RHEUMATOLOGY - Simply Revision · •81 year old female presents NWB with 4 days history painful...

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RHEUMATOLOGY

Dr Akon Esara

What we’re going to cover

Case-Based Approach to: • Common Presentations• Monoarthrits• Polyarthritis• Back Pain

• Common Conditions• Arthritis• CTDs• Vasculitis• Metabolic bone disease• Chronic pain

• Meds and Side Effects• OSCE spin

Acute Monoarthritis

Example Case 1…. To Kahoot

24M presents with an acutely hot swollen left knee having returned from holiday in Magaluflast week. On examination you note a pustular rash on his palms.

Example Case 2

A 47 year old diabetic man presents with a hot swollen left knee. The pain awoke him at night and was so painful he could not cover it with his sheet. He has a low grade fever. He reports a previous similar episode affecting his big toe a few months ago

Example Case 3

81 year old female presents, unable to weight bear with 4 days history painful swollen right knee. Temp 37.7C. Xray of the knee shows patchy demineralisation of the tibia and femur

Example Case 4

A 67 year old woman presents to accident and emergency unable to weight bear due to an acutely hot swollen knee. There is no history of trauma. She has a background of AF, for which she takes Apixaban.

Differential Diagnosis

• Septic Arthritis

• Crystal Arthritis

• Gout

• Pseudo-gout

• Haemarthrosis

• Post-traumatic

• Haemophilia

• Mono-arcticular presentation of a Poly-articular disease

• RA/PsA/Reactive/OA

How to differentiate….

History & Examination Investigations

History

• M/F? Young or Old?

• B/G: Drugs, Co-morbidities, Etoh

• Onset: Noctural? Post trauma?

Previous episodes?

• Other symptoms e.g. Rash, fever

Examination

• Hot or Not

• Weight bearing/ROM

• Other Clues

▫ Rashes

▫ Tophi

▫ Bruising

• Bloods

▫ WBC

▫ Plts/Clotting

▫ Inflammatory Markers

▫ U+E

▫ Uric Acid

• Xray

▫ Chrondrocalcinosis

▫ Erosions/Damage

• ASPIRATE

SEPTIC ARTHRITIS

Septic Arthritis

• Acute painful monoarthritis

(with fever)

• Irreversible loss of joint

function in 25-50%

• Mortality 10%

• >1 joint involved in 20%

• DON’T HESITATE, ASPIRATE

Septic Arthritis

Bugs Management

Organism

Staph. Aureus Most common

Underlying joint disease

(OA, RA)

N. Gonorrhoea Sexually active adults

Migratory Polyarthritis

Pustular Rash

Kleb.

Pneumoniae

Alcoholics

Gram

Negatives

Malignancy

Immunosuppresives

Pseudomonas IVDU

• Aspirate

• Blood Cultures

• IV Antibiotics

• NWB

• Washout

CRYSTAL ARTHROPATHIES

Gout

PODAGRA

TOPHIMETABOLIC

SYNDROME

DIURETICS LIFESTYLE

NEGATIVELY BIREFRINGENT

NEEDLE-SHAPED CRYSTALS

JOINT DAMAGE

EROSIONS

Gout: Treatment

Acute Phase Prevention: Urate Lowering Therapy

• NSAIDS

▫ Indomethacin, Naproxen,

Ibuprofen etc

▫ Caution:

➢ Renal impairment

➢ CV disease

• Colchicine

▫ Low doses

▫ Diarrhoea (lactose free diet helps)

• Steroids (PO/IA)

• Biologics: Anti IL1

▫ Anakinra

▫ Canakinumab

• Xanthine Oxidase Inhibitors▫ Allopurinol▫ Febuxostat

• Uricosuric Agents▫ Probenecid▫ Benzbromarone

• DO NOT START UNTIL ACUTE ATTACK RESOLVED

• Risk of gout flare at initiation:colchicine/NSAID prophylaxis

Crystal Arthropathies: Pseudogout

OLDER PATIENTS

MONO/POLYARTHRITIS

OA is RF

CHONRDOCALCINOSIS

WEAKLY POSITIVE BIREFRINGENT

RHOMBOID-SHAPED CRYSTALS

Crystal Arthropathies: The Rest

• Calcium Oxalate Crystals

• Dialysis patients

• Basic Calcium Phosphate Crystals

• Calcific tendonitis

• Hydroxyapatite Crystals

• Milwaukee Shoulder: Severe destructive arthritis

Haemarthrosis

• Not always post-trauma

• Twisting Injuries

• Haemophilia

▫ May progress to chronic

destructive arthritis with

time

• Iatrogenic

▫ Warfarin, DOACs, Dual

Antiplatelets

Example Cases

• 24M presents with an acutely hot swollen left knee having returned from holiday in Magaluf last week. On examination you note a pustular rash on his palms.

