen receptors in the uterus and bones. It helps prevent osteoporosis but increases the risk of endometrial cancer.

Answer 95

Anyone on long-term oral corticosteroids or with a previous fragility fracture Anyone 50 and over with risk factors All women 65 and over All men 75 and over The 10-year risk of a major osteoporotic fracture and a hip fracture can be calculated using either: QFracture tool (preferred by NICE) FRAX tool (NICE say this may underestimate the risk in some patients)

Answer 96

Patients are categorised as low, intermediate or high risk based on the risk calculator. For QFracture, this is based on the percentage, and patients above 10% are considered for a DEXA scan. For FRAX, this is based on the NOGG guideline chart (linked to on the online FRAX tool), which advises whether to arrange a DEXA scan or start treatment. These suggestions do not apply to specific groups. For example, NICE CKS (April 2023) suggest: A DEXA may be arranged without calculating the risk in patients over 50 with a fragility fracture Treatment may be started without a DEXA in patients with a vertebral fracture

Answer 97

1. adress reversible risk factors: increase exercise, weight management, stop smoking, reduce alcohol 2. Address calcium and vitamin D inadequatcies Calcium (at least 1000mg) Vitamin D (400-800 IU) First line treatment 1. bisphosphonates

Answer 98

Reflux and oesophageal erosions Atypical fractures (e.g., atypical femoral fractures) Osteonecrosis of the jaw (regular dental checkups are recommended before and during treatment) Osteonecrosis of the external auditory canal Oral bisphosphonates are taken on an empty stomach with a full glass of water. Afterwards, the patient should sit upright for 30 minutes before moving or eating to reduce the risk of reflux and oesophageal erosions.

Answer 99

Alendronate 70 mg once weekly (oral) Risedronate 35 mg once weekly (oral) Zoledronic acid 5 mg once yearly (intravenous)

Answer 100

The NICE CKS (2023) recommend reassessing treatment with bisphosphonates after 3-5 years. They suggest a repeat DEXA scan and stopping treatment if the T-score is more than -2.5. Treatment is continued in high-risk patients.

Answer 101

Denosumab (a monoclonal antibody that targets osteoclasts)

Answer 102

Sarcomas are a diverse group of malignant tumours originating from mesenchymal tissue. Sarcomas can be classified based on their tissue of origin: Bone Sarcomas Osteosarcoma Chondrosarcoma Ewing's sarcoma Soft Tissue Sarcomas Liposarcoma Rhabdomyosarcoma (striated muscle origin) Synovial sarcoma Fibrosarcoma Angiosarcoma Leiomyosarcoma (smooth muscle origin))

Answer 103

Pain: Often a presenting symptom in bone sarcomas and occasionally in soft tissue sarcomas. Swelling or a palpable mass: More common in soft tissue sarcomas. Impaired function: Depending on the location, sarcomas may cause limitations in motion, difficulty breathing, or other functional impairments. Pathologic fractures: Bone sarcomas can weaken the bone, leading to fractures. Systemic symptoms: Fatigue, weight loss, and fever may be present, particularly in advanced cases.

Answer 104

Large >5cm soft tissue mass Deep tissue location or intramuscular location Rapid growth Painful lump

Answer 105

2ww adult where x-ray suggests bone sarcoma 48hr children and young ppl unexplained bone swelling or pain OR where x-ray suggests bone sarcoma

Answer 106

Consider an urgent direct access ultrasound scan (to be performed within 2 weeks) to assess for soft tissue sarcoma in adults with an unexplained lump that is increasing in size. Consider a suspected cancer pathway referral (for an appointment within 2 weeks) for adults if they have ultrasound scan findings that are suggestive of soft tissue sarcoma or if ultrasound findings are uncertain and clinical concern persists. Consider a very urgent direct access ultrasound scan (to be performed within 48 hours) to assess for soft tissue sarcoma in children and young people an unexplained lump that is increasing in size. Consider a very urgent referral (for an appointment within 48 hours) for children and young people if they have ultrasound scan findings that are suggestive of soft tissue sarcoma or if ultrasound findings are uncertain and clinical concern persists.

Answer 107

Surgery: The primary treatment for most sarcomas, aiming for complete resection with negative margins to reduce the risk of local recurrence. In some cases, limb-sparing surgery can be performed to preserve function, while amputation may be necessary for more advanced or aggressive tumours. +/- radiation, chemo, targeted therapies

Answer 108

Myeloma is a cancer of the plasma cells (type of B lymphocytes that produce antibodies). Cancer in a specific type of plasma cell results in large quantities of a single antibody being produced.

Answer 109

is where there is an excess of a single type of antibody or antibody components without other features of myeloma or cancer.

