MUSC Flashcards

Question

Epidemiology of Carpal Tunnel Syndrome

Answer 1

* Prevalence: 2.7% | * Lifetime risk of 10%

Answer 2

- tingling and pain in the hand and fingers | - weakness and clumsiness of the hand

Answer 3

* Sensory impairment in the median nerve distribution * Weakness and wasting of thenar eminence * Tinel's Sign - tapping the carpal tunnel causes symptoms * Phalen's Test - flexion of the wrist for 1 min may cause symptoms * Look out for signs of the underlying cause (e.g. acromegaly, hypothyroidism)

Answer 4

• Bloods TFTs ESR • Nerve Conduction Study (not usually necessary) Shows impaired median nerve conduction across the carpal tunnel

Answer 5

Progressive, degenerative process affecting the cervical vertebral bodies and intervertebral discs, and causing compression of the spinal cord and/or nerve roots

Answer 6

• Osteoarthritic degeneration of the vertebral bodies leads to the formation of osteophytes • These osteophytes protrude on to the foramina and spinal canal • This leads to compression of: - Nerve roots = radiculopathy - Anterior spinal cord = myelopathy

Answer 7

* Mean age at diagnosis = 48 yrs | * More common in MALES

Answer 8

* Neck pain/stiffness * Arm pain (stabbing or dull ache) * Paraesthesia * Weakness * Clumsiness in the hands * Weak and stiff legs * Gait disturbance * Atypical chest pain * Breast pain * Pain in the face

Answer 9

ARMS: - Atrophy of the forearm and hand muscles - Segmental muscle weakness in a nerve root distribution (e.g. C5 --> shoulder abduction and elbow flexion weakness) - Hyporeflexia - Sensory loss (mainly pain and temperature) - Pseudoathetosis (writhing finger movements when hands are outstretched, fingers spread and eyes closed) LEGS: - (if cervical cord compression) -Increased tone -Weakness -Hyper-reflexia -Extensor plantar response -Reduced vibration and joint position sense • Lhermitte's Sign - neck flexion causes crepitus (grating sound) and/or paraesthesia down the spine

Answer 10

Spinal X-ray (lateral)

Answer 11

Chronic pain disorder with an unknown cause

Answer 12

* UKNOWN aetiology | * Thought to be something to do with altered pain perception

Answer 13

* COMMON * 10 x more common in WOMEN * Usual age of presentation: 20-50 yrs

Answer 14

* Pain at multiple sites (MAIN SYMPTOM) * Fatigue * Sleep disturbance * Morning stiffness * Paraesthesia * Feeling of swollen joints * Problems with cognition * Headaches * Light headedness * Anxiety * Depression * Urinary problems (dysmenorrhea in women)

Answer 15

• CLINICAL diagnosis • Key features of fibromyalgia: -Widespread pain involving both sides of the body, above and below the waist for at least 3 months - Presence of 11 tender points among the 9 pairs of specific sites shown in the diagram below

Answer 16

Granulomatous inflammation of large arteries, particularly branches of the external carotid artery, most commonly the TEMPORAL ARTERY

Answer 17

* UNKNOWN * More common with increasing age * Some associations with ethnic background and infections * Associated with HLA-DR4 and HLA-DRB1

Answer 18

* More common in FEMALES | * Peak age of onset: 65-70 yrs

Answer 19

• Subacute onset (usually over a few weeks) • Headache • Scalp tenderness • Jaw claudication • Blurred vision • Sudden blindness in one eye • Systemic: malaise, low-grade fever, lethargy, weight loss, depression • Symptoms of polymyalgia rheumatica - early morning pain and stiffness of muscles of the shoulder and pelvic girdle NOTE: 40-60% of GCA has polymyalgia rheumatica

Answer 20

* Swelling and erythema overlying the temporal artery * Scalp and temporal tenderness * Thickened non-pulsatile temporal artery * Reduced visual acuity

Answer 21

• Bloods: - High ESR - FBC - normocytic anaemia of chronic disease • Temporal Artery Biopsy: - Must be performed within 48 hrs of starting corticosteroids - Negative biopsy doesn't necessarily rule out GCA