• 47 year old diabetic man presents with a hot swollen left knee. The pain awoke him at night and was so painful he could not cover it with his sheet. He has a low grade fever. He reports a previous similar episode affecting his big toe a few months ago

• 81 year old female presents NWB with 4 days history painful swollen right knee. Temp 37.7. Xray of the knee shows patchy demineralisation of the tibia and femur

• A 67 year old woman presents to accident and emergency unable to weight bear due to an acutely hot swollen knee. There is no history of trauma. She has a background of AF, for which she takes Apixaban.

Gonococcal Septic Arthritis

Gout

Septic Arthritis

Haemarthrosis

POLYARTHRITIS

Example Case 5: (Kahoot Again)

52F presents with 1 year progressive joint pain, initially

affecting her right knee before spreading to her left hip and

hands. The pain worsens on activity. She has noticed lumps

on her DIPJs. She is weakly RhF+.

Example Case 6:

31F presents with 6 weeks history of pain in her hands and

feet which now prevents her from sleeping or picking up

her baby. She feels stiff in the morning for 2 hours. Bloods

show ESR 60, CRP 42.

Example Case 7:

45M presents with right knee and ankle pain. He also has

noticed his left 2nd toe has become diffusely swollen. He is

stiff in the morning for >30mins. You notice nail pitting on

examination.

Oligo/Polyarthritis

INFLAMMATORY NON-INFLAMMATORY

• Rheumatoid

• ‘Seronegatives’➢ Psoriatic

➢ Enteropathic

➢ Reactive

➢ AS

• CTDs➢ SLE

➢ Sjogrens

➢ etc

• Osteoarthritis

• Fibromyalgia

Describing Arthritis

• Inflammatory vs. Non-Inflammatory

• Acute vs. Chronic

• Small vs. Large joint

• Symmetric vs. Asymmetric

• Oligo vs. Poly vs Axial

• Oligo: 2-4 joints

• Poly: ≥5

• Axial: Shoulders/Spine/Hips

Describing Arthritis: Practice

• Asymmetric

• Polyarthritis

• Small and Large joint involvement

• Duration: >1year, Progressive

• Pain worse on activity, alleviated on rest

OSTEOARTHRITIS

Describing Arthritis: Practice (2)

• Symmetric

• Polyarthritis

• Small and Large Joint involvement but DIPJs spared

• 8 weeks duration

• Night pain, morning stiffness >1h

RHEUMATOID

Differential:

Psoriatic Arthritis

Describing Arthritis: Practice (3)

• Asymmetric

• Polyarthtiris

• Small, large and Axial joints

including DIPJs

• Relapsing/Remitting over 8 months

• 1h morning stiffness

PSORIATIC ARTHRITIS

Describing Arthritis: Practice (4)

• Asymmetric

• Axial/Large joint

• Insidious onset over last 9 months

• Initially left buttock, progressive

spread

• Morning stiffness >1h

ANKYLOSING SPONDYLITIS

OSTEOARTHRITIS

OSTEOARTHRITIS

• Most common form of arthritis

• Incidence increases with age

• Genetic component

• Cartilage loss

• Primary or secondary

• Weight bearing joints, joints of hands ‘generalised nodal OA’

• Management:

▫ Education

▫ Exercise/Physio

▫ Pharmacological

➢ Topical, simple analgesics

➢ IA corticosteroids

▫ Surgery

OA: X-ray findings

OSTEOPHYTES

SUBCHONDRAL

SCLEROSIS

(BONE CYSTS)

