RHEUMATOLOGY

important management considerations when starting on steroids

- Give patients a steroid card

- Advise never to stop steroids suddenly due to the risk of adrenal insufficiency

- If they are unable to take tablets (e.g. due to vomiting) they need to seek urgent medical advice

- Explain the risk of immunosuppression and check vaccination status

- bone protection with bisphosphonates +/- vitamin D and calcium supplementation; this should be continued for the duration of steroid treatment before reassessing fragility - fracture risk (osteoporosis)

- proton pump inhibitor for gastric protection

- Monitor blood pressure and glucose levels

when to avoid NSAIDs

Peptic ulcer disease / GI bleeding

Renal impairment (CKD, AKI)

Cardiovascular disease (IHD, HF, HTN)

what is the Beighton scale

evaluates joint hyper mobility

go through the whole antibody list

1. ANA (unspecific) - many conditions

2. anti-dsDNA - specific for lupus

3. ENA

Ro -

La - for sjogren

Smooth muscle - specific for lupus

RNP

Jo-1 = for myositis

Scl 70 = for limited systemic sclerosis

4. ANCA

5. RF

6. CCP - more specific for RA

ANTI-PHOSPHOLIPID SYNDROME definition

- you have anti-phospholipid antibodies

ANTI-PHOSPHOLIPID SYNDROME investigations

- anticardiolipin antibodies (aCL),

- anti-beta-2-glycoprotein I antibodies (anti-β2GPI),

- lupus anticoagulant (LA).

ANTI-PHOSPHOLIPID SYNDROME management

AYSMPTOMATIC: observe

SYMPTOMATIC 9i.e have had a thrombotic event) = thromboprophylaxis

- WARFARIN

(if warfarin contraindicated, low dose aspiring and low molecular weight heparin)

ANTI-PHOSPHOLIPID SYNDROME features

- venous/arterial thromboses BUT causes a paradoxical rise in APTT (APTT test relies on phospholipid)

- recurrent miscarriages

- livedo reticularis

- other features: pre-eclampsia, pulmonary hypertension

CLOTS

coagulation

livedo reticularis

obstetric complications

thrombocytopenia

ANKYLOSING SPONDYLITIS definition

a type of seronegative arthritis

ANKYLOSING SPONDYLITIS most commonly affected demographic

males aged 20-30

ANKYLOSING SPONDYLITIS common features

- back pain and stiffness (sacroileitis which is buttock pain as well)

- relieved with movement

extraarticular involement (rare)

- Apical fibrosis

- Anterior uveitis

- Aortic regurgitation

- Achilles tendonitis

- AV node block

- Amyloidosis

- Anterior uveitis -( this is common, so warn patients about this)

- Inflammatory bowel disease

- Osteoporosis

- IgA nephropathy

- Systemic symptoms of weight loss and fatigue

O/E

- restricted movement in lumbar spine - "schober's test" can be used to assess

ANKYLOSING SPONDYLITIS investigations

- best diagnostic = MRI

- X ray

- bloods

- no need to do hla-b27

ANKYLOSING SPONDYLITIS features on X-ray

- Bamboo spine with a single central radiodense line related to ossification of supraspinous and interspinous ligaments (shown in picture)

- fused sacroiliac joint

- Squaring of vertebrae

- syndesmophytes (which go above and below the bone)

- shiny corner sign (triangular areas of sclerosis at the anterior vertebral endplates on X-ray.)

ANKYLOSING SPONDYLITIS management

conservative

- physiotherapy

medical

- first line: NSAIDs

- second line: biologic DMARDs e.g infliximab

(do not use methotrexate, as useless for backpain)

ANKYLOSING SPONDYLITIS examination findings

- reduced chest expansion,

- reduced lateral flexion

- reduced forward flexion (Schober's test)

BEHCET'S DISEASE features

Relapsing-remitting oral and genital ulceration

as well as

arthritis

uveitis

mainly affects people from the Middle East, the mediterranean or east asia

DISORDERS OF ELBOW - medial epicondylitis definition

- aka golfers elbow

- pain worse on wrist flexion and pronation of the forearm

remember golfing and also f and p look alike

DISORDERS OF ELBOW - lateral epicondylitis features

- aka tennis elbow

- pain worse on wrist extension and supination of the forearm

DISORDERS OF ELBOW - lateral epicondylitis management options

- advice on avoiding muscle overload

- simple analgesia

- steroid injection

- physiotherapy

FIBROMYALGIA features

- widespread pain

- unrefreshing sleep

- chronic

FIBROMYALGIA management

- CBT

- aerobic exercise - graded exercise therapy

- medication for neuropathic pain

GOUT what is it

- Gout is a type of arthritis caused by the accumulation of monosodium urate crystals in and around the joints

- most commonly first presents in MTP - metatarsophalangeal joints

- chronic untreated gout can lead to the formation of tophi (hard nodules)

commonly in :

ankle, wrist, knee, 1st MTP

(gout =

GOUT risk factors

- CKD (can cause high urate)

- alcohol excess

- red meat

- fizzy drinks

- Medications such as thiazide diuretics and ciclosporin

- also tumour lysis syndrome

GOUT investigations

- mainly a clinical diagnosis

bedside:

- joint needle aspiration (synovial fluid analysis) - shows needle-shaped monosodium urate crystals with negative birefringence

- tophi can be biopsied

blood test:

- serum uric acid (if more than 360 micro mol/L, this is diagnostic)

GOUT management

ACUTE

- NSAIDs, colchicine. If both fail: short course of oral steroids.

