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Medicine year 4 CDM > Case 9: connective tissue disorders, Ankylosing spondylitis, gout, CPPD > Flashcards

Case 9: connective tissue disorders, Ankylosing spondylitis, gout, CPPD Flashcards

1
Q

Anti-nuclear antibodies and disease in connective tissue disorders

A
  • SLE=dsDNA, Smith
  • DLE (discoid lupus)= histone antibody
  • Diffuse systemic sclerosis: RNA pol III, SCL-70
  • Sjogrnes: SSA (Ro), SSB (La)
  • Nucleolar antibodies: SS andpolymyositis
  • Centromere antibodies: Limited SS
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2
Q

Sica symptoms

A
  • Lymphocytic infiltration of exocrine glands
  • Mucosal dryness - eyes, mouth, layrnx, pharynx & vagina
  • xerophthalmia (dry eyes), xerostomia (dry mouth)
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3
Q

Sjogren’s syndrome causes

A
  • Primary: systemic autoimmune disease
  • Secondary Sjogrens Syndrome when associated with another rheumatic condition or organ specific autoimmune disease: RA / SLE / Systemic Sclerosis / MCTD. Autoimmune Thyroiditis & Primary Biliary Cirrhosis
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4
Q

Sjogren’s syndrome

A
  • Increased risk of Non-Hodgkin lymphoma- most common subtype is MALT lymphoma
  • Increased incidence in females
  • Occurs in 40-50’s
  • Core symptoms: sicca, fatigue, joint involvement
  • Systemic, chronic inflammatory disorder causing lymphocytic infiltrates in the exocrine gland which can cause parotid swelling
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5
Q

Sjogren’s syndrome symptoms

A
  • Fatigue
  • Dry eyes (gritty, dry, red, painful, risk of infection)
  • Dry mouth (difficulty eating dry foods, dental caries, mouth ulcers)
  • Parotid and submandibular gland swelling - infiltrative process, ductal stones - obstruction’ & localised lymphoma).
  • Vaginal dryness - discomfort & infections
  • Joint involvement
  • Raynauls, myalgia, respiratory and GI disease and renal tubular acidosis
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6
Q

Sjogren’s syndrome investigations

A
  • Associated with: ANA, RF, ENA (Ro, La)
  • Serial: ESR, Complement, Immunoglobulins / EP-useful for screening for lymphoma
  • Schirmer’s Tear Test: positive if produces less then 5ml of tears in 5mins
  • Salivary flow: spitting as much saliva as you can in a pot which is weighed
  • Parotid and submandibular gland USS
  • Minor labial gland biopsy: used when Ro and La negative
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7
Q

Sjogrens syndrome management

A
  • Symptomatic: Eye drops (& eye surgical techniques), Saliva replacement (or + to saliva secretion), Topical oestrogen creams, good dental hygiene
  • Immunomodulatory: Hydroxychloroquine (for arthralgia and fatigue)
  • Screen patients for HIV and Hepatitis C
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8
Q

Mixed connective tissue disorder (MCTD)

A
  • Mixture of RA, SLE, Myositis, Scleroderma
  • Raynauds, digital ulcers
  • Puffy hands
  • Fatigue
  • Muscle Involvement (inflammatory myopathy)
  • Skin rash
  • Arthritis
  • ILD (Interstitial lung disease)
  • PAH (pulmonary arterial hypertension)
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9
Q

MCTD investigations

A
  • Has its own antibodies: RNP (anti-U1 RNP)
  • Look for erosive arthritis
  • If RNP positive screen for pulmonary hypertension, interstitial lung disease
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10
Q

Antiphospholipid syndrome

A
  • Screened when presenting to rheumatologist with autoimmune connective tissue disorder as can overlap with other conditions
  • An acquired autoimmune disorder
  • Recurrent venous or arterial thrombosis and/or foetal loss
  • Hypercoagulability and recurrent thrombosis can affect virtually any organ system: Peripheral venous system, CNS, Skin (levedo-reticularis rash)
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11
Q

Presentation of antiphospholipid syndrome

A
  • Peripheral venous system (DVT, PE)
  • CNS (stroke, sinus thrombosis, seizures, chorea, reversible cerebral vasoconstriction syndrome, headache)
  • Obstetric (pregnancy loss, eclampsia)
  • Pulmonary (PE, PH)
  • Dermatologic (livedo reticularis, purpura, infarcts/ulceration)
  • Cardiac (Libman-Sacks valvulopathy, MI, diastolic dysfunction)
  • Ocular (amaurosis, retinal thrombosis)
  • Adrenal (infarction/hemorrhage)
  • MSK (AVN of bone)
  • Renal (thrombotic microangiopathy)
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12
Q