Answer 22

• High dose oral prednisolone IMMEDIATELY to prevent visual loss • Reduce the dose of prednisolone gradually • Many patients will need to be kept on a maintenance dose of prednisolone for 1-2 yrs • Low dose aspirin (with PPIs and gastroprotection) - reduces risk of visual loss, TIAs and stroke • Annual CXR for up to 10 yrs to look for thoracic aortic aneurysms

Answer 23

* Carotid artery aneurysms * Aortic aneurysms * Thrombosis * Embolism to the ophthalmic artery leading to visual disturbance and loss of vision

Answer 24

• In most cases the condition will last for around 2 years before complete remission CXR: - Can detect osteoarthritic change Rarely diagnostic if non-traumatic • MRI o allows assessment of root and cord compression o helps exclude spinal cord tumour and nerve root infiltration by granulomatous tissue • Needle Electromyography (EMG)

Answer 25

A disorder of uric acid metabolism causing recurrent bouts of acute arthritis caused by deposition of monosodium urate crystals in joints, and also soft tissues and kidneys

Answer 26

* The main metabolic disturbance is hyperuricaemia * This may be caused by: Increased urate intake or production via : • Increased dietary intake • Increased nucleic acid turnover (e.g. lymphoma, leukaemia, psoriasis) • Increased synthesis of urate (e.g. Lesch-Nyhan syndrome) Decreased Renal Excretion via: • Idiopathic • Drugs (e.g. ciclosporin, alcohol, loop diuretics) • Renal dysfunction

Answer 27

* 10 x more common in MALES * Very rare pre-puberty * Rare in pre-menopausal women * More common in HIGHER social classes

Answer 28

- Precipitating factors: • Trauma • Infection • Alcohol • Starvation • Introduction or withdrawal of hypouricaemic agents Symptoms: • Sudden excruciating monoarticular pain - Usually affecting the metatarsophalangeal joint of the great toe (podagra) • Symptoms peak at 24 hrs • They resolve over 7-10 days • Sometimes, acute attacks can present with cellulitis, polyarticular or periarticular involvement • Attacks are often recurrent • Patients are symptom-free between attacks (INTERCRITICAL GOUT: - asymptomatic period between acute attacks )

Answer 29

• Chronic Tophaceous Gout Follow repeated acute attacks Symptoms: • Persistent low-grade fever • Polyarticular pain with painful tophi (urate deposits) Best seen on tendons and the pinna of the ear • Symptoms of urate urolithiasis (renal calculi symptoms)

Answer 30

``` • Synovial Fluid Aspirate -Monosodium urate crystals will be seen -They are: • Needle-shaped • NEGATIVE birefringence under polarised light microscopy -Microscopy and culture will also be performed to exclude septic arthritis • Bloods FBC - raised WCC U&Es Raised urate Raised ESR • AXR/KUB Film Uric acid renal stones may be seen ```

Answer 31

Joint inflammation resulting from intra-articular infection. Known as Septic Arthritis

Answer 32

``` • May be idiopathic • In most cases there is systemic infection allowing for haematogenous spread • Risk Factors - Recent orthopaedic procedures - Osteomyelitis - Diabetes - Immunosuppression -Alcoholism • Common causative organisms: Bacteria • All ages - Staphylococcus aureus - TB • <4yrs - Streptococcus pneumoniae - Streptococcus pyogenes - Neisseria meningitidis - Gram-negativerods • 16-40 yrs - Neisseriagonorrhoeae ``` ``` Viruses • Rubella • Mumps • Hepatitis B • Parvovirus B19 Fungi • Candida ```

Answer 33

- Common in Children and Elderly

Answer 34

• Fever • Excruciating joint pain • Joint redness, swelling and loss of joint function • Usually a monoarthropathy (usually affecting one large joint) NOTE: it may cause a polyarthropathy in the immunosuppressed • Tuberculous arthritis develops more slowly and is more chronic

Answer 35

* Painful, hot, swollen * Immobile joint * Erythema * Severe pain prevents passive movement * Pyrexia * Look for signs of aetiology

