MSK/Rheum Flashcards
Paget’s disease aetiology
excessive bone turnover
patchy areas of high density (sclerosis) and low density (lysis)
pathological fractures in axial skeleton
Paget’s disease presentation
Bone pain
Bone deformity
Fractures
Hearing loss can occur if it affects the bones of the ear
Paget’s disease ivx
Xray Findings
Bone enlargement and deformity
“Osteoporosis circumscripta” describes well defined osteolytic lesions that appear less dense compared with normal bone
“Cotton wool appearance” of the skull - increased density (sclerosis) and decreased density (lysis)
“V-shaped defects” in the long bones are V shaped osteolytic bone lesions
Biochemistry
Raised alkaline phosphatase (and other LFTs are normal)
Normal calcium
Normal phosphate
Paget’s management
Bis
NSAIDs for bone pain
Calcium and vitamin D supplementation, particularly whilst on bisphosphonates
Surgery is rarely required for fractures, severe deformity or arthritis
osteomalacia ivx
Serum 25-hydroxyvitamin D
osteomalacia tx
Colecalciferol -one of:
50,000 IU once weekly for 6 weeks
20,000 IU twice weekly for 7 weeks
4000 IU daily for 10 weeks
When to use FRAX score
Women aged > 65
Men > 75
Younger patients with risk factors such as a previous fragility fracture, history of falls, low BMI, long term steroids, endocrine disorders and rheumatoid arthritis.
frax outcomes
Low risk – reassure
Intermediate risk – offer DEXA scan and recalculate the risk with the results
High risk – offer treatment
Bis SE
Reflux and oesophageal erosions. Oral bisphosphonates are taken on an empty stomach sitting upright for 30 minutes before moving or eating to prevent this.
Atypical fractures (e.g. atypical femoral fractures)
Osteonecrosis of the jaw
Osteonecrosis of the external auditory canal
pseudogout aetiology
crystal arthropathy caused by calcium pyrophosphate crystals
pseudogout aspiration findings
No bacterial growth
Calcium pyrophosphate crystals
Rhomboid shaped crystals
Positive birefringent of polarised light
Pseudogout x-ray finding
Chondrocalcinosis
Osteoarthritis/pseudogout x-ray findings
L – Loss of joint space
O – Osteophytes
S – Subarticular sclerosis
S – Subchondral cysts
Pseudogout tx
NSAIDs Colchicine Joint aspiration Steroid injections Oral steroids Joint washout (arthrocentesis) is an option in severe cases.
Gout tophi areas
DIPJ Helix of ear Extensor surface of elbow Base of the big toe (metatarsophalangeal joint) Wrists Base of thumb (carpometacarpal joints)
Gout aspiration findings
No bacterial growth
Needle shaped crystals
Negatively birefringent of polarised light
Monosodium urate crystals
Gout x-ray findings
Typically the space between the joint is maintained
Lytic lesions in the bone
Punched out erosions
Erosions can have sclerotic borders with overhanging edges
Acute flare gout management
During the acute flare:
NSAIDs (e.g. ibuprofen) are first-line
Colchicine second-line
Steroids can be considered third-line
gout prophylaxis
Allopurinol is a xanthine oxidase inhibitor
Behcet’s disease gene
HLA B51 gene
Behçet’s Disease aetiology
recurrent oral and genital ulcers
Behçet’s disease test
pathergy test involves using a sterile needle to create a subcutaneous abrasion on the forearm. This is then reviewed 24 – 48 hours later to look for a weal 5mm or more in size. It tests for non-specific hypersensitive in the skin. It is positive in Behçet’s disease, Sweet’s syndrome and pyoderma gangrenosum.
Behçet’s disease mx
Topical steroids to mouth ulcers (e.g. soluble betamethasone tablets)
Systemic steroids (i.e. oral prednisolone)
Colchicine is usually effective as an anti-inflammatory to treat symptoms
Topical anaesthetics for genital ulcers (e.g. lidocaine ointment)
Immunosuppressants such as azathioprine
Biologic therapy such as infliximab
Types of Vasculitis Affecting The Small Vessels
Henoch-Schonlein purpura
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
Types of Vasculitis Affecting The Medium Sized Vessels
Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease
Types of Vasculitis Affecting The Large Vessels
Giant cell arteritis
Takayasu’s arteritis
Vasculitis Presentation
There are some generic features that apply to most types of vasculitis. These are things that should make you think about a possible vasculitis:
Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.