TO REDUCE FUTURE RISK

- Urate-lowering therapy with allopurinol - first line (must wait at least 2 weeks after an attack)** or febuxostat (these are xanthine oxidase inhibitors - so reduce uric acid production

** When starting allopurinol, cover with nsaids or colchicine as allopurinol can actually cause an acute flare

** side effect of allopurinol can be a rash in which case the medication should be stopped.

HYPERMOBILITY SYNDROME

MARFAN'S SYNDROME aetiology

is a genetic disorder (autosomal dominant) caused by mutations in the FBN1 gene, which encodes fibrillin-1

MARFAN'S SYNDROME features

- usually tall and slim

- long arms and digits

- a high-arched palate

- hypermobility

- cardiovascular abnormalities

ophthalmic:

- enopthalmos

- myopia

- superior lens disclocation

OSTEOARTHRITIS definition

- most common form of arthritis

- causes degeneration of joints due to "wear and tear"

OSTEOARTHRITIS risk factors

Overuse of joints

Excessive body weight

Age

OSTEOARTHRITIS symptoms

P – Pain

C – Crepitus

T – Tenderness

R – Relieved by rest (worse with activity)

G – Grating

S – Swelling

please can the radiologist go somewhere!

Bouchard’s nodes = swelling at the PIP joints

and

Heberden’s nodes = swelling at the DIP joints

(B is proximal and H is distal)

OSTEOARTHRITIS diagnosis

- clinical diagnosis

- if uncertainty, do an X-ray, which shows:

Loss of joint space

Osteophytes

Subchondral sclerosis - white opacification

Subchondral cysts - black things within the white

can also be used to rule out other causes of symptoms

OSTEOARTHRITIS management

conservative

- lifestyle factors: diet and exercise (exercise does help!), stopping smoking

- physiotherapy, occupational therapy

medical

- first line: topical NSAIDs e.g ibuprofen gel

- work up WHO pain ladder

surgical

- arthroplasty if medical options have been exhausted and disease is severe

OSTEOARTHRITIS complications

can form a bakers cyst

OSTEOMALACIA what is it

Metabolic condition where bones become soft secondary to a calcium, vitamin D or phosphate deficiency

OSTEOMALACIA what would the lab values be

Low serum calcium, low serum phosphate, raised ALP and raised PTH

PAGET'S DISEASE OF THE BONE classical presentation

old man, bone pain, raised ALP

PAGET'S DISEASE OF THE BONE investigations

1. raised alkaline phosphatase (ALP)

2. calcium and phosphate are typically normal

3. other markers of bone turnover

4. x-rays

5. bone scintigraphy - increased uptake is seen focally at the sites of active bone lesions

PAGET'S DISEASE OF THE BONE management

bisphosphonate (either oral risedronate or IV zoledronate)

PAGET'S DISEASE OF THE BONE complications

hearing loss - due to cranial nerve entrapment

fragility fractures

heart failure - because bones need more blood?

sarcoma

POLYMYALGIA RHEUMATICA definition

stiffness and pain of the proximal large joints.

- autoimmune

- acute onset

- common in the elderly

POLYMYALGIA RHEUMATICA investigations

ESR and CRP

- no particular antibody

- creatine kinase is normal

POLYMYALGIA RHEUMATICA features

- bilateral stiffness of shoulder, neck, pelvic joints

- movement worsens pain (better with rest)

- systemic systems e.g fatigue

- O/E power is usually preserved

- should screen for GCA

POLYMYALGIA RHEUMATICA treatment

steroids

15mg oral prednisolone daily

( if not responding well to Pred, consider an alternative diagnosis)

POLYMYOSITIS what is it

inflammatory disorder causing symmetrical, proximal muscle weakness

dermatomyositis is a variant of the disease where skin manifestations are prominent - eg a light purple (heliotrope) rash on the cheeks and eyelids.

Gotrans sign as well - which is rash on knuckles

- associated with malignancy, so should do a CT CAP

- can result in type 2 resp failure!