Answer 36

• Joint Aspiration (IMPORTANT) - In infective arthritis, the aspirate will be grossly purulent - Send synovial fluid for MC&S (Microscopy - rule out crystal arthritis) - PCR may be used if a viral cause is suspected • Bloods - FBC - high WCC, high neutrophils - High CRP and ESR - Blood cultures - MC&S - Viral serology may be useful • Plain Joint Radiographs - Affected joint may look normal initially - Can show signs of damage following the infection • MRI Scan - Useful for detecting osteomyelitis

Answer 37

Pain, tingling and numbness that arise from nerve root compression or irritation in the lumbosacral spine

Answer 38

• Most commonly caused by a disc herniation • Other causes: - Spinal stenosis - Spondylolisthesis -Spinal injury or infection - Tumour - Cauda equina syndrome • Risk Factors - Age - older people are more like to get slipped discs - Job that requires regular heavy lifting

Answer 39

- COMMON | - more common in males

Answer 40

* Pain (usually affects the buttocks and the legs more than the back) * Numbness * Tingling sensation that radiates from the lower back down one of the legs * Weakness in the calf muscles or the muscles that move the foot or ankle

Answer 41

• CLINICAL diagnosis • Straight Leg Raise If pain in the distribution of the sciatic nerve is reproduced on passive flexion of the straight leg at the hip between 30-70 degrees then it is considered a positive sign (Lasegue's sign) This test is sensitive but not very specific • CT/MRI - helps visualise a lumbar disc herniation

Answer 42

Age-related degenerative joint disease when cartilage destruction exceeds repair, causing pain and disability

Answer 43

• Can be classified according to the distribution of affected joints • Pathogenesis Synovial joint cartilage destruction Eventually, there is loss of joint volume due to altered chondrocyte activity Patchy chronic synovial inflammation Fibrotic thickening of joint capsules • Primary Osteoarthritis UNKNOWN aetiology Multifactorial causes • Secondary Osteoarthritis Other diseases lead to altered joint architecture and stability Commonly associated diseases include: • Developmental abnormalities (e.g. hip dysplasia) • Trauma (e.g. previous fractures) • Inflammatory (e.g. rheumatoid arthritis, gout, septic arthritis) • Metabolic (e.g. haemochromatosis, acromegaly)

Answer 44

* COMMON * 25% of those > 60 yrs * More common in FEMALES, CAUCASIANS and ASIANS

Answer 45

``` • Joint pain and discomfort Use-related • Stiffness or gelling after inactivity • Difficulty with certain movements • Feelings of instability • Restriction walking, climbing stairs and doing manual tasks • Systemic features are usually absent ```

Answer 46

• Local joint tenderness • Bony swellings along joint margins Heberden's Nodes - DISTAL interphalangeal joint Bouchard's Nodes - PROXIMAL interphalangeal joint • Crepitus and pain during joint movement • Joint effusion • Restriction of range of joint movement

Answer 47

``` • Joint X-Ray of the affected joint will show FOUR classic features: - Loss of joint space (narrowing) - Osteophytes - Subchondral cysts - Subchondral sclerosis ```

Answer 48

• Infection of the bone leading to inflammation, necrosis and new bone formation. Can be acute, subacute or chronic. • Causative organisms: - Staphylococcus aureus - Group A Streptococcus

Answer 49

``` • Bacterial infection from indirect inoculation from skin (e.g. trauma, operative, chronic skin ulcers, haematogenous spread) • Risk Factors - Diabetes -Immunosuppression -IV drug use -Prostheses -Sickle-cell anaemia ```

Answer 50

* Mostly in YOUNG CHILDREN | * < 20% of cases are in adults

Answer 51

* Pain in the affected area * Fever * Malaise * Rigors * History of preceding skin lesion, sore throat, trauma or operation * NOTE: infants may not show localising signs

Answer 52

* Localised erythema * Tenderness * Swelling * Warmth * Painful/limited movement of affected limb * Seropurulent discharge from an associated wound or ulcer

Answer 53

``` • Bloods -FBC -Blood culture -ESR -CRP • Swabs of wound or discharge • Radiographs • Radioisotope bone scan - shows areas of increased activity ```

Answer 54

An inflammatory condition of unknown cause, which is characterised by severe bilateral pain and morning stiffness of the shoulder, neck and pelvic girdle - does NOT cause weakness