Joint and muscle pain
Peripheral neuropathy
Renal impairment
Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)
Anterior uveitis and scleritis
Hypertension
They are also associated with systemic manifestations of:
Fatigue Fever Weight loss Anorexia (loss of appetite) Anaemia
vasculitis ivx
Inflammatory markers (CRP and ESR) are usually raised in vasculitis.
Anti neutrophil cytoplasmic antibodies (ANCA)
ANCA blood test specific results
p-ANCA (MPO antibodies): Microscopic polyangiitis and Churg-Strauss syndrome
c-ANCA (PR3 antibodies): Wegener’s granulomatosis
vasculitis mx
Steroids can be administered to target the affected area:
Oral (i.e. prednisolone)
Intravenous (i.e. hydrocortisone)
Nasal sprays for nasal symptoms
Inhaled for lung involves (e.g. Churg-Strauss syndrome)
Immunosuppressants that are used include:
Cyclophosphamide
Methotrexate
Azathioprine
Rituximab and other monoclonal antibodies
Henoch-Schonlein purpura aetiology
IgA vasculitis - often triggered by URTI or gastroenteritis
purpuric rash affecting the lower limbs or buttocks in children
Deposits affect skin, kidneys and gastro-intestinal tract
Henoch-Schonlein purpura classic features
purpura (100%), joint pain (75%), abdominal pain (50%) and renal involvement (50%).
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) aetiology
severe asthma in late teenage years or adulthood. A characteristic finding is elevated eosinophil levels
lung and skin problem, occasionally kidneys
Microscopic polyangiitis presentation
Renal failure, also SOB and haemoptosis
Granulomatosis with polyangiitis (Wegener’s granulomatosis) aetiology
small vessel vasculitis, affects respiratory tract and kidneys
Granulomatosis with polyangiitis (Wegener’s granulomatosis) presentation
Epistaxis, crusty nasal secretions
hearing loss and sinusitis
Saddle shaped nose due to perforated septum
Wheeze, cough, haemoptysis - can be misdiagnosed as pneumonia on CXR
can lead to rapidly progressing glomerulonephritis
Polyarteritis Nodosa aetiology
medium vessel vasculitis. It is most associated with hepatitis B
Polyarteritis Nodosa presentation
livedo reticularis. This is a mottled, purplish, lace like rash.
renal, GI, stroke, MI etc
Kawasaki Disease presentation
Persistent high fever > 5 days
Erythematous rash
Bilateral conjunctivitis
Erythema and desquamation (skin peeling) of palms and soles
“Strawberry tongue” (red tongue with prominent papillae)
usually under 5 years of age
Kawasaki Disease comp
coronary artery aneurysms.
Kawasaki Disease tx
aspirin and IV immunoglobulins.
Takayasu’s arteritis
affects aorta (pulseless disease)
CT or MRI angiography
Doppler of carotids
b-type symptoms and arm claudication
Sjogren’s Syndrome aetiology
autoimmune condition that affects the exocrine glands. It leads to the symptoms of dry mucous membranes
Sjogren’s Syndrome classification
Primary Sjogren’s is where the condition occurs in isolation.
Secondary Sjogren’s is where it occurs related to SLE or rheumatoid arthritis.
It is associated with anti-Ro and anti-La antibodies.
Sjogren’s Syndrome presentation
dry mucous membranes, such as dry mouth, dry eyes and dry vagina.
Sjogren’s Syndrome mx
Artificial tears
Artificial saliva
Vaginal lubricants
Hydroxychloroquine is used to halt the progression of the disease.
Sjogren’s Syndrome comp
Eye infections such as conjunctivitis and corneal ulcers
Oral problems such as dental cavities and candida infections
Vaginal problems such as candidiasis and sexual dysfunction
Sjogren’s Syndrome test
Schirmer test involves inserting a folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid. This is left in for 5 minutes and the distance along the strip hanging out that becomes moist is measured. The tears should travel 15mm in a healthy young adult. A result of less than 10mm is significant.