Antiphospholipid syndrome diagnostic criteria

A
  • At least one clinical and one lab criteria
  • Clinical: Vascular thrombosis (arterial or venous), Pregnancy morbidity (3 miscarriages, 1 late miscarriage, premature birth <34 weeks of healthy neonate due to severe preeclampsia, eclampsia or severe placental insufficiency)
  • Lab criteria: Elevated levels of immunoglobulin G (IgG) or immunoglobulin M (IgM) anticardiolipin (aCL) Anti–beta-2 glycoprotein I OR Lupus anticoagulant
  • Lupus anticoagulant (DRVVT) must be on at least two occasions at least 12 weeks apart
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13
Q

Planning for pregnancy in autoimmune connective tissue disorder

A
  • COCP: not recommended with history of thrombosis, LAC or APL antibodies
  • Increased risk of: pre-eclampsia, eclampsia, pre-term delivery and foetal growth restriction
  • Disease inactivity for at least 6 months prior to conception
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14
Q

Pregnancy- autoimmune connective tissue disorder

A
  • Specialist unit with an MDT including an obstetrician and rheumatologist
  • Disease activity should be measured at baseline and at regular intervals
  • Maternal Ro/La: risk of foetal congenital heart block, If positive, a foetal cardiac scanning at 16-20 weeks
  • Antiphospholipid syndrome: test antibody early, LAC (lupus anticoagulant) confers the strongest predictor of adverse pregnancy outcome in SLE. Assess for the need for therapeutic LMWH
  • Low dose aspirin therapy
  • Flares of disease should be treated promptly with the lowest effective dose of prednisolone
  • Advise AGAINST pregnancy in some women- severe PAH
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15
Q

DMARD’s in pregnancy

A
  • Withdraw known teratogens: Mycophenolate, methotrexate and cyclophosphamide. Advice about termination, guide with foetal US
  • Hydroxychloroquine in all SLE pregnancies
  • Azathioprine is safe in pregnancy
  • Rituximab is not teratogenic- avoid in 2nd and 3rd trimester due to neonatal B cell depletion
  • In severe refractory maternal disease consider pulsed iv methylprednisolone, IVIG or 2nd or 3rd trimester cyclophosphamide
  • Sexually active men (including those post vasectomy) are recommended to use condoms with teratogenic treatment due to the risk of transfer of the drug in seminal fluid
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16
Q

Neonatal lupus

A
  • Due to transplacental passage of maternal autoantibodies
  • Gives lupus like rash
  • Manifestation: dermatological, cardiac (congenital heart block) and hepatic
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17
Q

Summary of sjogrens syndrome, MCTD and APLS

A
  • Sjogrens syndrome: Ro/La, sicca and fatigue, Lymphoma
  • MCTD: RNP, combination of symptoms, PAH/Jt erosion
  • APLS: DRVVT, anticardiolipin, Antiβ2glycoprotein, pregnancy morbidity, thrombosis
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18
Q

Back pain causes

A
  • Bone: malignancy, ankolysing spondylitis, vertebral osteomyelitis, disc herniation, spinal stensosi
  • Nerve: cauda equina syndrome
  • Muscle/skin: strain/zoster
  • Thoracic: PE, Dissection
  • Lumbar: AAA, cholecystitis, pancreatitis, pyelonephritis
  • Pelvic- PVD, endometriosis
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19
Q

Risk factors for Ankylosing spondylitis

A
  • Males 20-30
  • HLA-B27 associated spondyloarthropathy
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20
Q

Pathophysiology of ankolysing spondylitis

A
  • Chronic autoimmune inflammation
  • Inflammation causes destruction of intervertebral joints and sacroiliac joint
  • Fibrous bands are formed around spinal joints causing reduced mobility
  • Syndesmophytes are formed causing reduced movement of the vertebrae and fusion of the vertebrae
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21
Q

Spondyloarthropathies- negative for RF

A
  • Ankylosing spondylitis
  • Psoriatic arthritis
  • Enteropathic arthritis
  • Reactive arthritis
  • Juvenile apondylarthropathies
  • There is an association between HLA B27 with all these conditions
22
Q