LOSS OF JOINT

SPACE

RHEUMATOID ARTHRITIS

RA: PATHOGENESIS

• Genetic component

• Autoimmune synovial inflammation

and proliferation

• Cytokine mediated

▫ TNFα

▫ IL-1

• Synovial hypertrophy causes

cartilage and bone destruction →

progressive joint damage

RA: Presentation

• Insidious onset• F>M (3:1)• Classic onset 40-60

• Inflammatory pain• Pain improving on activity• Swelling• Stiffness

• Classically symmetrical small joint involvement

• Examination: Swelling and tenderness of joints• Later: deformity (avoidable)

RA: Investigations

Blood Tests Imaging/Other

• Inflammatory Markers

• Antibodies

▫ RhF

➢ Antibody against the Fc portion of IgG. Predominantly IgM

➢ Specificity 60-70%

▫ CCP

➢ >95% Specificity

▫ ANA

• FBC

• Anaemia of chronic inflammation/ ^plts

• U&E, LFT

• ?DMARDs

• Plain Xrays

▫ Likely normal initially

▫ Periarticular osteopenia

▫ Joint space narrowing

▫ Erosions

• US/MRI

▫ More sensitive for early damage

▫ Show disease activity

RA for the OSCE: Rheumatoid Hands

• Ulnar Deviation

• Swelling and

Subluxation MCPJs

• Boutonniere

Deformities

• “Z” thumb

• Skin thinning

• Nodules (elbows)

• Signs of carpal tunnel

RA is a MULTISYSTEM DISEASE

RA: Treatment

Symptom Control Disease Modifying Drugs (DMARDS)

• Anaglesia

• NSAIDS

• COX-2 inhibitors

• Steroids

• Short term control for flares,

PO/IM/IA

• MDT ▫ Specialist nurse

▫ Physio

▫ Hand therapy, orthotics etc

• Synthetic: csDMARDS

• Biologic: bDMARDs

• Targeted Synthetic:

tsDMARDS

DMARDS

Methotrexate: Counselling

• WEEKLY • Co-administration with Folic acid to minimize SEs

▫ GI upset, mouth ulcers, hair fall• Limit alcohol intake to Govt. guidelines (i.e. max

14units/week)• Teratogenic- advise re: contraception to women of

childbearing age (stop 3 months preconception)• Blood monitoring:

▫ FBC, LFTs▫ fortnightly until established on stable dose for 6 weeks,

then monthly, then 2-3 montly

DMARDS (the rest)

• Sulfasalazine

▫ Monitor FBC and LFTs

▫ Safe in pregnancy/breast feeding

• Hydroxychloroquine

▫ Antimalarial

▫ All Lupus patients should be taking

▫ Macular damage

• Leflunomide

▫ Blood monitoring as for Methotrexate

▫ Teratogenic

▫ Long 1/2t: need washout if infection/pregnancy

▫ Can increase BP

Biologic Drugs in Rheumatoid Arthritis

• Anti-TNF

▫ Infliximab, Adalimumab (Humira),

Etanercept, Certoluzimab,

Golimumab

• Rituximab

▫ Anti-CD20 chimeric Ab

▫ Used in Chemo, RA, SLE

(nephritis), Vasculitis

▫ Hypogammaglobulinaemia

• Tocilizumab

▫ Anti- IL6- suppresses CRP

• Abatacept

▫ Anti-CTLA4

• JAK inhibitors (tsDMARDs)

▫ Barictinib, Tofacitinib.

Significant Immune Suppression

▫ Risk of severe sepsis

▫ Reactivation of TB

➢ CXR, IGRA

▫ Reactivation of Hepatitis

➢ Hep B+C screen

• PML

• Cancer Risk (recurrence/non-

melanoma skin cancer)

SERONEGATIVE

SPONDYLOARTHROPATHIES:

Psoriatic, Enteropathic, Reactive (and AS)

Seronegative Arthritis: Psoriatic

• 15-20% arthritis precedes skin Psoriasis

• Five Classical Patterns▫ Asymmetrical Oligoarticular▫ Symmetrical Polyarthritis (RA mimic)▫ DIPJ Arthropathy▫ Spondylitis +/- Sacroiliitis (AS mimic)▫ Arthritis mutilans

• Classical Xray findings▫ “Pencil in Cup”▫ Erosions and New bone formation

• Treatment: DMARDs (MTX), TNF

Seronegative Arthritis: Reactive/Enteropathic

Reactive Enteropathic

• 2-6 weeks after infection (may have been asymptomatic)