Antiphospholipid Syndrome aetiology
antiphospholipid antibodies where the blood becomes prone to clotting. The patient is in a hyper-coagulable state. The main associations are with thrombosis and complications in pregnancy, particularly recurrent miscarriage.
antiphospholipid antibodies:
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
Antiphospholipid Syndrome comp
Venous thromboembolism
Arterial thrombosis
Pregnancy complications
Antiphospholipid Syndrome signs
Livedo reticularis
Libmann-Sacks endocarditis
Thrombocytopenia
Antiphospholipid Syndrome dx
History of thrombosis or pregnancy complication plus:
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
Antiphospholipid Syndrome mx
Warfarin or LMWH plus aspirin in pregnancy
Dermatomyositis autoamtibodies
Anti-Jo-1 antibodies: polymyositis (but often present in dermatomyositis)
Anti-Mi-2 antibodies: dermatomyositis.
Anti-nuclear antibodies: dermatomyositis.
Dermatomyositis Skin Features
Gottron lesions (scaly erythematous patches) on the knuckles, elbows and knees
Photosensitive erythematous rash on the back, shoulders and neck
Purple rash on the face and eyelids
Periorbital oedema (swelling around the eyes)
Subcutaneous calcinosis (calcium deposits in the subcutaneous tissue)
Dermatomyositis
Corticosteroids are the first line treatment of both conditions.
Other medical options where the response to steroids is inadequate:
Immunosuppressants (such as azathioprine)
IV immunoglobulins Biological therapy (such as infliximab or etanercept)
GCA presentation
Severe unilateral headache typically around temple and forehead
Scalp tenderness my be noticed when brushing hair
Jaw claudication
Blurred or double vision
Irreversible painless complete sight loss can occur rapidly
GCA dx
Clinical presentation
Raised ESR: usually 50 mm/hour or more
Temporal artery biopsy findings - Multinucleated giant cells
GCA mx
pred
Aspirin 75mg daily decreases visual loss and strokes
Proton pump inhibitor (e.g. omeprazole) for gastric prevention while on steroids
Polymyalgia Rheumatica demographics
ass GCA
It usually affects old adults (above 50 years)
More common in women
More common in caucasians
Polymyalgia Rheumatica signs
Bilateral shoulder pain that may radiate to the elbow
Bilateral pelvic girdle pain
Worse with movement
Interferes with sleep
Stiffness for at least 45
minutes in the morning
Other features:
Systemic symptoms such as weight loss, fatigue, low grade fever and low mood
Upper arm tenderness
Carpel tunnel syndrome
Pitting oedema
PMR ivx
Full blood count
Urea and electrolytes
Liver function tests
Calcium can be raised in hyperparathyroidism or cancer or low in osteomalacia
Serum protein electrophoresis for myeloma and other protein disorders
Thyroid stimulating hormone for thyroid function
Creatine kinase for myositis
Rheumatoid factor for rheumatoid arthritis
Urine dipstick
PMR special tests
Anti-nuclear antibodies (ANA) for systemic lupus erythematosus
Anti-cyclic citrullinated peptide (anti-CCP) for rheumatoid arthritis
Urine Bence Jones protein for myeloma
Chest xray for lung and mediastinal abnormalities
PMR mx
15mg of prednisolone
long term Steroid important factors
Don’t STOP
DON’T – Make them aware that they will become steroid dependent after 3 weeks of treatment and should not stop taking the steroids due to the risk of adrenal crisis if steroids are abruptly withdrawn
S – Sick Day Rules: Discuss increasing the steroid dose if they become unwell (“sick day rules”)
T – Treatment Card: Provide a steroid treatment card to alert others that they are steroid dependent in case they become unresponsive
O – Osteoporosis prevention: Consider osteoporosis prophylaxis whilst on steroids with bisphosphonates and calcium and vitamin D supplements
P – Proton pump inhibitor: Consider gastric protection with a proton pump inhibitor (e.g. omeprazole)
Systemic Sclerosis aetiology
autoimmune inflammatory and fibrotic connective tissue disease.
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis
C – Calcinosis R – Raynaud’s phenomenon E – oEsophageal dysmotility S – Sclerodactyly T – Telangiectasia
Diffuse Cutaneous Systemic Sclerosis
Cardiovascular problems, particularly hypertension and coronary artery disease.
Lung problems, particularly pulmonary hypertension and pulmonary fibrosis.
Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.
PLUS CREST
Systemic Sclerosis antibodies
ANA
Anti-centromere antibodies are most associated with limited cutaneous systemic sclerosis.
Anti-Scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis.