Extra-articular features of ankolysing spondylitis

A
  • Anterior uveitis
  • Apical lung fibrosis
  • IgA nephropathy amyloidosis
  • Aortic insufficiency/AV block/IHD/ Aortic regurgitation
  • Amyloidosis
  • Cauda equina syndrome
  • Peripheral arthritis
  • Anaemia of chronic disease
  • Autoimmune bowel disease
  • (Achilles tendon)- Enthesitis (inflammation where tendons or ligaments insert in bone)
23
Q

Clinical examination of ankylosing spondylitis

A
  • Pain and stiffness in lower back and Sacroiliac pain (buttock region)
  • Stiffness is worse in the morning and improves with exercise
  • Clinical examination: reduced lateral flexion and forward flexion. Reduced chest expansion
  • Schober’s test: for forward flexion. Line is drawn 10cm above and 5cm below back dimples, distance between the two line should increase by >5cm when the patient leans froward
24
Q

Investigations for Ankylosing spondylitis

A
  • Bedside: urine dip, faecal calprotectin, ECG
  • Bloods: CRP, ESR
  • HLA-B27 genetic test
  • Imaging: x-rays, MRI, joint US
  • MRI: if uncertain shows inflammation of sacroiliac joints (bone marrow oedema)
  • Special: visual assessment, Echo, lung function, flexi sig
25
Q

X-ray for ankylosing spondylitis

A
  • Sclerosis of bone around the joint space
  • Widening of joint space
  • Sacroiliitis: subchondral erosions and sclerosis
  • Squaring of lumbar vertebra
  • Syndesmophytes: due to ossification of outer fibres of annulus fibrosus
  • Fusion of facet, sacroiliac and costovertebral joint
  • Ossificationof the ligaments, discs and joints (these structures start turning into bone
  • Bamboo spine: formation of syndesmophytes between vertebral bodies, appearance of completing bridging across vertebrae
  • Dagger sign: a single central radiodense line due to ossification of the supraspinous and interspinous ligaments secondary to enthesitis
26
Q

Management of ankylosing spondylitis

A
  • Conservative: patient education, specialist MDT management, Specialist nurses, Physiotherapy, Occupation therapy, adjustments to the home, ice packs, avoid smoking, exercise and movement
  • Medical: management if comorbidities, NSAIDS (often continuous), DMARDS (sulfasalazine- if peripheral joint involvement), Biologics (anti-TNFalpha, infliximab/adalimumab- if high disease activity)
  • Surgical: joint replacement
27
Q

Medical management of ankylosing spondylitis

A
  • Non-steroidal anti-inflammatory drugs(NSAIDs) are first-line
  • Anti-TNF medications are second-line (e.g., adalimumab, etanercept or infliximab)
  • Secukinumab or ixekizumabare third-line (monoclonal antibodies against interleukin-17)
  • Upadacitinibis another third-line option (JAK inhibitor)
  • Bisphosphonates in osteoporosis
28
Q

Septic arthritis

A

Red, hot, swollen joint. Painful and reduced range of movement

Will cause Tachycardia and a fever.

Medical emergency: regard a hot, swollen, acutely painful joint with restriction of movement as septic arthritis until proven otherwise.

29
Q

Septic arthritis: how pathogens are spread

A
  • Direct injury: injury to a joint with skin break or infected neighbouring bone (infection spreads into joint)
  • Haematogenous: infection in other organs and spreads to joint via blood stream

Bacterial toxins destroy cartilage and cause progressive joint destruction

30
Q

Septic arthritis pathogens

A
  • Gonococcal arthritis: Neisseria gonorrhoea, haematogenous spread from sexually transmitted goncoccal infection
  • Non gonococcal arthritis: staph aureus- may be direct infection from a wound, can cause rapid joint destruction in days
31
Q

Septic arthritis risk factors

A
  • Established joint disease
  • Recent joint injection/sugery
  • Immunosuppression- diabetes, alcoholism
  • IVDU
  • Prosthetic joints
  • UTI, indwelling catheter, recent abdominal surgery
32
Q

Septic arthritis investigations

A
  • Bedside: Obs, urine dip, ECG, CXR (for haematogenous spread infection)
  • Bloods: FBC, U&E, LFT, CRP, Lactate, Coag, culture
  • Imaging: X-ray is not diagnostic is useful to see baseline joint condition. May see increased synovial fluid or bone destruction
  • Special- joint aspiration
33
Q

Septic arthritis Management

A
  • IV ABX
  • Analgesia
  • May require joint washout with surgeons
34
Q

Arthrocentesis

A
  • Contraindications: overlying skin infection, anti-coagulation, low platelets
  • Aspirate to dryness
  • Look at colour, viscosity and clarity of the joint aspirate
  • Send for: gram stain, WCC, microscopy, culture, polarising microscopy (for crystals)
  • Once done give IV antibiotics, immobilise the joint and analgesia
35
Q

Aspirate in septic arthritis

A

The aspirate will look thick, yellow and turbid. It will return as ‘positively birefringent rhomboid shaped crystals under polarised light microscopy.’