Answer 55

• UNKNOWN • Genetic and environmental factors • Associations - Temporal Arteritis • 40-50% of people with temporal arteritis have polymyalgia rheumatica • 15% of people with polymyalgia rheumatica will go on to develop temporal arteritis • Both conditions respond to corticosteroids

Answer 56

* Relatively common * Occurs in people aged > 50 yrs * Peak age of onset: 73 yrs * 3 x more common in FEMALES

Answer 57

• Tend to be relatively non-specific • Usual inclusion criteria for polymyalgia rheumatica: Age > 50 yrs Duration of symptoms > 2 weeks Bilateral shoulder or pelvic girdle aching, or both Morning stiffness lasting > 45 mins High ESR/CRP • The characteristic clinical picture of polymyalgia rheumatica: bilateral shoulder pain and stiffness of acute or subacute onset with bilateral arm tenderness • NO WEAKNESS • Symptoms are worst when walking • Morning stiffness may be so bad that they find it difficult to get out of bed, or raise their arms enough to brush their hair • May be flu-like symptoms at onset

Answer 58

* ESR/CRP - raised in polymyalgia rheumatica * FBC * U&Es * LFTs * Bone profile * Protein electrophoresis * TFTs * Creatine kinase * Others: urinary Bence Jones proteins, autoantibodies (e.g. anti-CCP antibodies)

Answer 59

* CORTICOSTEROIDS * Steroid-sparing agents (e.g. methotrexate) are sometimes used * Assistance from physiotherapy and occupational therapy * Monitor for adverse effects of steroids (e.g. osteoporosis)

Answer 60

* Temporal arteritis * Relapse of disease * Complications of steroid use (e.g. fracture risk)

Answer 61

* 15% risk of getting temporal arteritis * Variable course and prognosis * Usually responds rapidly to steroid treatment * Relapse is common

Answer 62

Connective tissue diseases characterised by inflammation of muscles

Answer 63

* Autoimmune basis | * Viral infection has been implicated in its pathogenesis

Answer 64

* Polymyositis presents between 30-60 yrs * Dermatomyositis can occur at any age (peak onset: 5-10 (children) and 50 (adults)) * Both diseases are 2 x more common in FEMALES

Answer 65

• Polymyositis - Inflammatory myopathy with onset over weeks or months -Steady progression of symptoms - Diffuse weakness in proximal muscles: • Causing difficulty rising from a low chair, climbing steps, lifting objects and combing hair • Also fatigue, myalgia and muscle cramps - Distal muscles are spared - so fine motor coordination tends to be preserved in the early stages - Pharyngeal weakness --> dysphagia - NO rash • Dermatomyositis - Inflammatory myopathy with onset over weeks or months - RASH - Systemic upset with fever, arthralgia, malaise and weight loss - Possible cardiac disease (e.g. conduction blocks, tachyarrhythmia) - GI ulcers and infections - Interstitial lung disease (30-50%) - Children have more non-muscular features (e.g. GI ulcers and infections)

Answer 66

• Polymyositis - Muscle weakness - NOT painful in most patients - Proximal myopathy - Extraocular muscles and distal muscles are spared - Weak forced flexion of the neck - Muscular atrophy - Muscles may be tender on palpation • Dermatomyositis - RASH characteristics: • Blue-purple discolouration of the upper eyelids with periorbital oedema • Flat red rash involving the face and upper trunk • Raised purple-red scaly patches over the extensor surfaces of joints and fingers - Rash may affect knees, shoulders, back and upper arms -Rash may be exacerbated by sunlight - Proximal myopathy - Muscle pain and tenderness in early disease

Answer 67

• Polymyositis - Creatine kinase - up to 50 x higher than normal - Electromyography (EMG) - Muscle biopsy - DEFINITIVE TEST - Autoantibodies (e.g. myositis specific antibody, anti-Jo-1 antibody) - Enzymes (e.g. SGOT, SGPT, LDH) • Dermatomyositis - Creatine kinase - not as reliable as in polymyositis - Enzymes (SGOT, SGPT and LDH may be raised) - Autoantibodies • ANA • Anti-Mi-2 • Anti-Jo-1 (more common in polymyositis) - EMG : may be helpful but can be normal as well - Muscle biopsy