• Gut infections/STIs/post strep▫ Chlamydia Trachomatis▫ Yersinia, Salmonella, Shigella,

Campylobacter• Asymmetric Oligoarthritis, Dactlylitis,

Eye symptoms, occasionally rash, urethritis

• Investigate:▫ STI swabs▫ ASOT▫ Aspirate

• Treatment:▫ NSAIDS▫ Short course Pred▫ DMARDs if persistent

• Patients with IBD

• Similar pattern to other SpAs

Example Cases

• 52F presents with 1 year progressive joint pain, initially affecting her right knee before spreading to her left hip and hands. The pain worsens on activity. She has noticed lumps on her DIPJs. She is weakly RhF+.

• 31F presents with 6 weeks history of pain in her hands and feet which now prevents her from sleeping or picking up her baby. She feels stiff in the morning for 2 hours. Bloods show ESR 60, CRP 42.

• 45M presents with right knee and ankle pain. He also has noticed his left 2nd toe has become diffusely swollen. He is stiff in the morning for >30mins. You notice nail pitting on examination.

Osteoarthritis

Rheumatoid Arthritis

Psoriatic Arthritis

BACK PAIN

Back Pain: Example Case 8:

32M presents with intermittent flitting buttock pain for the last 1 year. He initially attributed the first episode to a football injury but subsequent episodes had no preceding injury. The pain dull and radiates down his left leg. The pain wakes him from sleeping. He feels stiff in the morning for 30minutes.

Back Pain: Example Case 9:

41M with pain in his low back radiating down his left leg. The pain is sharp and worse on movement. He reports paraesthesia on the lateral aspect of his foot. On examination his pain is increased on passive dorsiflexion of the foot during straight leg raise.

Back Pain: Example Case 10:

75F with longstanding Rheumatoid Arthritis on anti-TNF. Presents with severe sharp thoracic pain for last 1 month. The pain is worse on movement, but also prevents her from sleeping. The pain radiates around her chest wall.

Back Pain: Example Case 11.

62F presents with severe constant low back pain for a few months. PMHx Ca Breast 12 years ago, given all clear. On examination she has a sensory level at L2 and brisk reflexes bilaterally.

Back Pain: Red Flags

• Age of onset <20 or >55 years• Recent history of violent trauma• Nocturnal Pain• Thoracic Pain• History of Cancer• Unexplained weight loss• Systemically unwell• Fever• IVDU, immunosuppression, HIV• Prolonged use of corticosteroids• Neurological deficit• Bowel or bladder dysfunction

Back Pain: Yellow Flags

• Attitude that pain is harmful or potentially severely

disabling

• Fear avoidance behavior, reduced activity levels

• Expectation that passive vs active treatment will be

beneficial

• Tendency to depression, low morale, social withdrawal

• Social or financial problems

Mechanical Back Pain

• Common: >85% of population experience LBP at some

point in life

• Usually resolves in 2-4/52

• Usually inciting event

• Treatment:

• Reassurance

• Stretches, Exercises, PT

• Analgesia

Disc Prolapse

• Sometimes no inciting event

• Low back pain accompanied by

radicular symptoms

▫ Shooting, Electric Shock, Burning,

Tingling in dermatomal dist.

▫ Sciatica = L5/S1 disc herniation. S1

dermatomal radiculopathy. Positive

sciatic stretch test. EHL weakness

Cauda Equina/Spinal Cord Compression

• Causes

▫ Malignant

▫ Degenerative

• Symptoms

▫ Low back pain (constant, nocturnal)

▫ Paraesthesia/sensory loss

▫ Weakness

▫ Sphincter disturbance

• Signs

▫ Sensory level

▫ UMN weakness if compression at

cord level

▫ LMN if below

• Investigations

▫ MRI whole spine URGENTLY

• Action

▫ Neurosurgeons

▫ High dose dexamethasone for

malignant tumours, Radiotherapy

AXIAL

SPONDYLOARTHROPATHY

(ANKYLOSING SPONDYLITIS)

Ankylosing spondylitis

• Axial Spondyloarthropathy with progression to Ankylosis of Axial Skeleton

• Ossification of ligaments and costovertebral joints

• Insidious Progressive Symptom onset

• Inflammatory, dull pain lumbosacral junction

• Classically affects men 20-40 years

Ankylosing Spondylitis: OSCE favourite

• LOOK:

▫ Loss of Lumbar Lordosis

▫ Exaggerated Thoracic Kyphosis

▫ Hyper-extension of neck to compensate

▫ = “Question Mark Posture”

• FEEL:

▫ Palpate for spinal/paraspinal tenderness

▫ Sacroiliac stress test

• MOVE:

▫ Globally restricted range of movement all planes

• SPECIAL TESTS:

▫ Schoeber’s

▫ Wall-Occiput

▫ Chest Expansion

AS: Extra-Articular

Features

Anterior Uveitis

Apical Lung Fibrosis

Aortic regurgitation

AV nodal block

IgA Nephropathy

Amyloidosis

Achilles Tendonopathy

AS: Investigations and Treatment

Investigations Treatment

• Patient Education

• Physiotherapy

• NSAIDs

• Anti-TNF

• Anti- IL17 (Secukinumab)

HLA-B27

Back Pain: Example Cases

• 32M presents with intermittent flitting buttock pain for the last 1 year. He initially attributed the first episode to a football injury but subsequent episodes had no preceding injury. The pain dull and radiates down his left leg. The pain wakes him from sleeping. He feels stiff in the morning for 30minutes.

• 41M with pain in his low back radiating down his left leg. The pain is sharp and worse on movement. He reports paraesthesia on the lateral aspect of his foot. On examination his pain is increased on passive dorsiflexion of the foot during straight leg raise.

Ankylosing Spondylitis

Sciatica (L5/S1 disc

prolapse)

Back Pain: Example Cases

• 75F with longstanding Rheumatoid Arthritis on anti-TNF. Presents with severe sharp thoracic pain for last 1 month. The pain is worse on movement, but also prevents her from sleeping. The pain radiates around her chest wall.

• 62F presents with severe constant low back pain for a few months. PMHx Ca Breast 12 years ago, given all clear. On examination she has a sensory level at L2 and brisk reflexes bilaterally.

Vertebral crush fracture

Spinal Cord Compression

CONNECTIVE TISSUE

DISEASES

Example Case 12

21F presents with sharp central chest pain and dull aches in her hands and feet. Over the last few months she reports profound fatigue, mouth ulcers and arthralgia. Over summer she noticed a rash on her hands which has now resolved.

Example Case 13

41M presents profoundly short of breath. An ECHO shows severe pulmonary hypertension. He has a background of Raynaud's phenomenon.

Example Case 14

52F presents with several months of a gritty sensation affecting both eyes. She has also been profoundly fatigued, and has recently had difficulty swallowing solids without taking a sip of water to wash it down. Her blood tests show that she is Anti-Ro antibody positive.

Example Case 15

77F presents with a fall. She has profound flaccid weakness, more prominent in proximal muscle groups. On examination she is found to have a few violacious macules over her knuckles and a rash in a shawl distribution. Her family are concerned she has lost weight.

Connective Tissue Diseases

• SLE

• Systemic Sclerosis

▫ Limited vs Diffuse

• Idiopathic Inflammatory Myopathies

▫ Polymyositis

▫ Dermatomyositis

• Sjogren’s Syndrome

• Undifferentiated CTDs/Overlap

syndromes

SLE• Constitutional Symptoms

▫ Fatigue, weight loss, fevers

• Muco-cutaneous

• Mouth Ulcers

• Photosensitive rash

• Hair loss

• Raynauds

• MSK

• Arthritis (non erosive), Arthralgia, Myalgia

• Renal

• Lupus Nephritis- proteinurea/haematurea on dip

• CNS

• Headache, mood disorder, Mononeuritis, demyelination, Vasculitis, Seizure

• Cardiac

• Myo/Pericarditis, Libman-Sachs Endocarditis

• Respiratory

• Pleuritis, Pulmonary Fibrosis

• Haematological

• Antiphospholipid, Cytopenias, Haemolysis

SLE: Investigations

• Autoantibodies

▪ ANA

➢ 90-95% Sensitive, non-specific

▫ dsDNA

➢ 70% Sens, 95% Specific

▫ Extractable Nuclear Antigens

➢ Sm (pathognomic)