36
Q

Different crystals in aspirate

A
  • Positively birefringent rhomboid shaped crystals under polarised light microscopy- calcium pyrophosphate crystals. CPPD or pseudogout.
  • Strongly negative birefringent needle shaped crystals under polarised light microscopy- monosodium urate crystals, Gout
37
Q

Calcium Pyrophosphate Deposition Disease (CPPD)

A

Calcium pyrophosphate dihydrate crystal deposition in articular cartilage. Also known as pseudogout

Usually effects individuals >50

38
Q

Causes of CPPD

A
  • Sporadic
  • Secondary causes: Hyperparathyroidism, Haemochromatosis, Hypothyroidism, Diabetes, Low magnesium. Look for secondary causes in younger patients
39
Q

CPPD presentation

A
  • Joint swelling
  • Erythema
  • Pain
  • Oligo or polyarticular symptoms for days or weeks
40
Q

CPPD joints effected and joint aspiration

A

Acute CPP crystal arthritis (pseudogout)- knees, wrists and shoulders are effected, the crystals stimulate inflammation in the joints. Most commonly effected joint is the knees

Joint aspiration- white chalky fluid, crystals are positively birefringent rhomboid shaped. Always do joint aspiration to exclude septic arthritis

41
Q

CPPD x-ray

A

Chondrocalcinosis. In the knees this in linear calcification of the meniscus and articular cartilage. Other joint x-ray changes are similar to osteoarthritis

42
Q

CPPD management

A
  • NSAIDs(e.g., naproxen) first-line (co-prescribed with aproton pump inhibitorforgastroprotection)
  • Colchicine
  • Intra-articular steroid injections(septic arthritis must be excluded first)
  • Oral steroids
43
Q

CPPD risk factors

A
  • haemochromatosis
  • hyperparathyroidism
  • acromegaly
  • low magnesium, low phosphate
  • Wilson’s disease
43
Q

Gout

A

Monosodium urate deposition in the joints, due to overproduction or under excretion. It is triggered following prolongedhyperuricaemiadue to purine breakdown which results in the accumulation ofmonosodium urate (MSU) crystalsin the joint

44
Q

Gout risk factors

A
  • Age 30-50
  • African-Caribbean
  • Male
  • High uric acid levels
  • Diet (purine rich foods – red meat, shellfish)
  • Obesity, diabetes
  • Alcohol use
  • Diuretics (decrease urate excretion)
  • Chemo agents – increased cell turn over
  • Polycythaemia Vera
45
Q

Gout presentation

A
  • Acute swollen hot painful joint
  • Intense stabbing pain
  • Erythema-red and ward
  • Usually 1st MTP joint (big toe)
  • Can effect peripheral joints like ankle, knee and finger
  • Monoarticular
  • May have asymptomatic periods and periodic flare ups
46
Q

Gout risk factors

A
  • Family history and genetics
  • Dietary: purine rich food- red meat an shellfish, high fructose beverages, alcohol particularly beer
  • Metabolic syndrome: obesity, hypertension and insulin resistance
  • Impaired renal function i.e. CKD causes impaired excretion of uric acid causing hyperuricaemia
  • Medication: thiazide diuretics, asprin at low doses and certain anti-tuberculosis medication
47
Q

Gout chronic disease

A

Gouty tophi (MSU deposition in the joint) with joint damage and chronic pain. Can have eye involvement where MSU crystals deposit in the cornea (rare).

48
Q

Gout investigations

A
  • X-rays shows tophi. Used in chronic gout-n shows punched out lytic lesions, sclerotic margins and tophi
  • Joint aspiration shows negatively birefringent crystals (gold standard). However, if the clinical diagnosis is clear aspiration is not always needed
  • High urate level: not diagnostic
  • Serum uric acid measurement: 4-6 weeks after first presentation, also in chronic gout for monitoring
  • Screen for cardiovascular risk and kidney disease
49
Q

Other investigations in gout

A
  • U&Es:renal function should be measured to ensure appropriate dose of allopurinol.
  • FBC:WBC may be raised
  • Fasting glucose and lipid profile: gout is associated with metabolic syndrome.
50
Q
A

Medicine year 4 CDM (41 decks)

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