Answer 68

Arthritis associated with deposition of calcium pyrophosphate dehydrate (CPPD) crystals in the joint cartilage

Answer 69

• CPPD crystal formation is initiated in cartilage located near the surface of chondrocytes • It is linked with excessive calcium pyrophosphate production • This abundance of calcium pyrophoshpate leads to the formation of CPPD crystals • Shedding of crystals in to the joint cavity leads to acute arthritis • Most causes of joint damage predispose to pseudogout (e.g. osteoarthritis, trauma) • Rarer conditions that increase the risk of pseudogout: -Haemochromatosis -Hyperparathyroidism -Hypomagnesaemia -Hypophosphatasia • Precipitating factors: -Intercurrent illness -Surgery -Local trauma

Answer 70

- 2x more common in Women | - more common in elderly

Answer 71

``` • Acute Arthritis -Painful -Swollen Joint (e.g. knee, ankle, shoulder, elbow, wrist) • Chronic Arthropathy -Pain -Stiffness - Functional impairment • Uncommon Presentations -Tendonitis -Tenosynovitis -Bursitis ```

Answer 72

``` • Acute Arthritis -Red -Hot -Tender -Restricted range of movement -Fever • Chronic Arthropathy -Similar to osteoarthritis -Bony swelling -Crepitus -Deformity -Restriction of movement ```

Answer 73

• Bloods -High WCC in acute attacks -High ESR -Blood culture - to exclude septic arthritis • Joint Aspiration -Rhomboid, brick-shaped crystals -POSITIVE birefringence -Culture or Gram-staining to exclude septic arthritis • Plain Radiograph of the Joint - Chondrocalcinosis -Signs of osteoarthritis : loss of joint space, osteophytes, subchondral cysts, sclerosis

Answer 74

Characterised by a sterile arthritis occurring after an extra-articular infection (commonly GI or urogenital) - Reiter's Syndrome is defined as a triad of reactive arthritis, urethritis, and conjunctivitis

Answer 75

``` • Associated with infections: - GI: • Salmonella • Shigella • Yersinia • Campylobacter - Urogenital: • Chlamydia trachomatis (60%) - It is thought that initial activation of the immune system by a microbial antigen is followed by an autoimmune reaction that involves the skin, eyes and joints • HLA-B27 allele is identified in 70-80% of patients ```

Answer 76

* 20 x more common in MALES * Age of onset: 20-40 yrs * Seen in 2% of patients with non-specific urethritis

Answer 77

* Symptoms can develop 3-30 days after infection * Burning or stinging when passing urine (due to urethritis) * Arthritis * Low back pain (due to sacroiliitis) * Painful heels (due to enthesitis and plantar fasciitis) * Conjunctivitis

Answer 78

Signs of arthritis: - asymmetrical oligoarthritis - often affects the lower extremities - sausage-shaped digits Signs of conjunctivitis : - anterior uveitis (painful red eye) Oral Ulceration Circinate Balanitis - Scaling red patches on the glans Keratoderma Blenorrhagica - Brownish-red macules - Vesicopustules - Yellowish-brown scales - Found on the soles and palms Others - nail dystrophy - hyperkeratosis - onycholysis

Answer 79

``` • Bloods -FBC -High ESR and CRP -HLA-B27 testing • Stool or Urethral Swabs and Cultures -May be negative by the time the arthritis develops (because the arthritis occurs post-infection) • Urine -Screen for Chlamydia trachomatis • Plain X-Rays -Useful in chronic cases -Erosions seen at the entheses (insertion of tendons into bone) • Joint Aspiration To exclude septic or crystal arthritis ```

Answer 80

Chronic inflammatory systemic disease characterised by symmetrical deforming polyarthritis and extra-articular manifestations

Answer 81

``` • AUTOIMMUNE disease • UNKNOWN cause • Associated with other autoimmune diseases (e.g. Sjogren's syndrome) • HLA associations: -HLA-DR1 -HLA-DR4 ```

Answer 82

- common - 1% general population - 3x more common in females - peak incidence : 30-50years

Answer 83

``` • GRADUAL onset • Joint pain • Swelling • Morning stiffness • Impaired function • Usually affects peripheral joints symmetrically • Systemic Symptoms: Fever Fatigue Weight loss ```