Systemic Sclerosis medical mx
Steroids and immunosuppressants are usually started with diffuse disease and complications such as pulmonary fibrosis
Nifedipine can be used to treat symptoms of Raynaud’s phenomenon
Anti acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
Analgesia for joint pain
Antibiotics for skin infections
Antihypertensives
Systemic Sclerosis non- medical mx
Avoid smoking
Gentle skin stretching to maintain the range of motion
Regular emollients
Avoiding cold triggers for Raynaud’s
Physiotherapy to maintain healthy joints
Occupational therapy for adaptations to daily living to cope with limitations
Discoid Lupus Erythematosus presentation
women aged 20-40
SLE ass.
Photosensitive
Scarring alopecia and hyper/hypo-pigmented scars
Discoid Lupus Erythematosus lesions
Inflamed Dry Erythematous Patchy Crusty and scaling
Discoid Lupus Erythematosus mx
Sun protection
Topical steroids
Intralesional steroid injections
Hydroxychloroquine
SLE presentation
Fatigue Weight loss Arthralgia (joint pain) and non-erosive arthritis Myalgia (muscle pain) Fever Photosensitive malar rash. This is a “butterfly” shaped rash across the nose and cheek bones that gets worse with sunlight. Lymphadenopathy and splenomegaly Shortness of breath Pleuritic chest pain Mouth ulcers Hair loss Raynaud’s phenomenon
SLE ivx
Autoantibodies (see below)
Full blood count (normocytic anaemia of chronic disease)
C3 and C4 levels (decreased in active disease)
CRP and ESR (raised with active inflammation)
Immunoglobulins (raised due to activation of B cells with inflammation)
Urinalysis and urine protein:creatinine ratio for proteinuria in lupus nephritis
Renal biopsy can be used to investigate for lupus nephritis
SLE dx
SLICC Criteria or the ACR Criteria
SLE tx
First line treatments are:
NSAIDs
Steroids (prednisolone)
Hydroxychloroquine (first line for mild SLE)
Suncream and sun avoidance for the photosensitive the malar rash
Other commonly used immunosuppressants in resistant or more severe lupus:
Methotrexate Mycophenolate mofetil Azathioprine Tacrolimus Leflunomide Ciclosporin
Rituximab is a monoclonal antibody that targets the CD20 protein on the surface of B cells
Belimumab is a monoclonal antibody that targets B-cell activating factor
Ankylosing Spondylitis aetiology
seronegative spondyloarthropathy group of conditions relating to the HLA B27 gene
Spine fusion - “bamboo spine”
Ankylosing Spondylitis associations
Systemic symptoms such as weight loss and fatigue
Chest pain related to costovertebral and costosternal joints
Enthesitis is inflammation of the entheses. This is where tendons or ligaments insert in to bone. This can cause problems such as plantar fasciitis and achilles tendonitis.
Dactylitis is inflammation in a finger or toe.
Anaemia
Anterior uveitis
Aortitis is inflammation of the aorta
Heart block can be caused by fibrosis of the heart’s conductive system
Restrictive lung disease can be caused by restricted chest wall movement
Pulmonary fibrosis at the upper lobes of the lungs occurs in around 1% of AS patients
Inflammatory bowel disease is a condition associated with AS
Ankylosing spondylitis tests
Inflammatory markers (CRP and ESR) may rise with disease activity
HLA B27 genetic test
Xray of the spine and sacrum
MRI of the spine can show bone marrow oedema early in the disease before there are any xray changes
Ankylosing spondylitis xray signs
Squaring of the vertebral bodies
Subchondral sclerosis and erosions
Syndesmophytes are areas of bone growth where the ligaments insert into the bone. They occur related to the ligaments supporting the intervertebral joints.
Ossification of the ligaments, discs and joints. This is where these structures turn to bone.
Fusion of the facet, sacroiliac and costovertebral joints
Ankylosing spondylitis mx
NSAIDs can be used to help with for pain.
Steroids can be use during flares to control symptoms. This could oral, intramuscular slow release injections or joint injections.
Anti-TNF medications such as etanercept or a monoclonal antibody against TNF such as infliximab
Secukinumab is a monoclonal antibody against interleukin-17. It is recommended by NICE if the response to NSAIDS and TNF inhibitors is inadequate.
Reactive arthritis aetiology
Synovitis following recent infection
aka Reiter syndrome
Acute mono-arthritis
gastroenteritis or sexually transmitted infection. Chlamydia is most common
HLA B27 gene - seronegative spondylarthropathy
Reactive arthritis associations
Bilateral conjunctivitis (non-infective)
Anterior uveitis
Circinate balanitis is dermatitis of the head of the penis
“can’t see, pee or climb a tree”.