Answer 110

B cells in bone marrow producing antibodies → genetic mutation causing one antibody to be produced uncontrollably (50% of the time this is IgG) Bone marrow infiltration → anaemia, neutropaenia, thrombocytopaenia Plasma cells releasing cytokines → increased osteoclast activity → myeloma bone disease → skull, spine, long bones affected → bone pain / pathological fractures → releasing lots of calcium from bones → hypercalcaemia Kidney failure is common in patients with a monoclonal gammopathy, most frequently due to hypercalcemia or myeloma cast nephropathy. Immunoglobulin crystallization is an uncommon phenomenon that also results in kidney injury hypercalcaemia can ppt kidney stones,

Answer 111

PC: anaemia PC: neutropenia PC: thrombocytopenia PC: pathological fractures PC: bone pain, particularly back pain PC: myeloma renal disease PC: hyperviscosity PC: kidney stones

Answer 112

FBC: anaemia Calcium and bone profile: hypercalcaemia U&Es : renal failure ESR Plasma viscosity If suggetsive --> then very urgent (within 48hrs) - protein electrophoresis - bence-jones urine test if +ve 2ww bone marrow biopsy and look at all results

Answer 113

The diagnostic criteria for multiple myeloma requires one major and one minor criteria or three minor criteria in an individual who has signs or symptoms of multiple myeloma. Major criteria Plasmacytoma (as demonstrated on evaluation of biopsy specimen) 30% plasma cells in a bone marrow sample Elevated levels of M protein in the blood or urine Minor criteria 10% to 30% plasma cells in a bone marrow sample. Minor elevations in the level of M protein in the blood or urine. Osteolytic lesions (as demonstrated on imaging studies). Low levels of antibodies (not produced by the cancer cells) in the blood.

Answer 114

involves the removal of a patient's own stem cells prior to chemotherapy, which are then replaced after chemotherapy

Answer 115

Allogenic hematopoietic cell transplantation - from someone else is not commonly used in myeloma due to high rates of overall mortality and symptoms of graft-ve

Answer 116

1. induction 2. autologous stem cell transplant if eligible 3. maintenance A combination of drugs is used to treat myeloma - 'induction therapy targeted drugs (such as thalidomide, lenalidomide, bortezomib, daratumumab) chemotherapy (such as cyclophosphamide or melphalan) steroids (such as prednisolone or dexamethasone) The particular combination depends on whether a patient may be suitable for autologous hematopoietic cell transplantation or not. Autologous hematopoietic cell transplantation involves the removal of a patient's own stem cells prior to chemotherapy, which are then replaced after chemotherapy prolong both event-free and overall survival when compared with non-transplant strategies typically it is younger, healthier patients who are suitable for stem cell transplantation and rigorous chemotherapy regimes.

Answer 117

pain: treat with analgesia (using the WHO analgesic ladder) pathological fracture: zoledronic acid is given to prevent and manage osteoporosis and fragility fractures as these are a large cause of morbidity and mortality, particularly in the elderly. infection patients receive annual influenza vaccinations they may also receive Immunoglobulin replacement therapy. venous thromboembolism prophylaxis fatigue treat all possible underlying causes if symptoms persist consider an erythropoietin analogue.

Answer 118

Ganglion cysts are sacs of synovial fluid that originate from the tendon sheaths or joints. They commonly occur in the wrist and fingers but can occur anywhere there is a joint or tendon sheath.

Answer 119

Ganglion cysts can appear rapidly (over days) or gradually. Patients present with a visible and palpable lump. It is not usually painful. Rarely, they may compress nerves, leading to sensory or motor symptoms. On examination, ganglion cysts: Range in size from 0.5 to 5cm or more (most are 2cm or less) Firm and non-tender on palpation Well-circumscribed Transilluminates (shining a torch into the cyst causes the whole lump to light up)

Answer 120

Diagnosis Ganglion cysts are mostly diagnosed clinically, based on the history and examination findings. X-rays will show normal bones and joints (unless there are co-existing conditions). Ultrasound can help confirm the diagnosis and exclude other causes of lumps.

Answer 121

Ganglion cysts may be managed conservatively, without any intervention. 40-50% of cysts will resolve spontaneously, but this can take several years. Active management options for ganglion cysts are: Needle aspiration (draining the cyst by aspirating the fluid with a needle) Surgical excision (open or endoscopic removing the cyst, usually under local anaesthetic) Needle aspiration has a high rate of recurrence (50% or more). Surgical excision involves removing the entire cyst and the affected part of the joint capsule or tendon sheath. Therefore, the recurrence rate is low. However, there is a risk of complications, such as infection and scarring.

Answer 122

Genetics and smoking HLA DR4 (a gene often present in RF positive patients) HLA DR1 (a gene occasionally present in RA patients)

Answer 123

pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy

Answer 124

keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy

Answer 125

ischaemic heart disease RA carries a similar risk to type 2 diabetes mellitus

Answer 126

Felty's syndrome (RA + splenomegaly + low white cell count) amyloidosis

Answer 127

RA + splenomegaly + low white cell count his is important to be aware of, as patients present with recurrent and severe infections.

Answer 128

spondyloarthritis: e.g. Psoriatic and reactive arthritis sickle-cell disease other rare causes include tuberculosis, sarcoidosis and syphilis

Answer 129

Schober's test <5cm is suggestive of ankylosing spondylitis. This is an indication of reduced lumbar flexion.

Answer 130

ANA - SENSITIVE - 85% will have positive - other things can make it positive Anti-dsDNA - SPECIFIC 50% will have positive - specific

Answer 131

Impaired activation of vitamin D