Answer 84

``` • Arthritis - most common in the HANDS • Early Signs -Spindling of fingers -Swelling of MCP and PIP joints -Warm, tender joints -Reduction in range of movement • Late Signs -Symmetrical deforming arthropathy -Ulnar deviation of fingers as a result of subluxation (partial dislocation) of the MCP joints -Radial deviation of the wrist -Swan neck deformity - Boutonniere deformity - Z deformity of the thumb - Trigger finger (inability to straighten the finger, tendon nodule palpable) - Tendon rupture - wasting of small muscles of the hand - palmar erythema • Rheumatoid Nodules - firm subcutaneous nodules (usually found on the elbows, ulnar margin, palms and over extensor tendons) • Signs of complications ```

Answer 85

``` • Bloods -FBC - low Hb, high platelets -High ESR and CRP -Rheumatoid factor (found in 70% of RhA patients) • The presence of rheumatoid factor is associated with rheumatoid nodules and extra-articular manifestations - Antinuclear antibodies (30%) • Joint Aspiration - May be performed in the acute setting to rule out septic arthritis • Joint X-Ray - Deformity - Osteopaenia - Narrowing of joint space - Soft tissue swelling ```

Answer 86

Multisystem granulomatous inflammatory disorder

Answer 87

• UNKNOWN • Transmissible agents (e.g. viruses), environmental triggers and genetic factors have all been suggested • Pathogenesis: - An UNKNOWN antigen is presented on MHC class 2 complexes on macrophages to CD4+ T-lymphocytes - These accumulate and release cytokines - This leads to the formation of non-caseating granulomas in organs

Answer 88

* Most common in AFRICANS and SCANDINAVIANS | * Can occur at any age but tends to be in adults > 50 yrs

Answer 89

``` • General Symptoms - Fever -Malaise -Weight loss -Bilateral parotid swelling -Lymphadenopathy -Hepatosplenomegaly • Pulmonary Symptoms -Breathlessness -Dry cough - Chest discomfort -Minimal clinical signs • Musculoskeletal Symptoms -Bone cysts (e.g. dactylitis) -Polyarthralgia - Myopathy • Eye Symptoms -Keratoconjunctivitis sicca (inflammation of the conjunctivitis and surrounding tissues due to the eyes being dry) - uveitis - papilloedema • Skin Symptoms - Lupus pernio (red-blue infiltrations of the nose, cheeks, ears and terminal phalanges) - Erythema nodosum - Maculopapular eruptions • Neurological Symptoms - Lymphocytic meningitis - Space-occupying lesions - Pituitary infiltration - Cerebellar ataxia - Cranial nerve palsies - Peripheral neuropathy • Cardiac Symptoms - Arrhythmia - Bundle branch block - Pericarditis Cardiomyopathy - Congestive cardiac failure ```

Answer 90

• Bloods - High serum ACE - High calcium - High ESR - FBC - WCC may be low due to lymphocyte sequestration in the lungs -Immunoglobulins - polyclonal hyperglobulinaemia - LFTs - high ALP + GGT • 24 hr Urine Collection - Hypercalciuria • CXR - Stage 0 - may be clear - Stage 1 - bilateral hilariously lymphadenopathy - Stage 2- stage 1 with pulmonary infiltration and paratracheal node enlargement - Stage 3 - pulmonary infiltration and fibrosis • High-Resolution CT Scan - Check for diffuse lung involvement • Gallium Scan - shows areas of inflammation • Pulmonary Function Tests - Low FEV1 - FVC shows restrictive picture • Bronchoscopy and Bronchoalveolar Lavage - High lymphocytes - High CD4: CD8 ratio • Transbronchial Lung Biopsy (or lymph node biopsy) - Shows non-caseating granulomas consisting of: • Epithelioid cells (activated macrophages) • Multinucleate Langerhans cells • Mononuclear cells (lymphocytes)

Answer 91

Characterised by inflammation and destruction of exocrine glands (usually salivary and lacrimal glands) n.b. when associated with other autoimmune diseases, Sjorgren's syndrome is termed secondary

Answer 92