Reactive arthritis mx
Aspirate - gram stain, culture etc - crystal inv.
treat with NSAIDS, steroid injections
Psoriatic Arthritis aetiology
seronegative spondyloarthropathy
Psoriatic Arthritis signs
Plaques of psoriasis on the skin Pitting of the nails Onycholysis Dactylitis Enthesitis
inflammatory arthritis
Psoriatic Arthritis screening
Psoriasis Epidemiological Screening Tool (PEST)
Psoriatic Arthritis xray
Periostitis is inflammation of the periosteum causing a thickened and irregular outline of the bone
Ankylosis is where bones joining together causing joint stiffening
Osteolysis is destruction of bone
Dactylitis is inflammation of the whole digit and appears on the xray as soft tissue swelling
Pencil-in-cup appearance
Psoriatic Arthritis mx
NSAIDs for pain
DMARDS (methotrexate, leflunomide or sulfasalazine)
Anti-TNF medications (etanercept, infliximab or adalimumab)
Ustekinumab is last line (after anti-TNF medications) and is a monoclonal antibody that targets interleukin 12 and 23
Psoriatic arthritis most severe sign
Osteolitis of phalanxes/joints - progressive shortening of digit - skin folds over digit - telescopic finger
RF genes
HLA DR4 (a gene often present in RF positive patients)
HLA DR1 (a gene occasionally present in RA patients)
RF autoantibodies
anti-CCP
RF
Palindromic Rheumatism
This involves self limiting short episodes of inflammatory arthritis with joint pain, stiffness and swelling typically affecting only a few joints. The episodes only last 1-2 days
RA joints
Proximal Interphalangeal Joints (PIP) joints
Metacarpophalangeal (MCP) joints
Wrist and ankle
Metatarsophalangeal joints
Cervical spine
Large joints can also be affected such as the knee, hips and shoulders
RA hand signs
Z shaped deformity to the thumb
Swan neck deformity (hyperextended PIP with flexed DIP)
Boutonnieres deformity (hyperextended DIP with flexed PIP)
Ulnar deviation of the fingers at the knuckle (MCP joints)
RA Extra-articular Manifestations
Pulmonary fibrosis with pulmonary nodules
(Caplan’s syndrome)
Bronchiolitis obliterans
(inflammation causing small airway destruction)
Felty’s syndrome (RA, neutropenia and splenomegaly)
Secondary Sjogren’s
Syndrome (AKA sicca syndrome)
RA xray signs
Joint destruction and deformity
Soft tissue swelling
Periarticular osteopenia
Boney erosions
RA scoring
The joints that are involved (more and smaller joints score higher)
Serology (rheumatoid factor and anti-CCP)
Inflammatory markers (ESR and CRP)
Duration of symptoms (more or less than 6 weeks)
RA mx
NSAIDS PPI Methotrexate etc 2 second line Plus biologic 3rd line
Common biologics
Anti-TNF (adalimumab, infliximab, etanercept, golimumab and certolizumab pegol)
Anti-CD20 (rituximab)
Anti-IL6 (sarilumab)
Anti-IL6 receptor (tocilizumab)
JAK inhibitors (tofacitinib and baricitinib)
Methotrexate prescribing consideration
Folic acid 5mg, on a different day to methotrexate
Unique DMARD side-effects
Methotrexate: pulmonary fibrosis
Leflunomide: Hypertension and peripheral neuropathy
Sulfasalazine: Male infertility (reduces sperm count)
Hydroxychloroquine: Nightmares and reduced visual acuity
Anti-TNF medications: Reactivation of TB or hepatitis B
Rituximab: Night sweats and thrombocytopenia
OA hand signs
Heberden’s nodes (in the DIP joints) Bouchard’s nodes (in the PIP joints) Squaring at the base of the thumb at the carpo-metacarpal joint Weak grip Reduced range of motion
OA management
Physio/lifestyle/education
Paracetamol
NSAIDS plus PPI
Opiates
joint injections
Felty’s syndrome
rheumatoid arthritis, enlargement of the spleen and low neutrophil count
types of psoriasis
rheumatoid arthritis, enlargement of the spleen and low neutrophil count
Telescope finger aka
Arthritis mutilans