➢ RNP, Ro- can be positive but non-

specific

▪ Anti-Phospholipid Antibodies

• Other bloods

▫ ESR (CRP classically does not rise)

▫ Complement

▫ FBC, U+E, LFT etc- look for organ

involvement

• Urine

▫ Dip

▫ Sediment (Casts)

▫ Protein-Creatinine Ratio

• Imaging

▫ Not very useful for diagnosis

▫ Look for organ damage

SLE: Treatment

• Steroids• For acute flares, smallest effective dose

• Anti-Malarials• Hydroxychloraquine reduces all-cause mortality for SLE

• DMARDs• Azathioprine, Mycophenolate

• The strong Stuff• Cyclophosphamide – Nephritis, Myocarditis, CNS• Rituximab (Anti-CD20), Belimumab (Anti-BAFF)

• Others• Asprin/LMWH/Warfarin in Antiphospholipid

Systemic Sclerosis

• Rare Autoimmune disease characterisedby fibrosis of skin and internal organs with pronounced alterations in microvasculature

• F:M ratio 3:1

• Localised Scleroderma ▫ Morphea, linear

• Systemic Sclerosis- A Spectrum▫ Limited

➢ Below Elbow/knee

▫ Diffuse➢ Above Elbow/knee, trunk involved

• Antibodies▫ ANA▫ Anti-Centromere (limited)▫ Anti-Scl70 (diffuse)

Limited Cutaneous Systemic Sclerosis

…and Pulmonary

Arterial Hypertension

Diffuse Cutaneous Systemic Sclerosis

• As with limited +

• Pulmonary Fibrosis

▫ HRCT

▫ Pulmonary function tests

• Gut disease

▫ Malabsorbtion due to bacterial

overgrowth

➢ Rx broad spec Abx

▫ Constipation

• Scleroderma Renal Crisis

▫ Sudden onset Hypertension,

Oligo/anuric AKI, encephalopathy,

Cardiac Failure

▫ Vague presentation with headache

and visual disturbance

▫ ACE inhibitor

▫ Best supportive care

Sjogren’s Syndrome

• Autoimmune disease affective the exocrine glands

• Symptoms

▫ Dry ‘gritty’ eyes

▫ Dry mouth

▫ Parotid swelling

▫ Arthralgia

• Antibodies

▫ ANA

▫ Ro (SS-A)

▫ La (SS-B)

▫ RhF

• Treatment

▫ Hydroxychloraquine

▫ Pilocarpine

• Risk of lymphoma

Polymyositis/Dermatomyositis

• Autoimmune inflammatory

myopathies

▫ Proximal weakness

▫ Bulbar muscles, cardiac

involvement

▫ Lung fibrosis

• Investigations:

▫ Elevated CK

▫ EMG

▫ Muscle Biopsy

▫ Antibodies: Jo-1

• Strong association with underlying

malignancy (DM>PM)

Questions

• 21F presents sharp central chest pain. Over the last few months she reports profound fatigue, mouth ulcers and arthralgia. Over summer she noticed a rash on her hands which has now resolved.

• 41M presents profoundly short of breath. An ECHO shows severe pulmonary hypertension. He has a background of Raynaud's phenomenon.

• 77F presents with a fall. She has profound flaccid weakness, more prominent in proximal muscle groups. On examination she is found to have a few violacious macules over her knuckles and a rash in a shawl distribution. Her family are concerned she has lost weight.

SLE

SYSTEMIC SCLEROSIS (LIMITED)

DERMATOMYOSITIS

VASCULITIS

Example Case 16:

72M presents to GP with fatigue and >10kg weight loss in

2 months. He has been struggling to dress and undress due

to stiffness. In the last week he has developed a severe

headache with pain on combing his hair

Example Case 17:

37M with a background of nasal polyps and longstanding

asthma presents with tingling and numbness of his right

foot and a new foot drop. On examination you notice he

has a non-blanching rash over his lower limbs.

Example Case 18:

56F has been in hospital for 1 week with an atypical

pneumonia with fever and patchy pulmonary infiltrates,

which has not responded to multiple antibiotic agents. She

develops a rapidly progressive AKI and urine dip

demonstrates 3+ blood and 3+ protein. She mentions she

has had recurrent sinus infections and nasal crusting prior

to admission.