``` • UNKNOWN • Genetic associations: -HLA-B8 -HLA-DR3 • Associated autoimmune diseases: -Rheumatoid arthritis -Scleroderma -SLE -Polymyositis -Organ-specific autoimmune diseases (e.g. PBC, autoimmune hepatitis, myasthenia gravis) ```

Answer 93

* Fatigue * Fever * Weight loss * Depression * Dry eyes (keratoconjunctivitis sicca) - they will be gritty and sore * Dry mouth - leads to secondary dysphagia * Dry upper airways - leads to a dry cough and recurrent sinusitis * Dry skin or hair * Dry vagina - may cause dyspareunia * Reduced GI mucus secretions leads to reflux oesophagitis, gastritis and constipation

Answer 94

* Parotid or salivary gland enlargement * Dry eyes * Dry mouth or tongue * Signs of associated conditions

Answer 95

• Bloods - High ESR - High amylase (if salivary glands involved) • Autoantibodies - Rheumatoid factor - ANA - Anti-ENA (extractable nuclear antigens) • Schirmer's Test - A strip of filter paper is placed under the eyelid - Positive for Sjogren's syndrome if < 10 mm of the strip is wet after 5 mins • Fluorescein/Rose Bengal Stains - May show punctate or filamentary keratitis • Other Investigations - Reduced parotid salivary flow rate - Reduced uptake or clearance on isotope scan • Biopsy - of salivary or labial glands

Answer 96

Multi-system inflammatory autoimmune disorder 4/11 of the diagnostic criteria of the American College of Rheumatology provides high sensitivity and specificity for the diagnosis of SLE: SOAP BRAIN MD - Serositis - Oral Ulcers - Arthritis (non-erosive) - Photosensitivity - Bloods (haemolytic anaemia/ leukopenia/ thrombocytopenia) - Renal disease (urine casts/ proteinuria) - ANA - Immunological disorder (anti-dsDNA/ anti-Sm/ anti-phospholipid) - Neurological disease (psychosis/ seizures) - Malar rash - Discoid rash

Answer 97

* UNKNOWN * Tissue damage may be caused by vascular immune complex deposition * Could be due to a combination of hormonal, genetic and exogenous factors

Answer 98

* COMMON * 1-2/1000 * More common in the YOUNG * More common in AFRO-CARIBBEAN and CHINESE * 9 x more common in FEMALES

Answer 99

``` • General Symptoms: - Fever - Fatigue - Weight loss - Lymphadenopathy -Splenomegaly • Raynaud's phenomenon • Oral ulcers • Skin Rash - Malar rash - Discoid lupus (red scaly patches) - Atypical rashes (e.g. photosensitivity, vasculitis, urticaria, purpura) ``` • Systemic Involvement: - Musculoskeletal - arthritis, tendonitis, myopathy - Heart - pericarditis, myocarditis, arrhythmias, Libman-Sacks endocarditis - Lung - pleurisy, pleural effusion, basal atelectasis, restrictive lung defects - Neurological - headache, stroke, cranial nerve palsies, confusion, chorea - Psychiatric - depression, psychosis - Renal - glomerulonephritis

Answer 100

``` • Bloods - FBC - U&E - LFT - Raised ESR - Normal CRP Clotting - Complement • Autoantibodies -Anti-dsDNA (60%) - Rheumatoid factor (30-50%) -Anti-ENA - Anti-RNP - Anti-SM -Anti-Ro - Anti-La - Anti-histone - Anti-cardiolipin • Urine - haematuria, proteinuria, red cell casts • Joints - plain radiographs • Heart and Lungs - CXR, ECG, echocardiogram, CT • Kidneys - renal biopsy (if glomerulonephritis suspected) • CNS - MRI scan, lumbar puncture ```

Answer 101

Rare connective tissue disease characterised by widespread small blood vessel damage and fibrosis in skin and internal organs - Also known as scleroderma - It's a spectrum of diseases ``` - Pre-Scleroderma • Raynaud's phenomenon • Nail-fold capillary changes • Antinuclear antibodies - Diffuse Cutaneous Systemic Sclerosis (40%) • Raynaud's phenomenon • Followed by skin changes with truncal involvement • Tendon friction • Joint contracture • Early lung disease • Heart, GI and renal disease • Nail-fold capillary dilatation - Limited Cutaneous Systemic Sclerosis (60%) • Previously known as CREST Syndrome because of its FIVE characteristic features: - Calcinosis - Raynaud'sphenomenon - (O)esophageal dysmotility - Sclerodactyly - Telangiectasia Scleroderma sine Scleroderma • Internal organ disease with NO skin changes ```