Polymyalgia Rheumatica and GCA

ELDERLY PATIENTS

PROXIMAL LIMB

GIRLDLE

PAIN AND STIFFNESS

ELEVATED ESR/CRP

HEADACHE, SCALP PAIN

THICKENED TA

LARGE VESSEL

VASCULITIS

AFFECTING BRANCHES

OF AORTA

RISK OF BLINDNESS

Rarer Large/Medium Vasculitides

• Takayasu’s • Large Vessel Vasculitis in >50s• “Pulseless disease”

• Kawasaki’s• Children• Febrile muco-cutaneous and lymph node illness• Coronary Arteries

• Polyarteritis nodosa (PAN)• Hep B• Cutaneous nodules• Testicular pain

ANCA- Associated Vasculitis

• Granulomatosis with Polyangiitis (GPA) • Wegner’s Granulomatosis

• c-ANCA, PR3 antibody

• ENT (saddle shaped nose), Pulmonary haemorrhage, Glomerulonephritis, neuropathy, rash

• Eosinophilic Granulomatosis with Polyangiitis (eGPA)• Churg-Strauss

• p-ANCA, MPO antibody

• Eosinophilia

• Asthma, lung infiltrates ENT, Neuropathy, GN

• Microscopic Polyangiitis• p-ANCA, MPO antibody

• Glomerulonephritis, rash, pulmonary haemorrhage

Example Cases: Answers

• 72M presents to GP with fatigue and >10kg weight loss in 2 months. He has been struggling to dress and undress due to stiffness. In the last week he has developed a severe headache with pain on combing his hair.

• 37M with a background of nasal polyps and longstanding asthma presents with tingling and numbness of his right foot and a new foot drop. On examination you notice he has a non-blanching rash over his lower limbs.

• 56F has been in hospital for 1 week with an atypical pneumonia with fever and patchy pulmonary infiltrates, which has not responded to multiple antibiotic agents. She develops a rapidly progressive AKI and urine dip demonstrates 3+ blood and 3+ protein. She mentions she has had recurrent sinus infections and nasal crusting prior to admission.

Giant Cell Arteritis

eGPA (Churg-Strauss)

GPA (Wegners)

THE REST….

FIBROMYALGIA

• Diffuse myaglgia and arthrlagia• Poor sleep and fatigue• “Brain Fog”• No evidence of joint inflammation or

damage

• Pain processing/sleep disorder• Common

• Treatment▫ Education/Reassurance▫ Graded Exercise therapy▫ CBT▫ Neuropathic Analgesia: ⚫Amitriptylline, Duloxetine,

Gabapentin

METABOLIC BONE DISEASE

OSTEOMALACIA

• Inadequate bone mineralisation ‘soft bones’

• Caused by Vitamin D deficiency

• Before fusion of bone epiphyses = Rickets

• Waddling gait

• Myalgia, arthralgia. Nocturnal pain

• Pseudofractures on XR: ‘Looser’s Zones’

• Low/N Calcium, ↑ALP, ↑ PTH

• Treatment: Replace Vitamin D

OSTEOPOROSIS

• Reduction in bone mass and density

leading to increased fracture risk

• Colles, Vertbral, NOF fractures

• Primary

▫ Post-menopausal women

• Secondary

▫ Chronic inflammatory disease,

Malabsorption, Endocrine, Steroids,

AEDs, aromatase inhibitors

• Defined as BMD ≥-2.5 SD from

mean peak bone mass (T-score)

• Z-score: age matched individuals

OSTEOPOROSIS: Treatment

• Calcium and Vitamin D Replacement• Anti-resorbtive Medications• Bisphosphonates: Alendronate, Risedronate, Zoledronate

• SE: Oesophageal erosions, GORD, osteonecrosis jaw, atypical femoral Fractures

• Denosumab: mAb against RANK ligand• HRT

• Only in women with menopausal symptoms

• Newer Stuff• Teriparatide: rPTH

PAGET’S DISEASE

• Excessive osteoclastic bone

resorbtion followed by disordered

osteoblastic activity → structurally

abnormal new bone formation

• Bone pain, deformity

• Complications: Nerve compression,

fracture, high output cardiac failure

• ↑ALP

• XR: Bony enlargement and

distortion, sclerosis and lytic areas

• NM-Bone

• Bisphosphonates

QUESTIONS?

THANK YOU