Answer 102

• UNKNOWN • Genetic and environmental factors • Pathogenesis is unclear • Activated monocytes, macrophages and lymphocytes may interact with: - Endothelial cells --> endothelial cell damage, platelet activation, narrowing of blood vessels - Fibroblasts --> lay down collagen in the dermis

Answer 103

* Age of onset: 30-60 yrs | * 3 x more common in FEMALES

Answer 104

• Skin - Raynaud's phenomenon • Hands - Initially swollen painful fingers - Later, they become thickened, tight, shiny and bound to underlying structures - Changes in pigmentation - Finger ulcers • Face - Microstomia (puckering of the skin around the mouth) - Telangiectasia • Lung - pulmonary fibrosis ---> pulmonary hypertension • Heart - pericarditis or pericardial effusion, myocardial fibrosis, heart failure, arrhythmias • GI - dry mouth, oesophageal dysmotility, reflux oesophagitis, gastric paresis • Kidneys - hypertensive renal crisis, chronic renal failure • Neuromuscular - trigeminal neuralgia, muscular wasting, weakness • Others - hypothyroidism, impotence

Answer 105

• Autoantibodies - Antinuclear - Anti-centromere (70% of limited cutaneous systemic sclerosis cases) -Anti-topoisomerase II (anti-Scl-70) - 30% of diffuse cutaneous systemic sclerosis cases - Anti-nucleolar - Anti-RNA polymerase • Lungs - CXR, pulmonary function tests, CT scan • Heart - ECG, echocardiography • GI - endoscopy, barium studies • Kidneys - U&Es, creatinine clearance • Neuromuscular - electromyography, biopsy • Joints - radiography • Skin - biopsy (rarely needed)

Answer 106

Vasculitis is the inflammation and necrosis of blood vessels - Primary vasculitides are classified based on the main vessel size affected: LARGE: - giant cell arteritis - Takayasu's aortitis MEDIUM: - polyarteritis nodosa - Kawasaki's disease SMALL: - churg-strauss syndrome - microscopic polyangiitis - Werner's granulomatosis - HEnoch-Schonlein purpura - Mixed essential cryoglobulinaemia (MEC) - Relapsing polychondritis

Answer 107

Large vessel vasculitides have classic clinical patterns based on the vessels affected (e.g. GCA and loss of vision/headache) Medium and small vessel vasculitides are characterised by multiorgan involvement and have less specific clinical features Possible features of ALL vasculitides: - General: fever, malaise, night sweats, weight loss - Skin: rash - Joint: arthralgia, arthritis - GI: abdominal pain, haemorrhage, diarrhoea - Kidneys: glomerulonephritis, renal failure - Lungs: dyspnoea, cough ,chest pain, haemoptysis, haemorrhage - CVS: pericarditis, coronary arteritis, myocarditis - CNS: mononeuritis multiplex, infarctions - Eyes: retinal haemorrhage, cotton wool spots Specific: GCA: loss of vision, jaw claudication, headache, scalp tenderness Polyarteritis Nodosa: microaneurysms, thrombosis, infarctions, hypertension, testicular pain Henoch-Schonlein Purpura: purpura, arthritis, gut symptoms, glomerulonephritis, IgA deposition Wegner's Granulomatosis: granulomatous vasculitis of upper and lower respiratory tract, nasal discharge, ulceration and deformity, haemoptysis, sinusitis, glomerulonephritis, saddle nose

Answer 108

• Bloods - FBC - normocytic anaemia, high platelets, high neutrophils - High ESR/CRP • Autoantibodies - e.g. cANCA in Wegner's • Urine - haematuria, proteinuria, red cell casts (if glomerulonephritis) • CXR - diffuse, nodular or flitting shadows, atelectasis • Biopsy - renal, lung, temporal artery (in GCA) • Angiography - to identify aneurysms (in PAN)