- anti -Jo antibodies

POLYMYOSITIS commonly affected demographic

typically affects middle-aged, female:male 3:1

POLYMYOSITIS classical presentation

malignancy + raised CK

"Dermatomyositis and polymyositis turn your muscles into CLAAA (clay)':

enzymes elevated are :

Creatine kinase,

Lactate dehydrogenase,

Aldolase,

ALT

AST"

PSEUDOGOUT definition

a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium

awkward news

- knee wrist shoulder

PSEUDOGOUT risk factors

haemochromatosis (iron drives pyrophosphate overproduction)

hyperparathyroidism

low magnesium, low phosphate

acromegaly,

Wilson's disease

PSEUDOGOUT investigations

1. joint aspiration: weakly-positively birefringent rhomboid-shaped crystals

2. x-ray: chondrocalcinosis* in the knee this can be seen as linear calcifications of the meniscus and articular cartilage

* this is excessive accumulation of calcium crystals in the cartilage of joint

PSEUDOGOUT management

RICE

Intraarticular steroids, NSAIDs

PSORIATIC ARTHRITIS definition

inflammatory arthritis associated with psoriasis and is classed as one of the seronegative arthritis

target is the enthuses (where ligaments and tendons attach to the bone)

PSORIATIC ARTHRITIS subtypes

1. Asymmetric oligoarticular arthritis

- 4 or fewer joints are involved;

- most common form at presentation although this may become polyarticular with time

2. Polyarticular arthritis

- is usually symmetrical and presents similarly to rheumatoid arthritis, although distal interphalangeal joints are more likely to be involved than metacarpophalangeal joints

3. Distal interphalangeal disease

- affects the distal interphalangeal joints only; nail changes are especially common

4. Arthritis mutilans

- is a severely destructive arthritis with resulting deformities including telescoping of digits

5. Spondyloarthritis involves sacroiliitis and involvement of the spine, often with concurrent peripheral joint involvement

PSORIATIC ARTHRITIS features

X-ray

- unusual combination of erosive changes (resorption) and new bone formation

- "pencil in cup" deformity

- arthritis mutilans (looks really deformed)

- dactylitis = self limiting episode of a swollen digit

- rash on extensor surfaces

'Rheumatoid'-like joint problems but nail changes → ?psoriatic arthritis

PSORIATIC ARTHRITIS management

lifestyle + NSAIDs → DMARDs (MTX) → biologics

RAYNAUD'S PHENOMENON management

conservative

- wear gloves

medical

CCB e.g nifedipine

REACTIVE ARTHRITIS definition

- typically affects the lower extremity joints (most commonly the knee)

- occurs classically with conjunctivitis and urethritis following an enteric or sexually transmitted infection

- "cant see, cant pee, cant climb a tree"

(uveitis, urethritis, arthritis)

REACTIVE ARTHRITIS what organism is associated

chlamydia (so you get post STI arthritis)

RHEUMATOID ARTHRITIS features

- affects MCP and PIP joints. distal joints are spared

- affects small joints (e.g feet and hands)

- relieved with movement (you get morning stiffness- abnormal is more than 30 mins)

- sudden onset

- symmetrical

- many joints affected (polyarthritis)

- additive (more joints keep getting affected)

- constitutional: fatigue, malaise

a systemic condition

- episcleritis/scleritis

- lung: ILD (interstitial lung disease), bronchiectasis

- rheumatoid nodules

- heart: pericarditis, cardiac effusion (at an increased risk of ischaemic heart disease)

- small vessel vasculitis

in the hand:

- ulnar deviation

- Z thumb

- swan neck deformity

- boutonniere's deformity

RHEUMATOID ARTHRITIS investigations

1. FBC, CRP, ESR, U/E, LFT, C3/C4 (to rule out differentials)

2. antibody screen:

- Rheumatoid Factor (an antibody against Fc potion of human IgG) - do this test initially. If you still suspect RA, do:

- anti-CCP (more specific)

(if both positive = seropositive - it is a sign of worse prognosis

70-80% are seropositive)

Also do:

- ANA

3. Imaging:

- X-ray of hands and feet: shows erosive changes

- US scans

- MRI

RHEUMATOID ARTHRITIS features on an x ray

LESSS:

loss of joint space,

periarticular erosions,

subluxation,

soft tissue swelling,

subarticular (periarticular) osteopenia (darkening of bones) - check pic

RHEUMATOID ARTHRITIS score to measure severity

DAS28 test

RHEUMATOID ARTHRITIS treatment

goal of treatment: reduce inflammation and pain, promote joint function, and prevent joint destruction and deformity

acute:

includes NSAIDs to reduce inflammation and pain.

If severe, corticosteroids - oral pred or IM methyl pred

-long term:

first line cDMARDS e.g methotrexate. Taken once a week. side effects: hair thinning, nausea. Can cause bone marrow suppression

Other 6 days: folic acid supplementation.

second line: increase dose

third line: add another cDMARD e.g sulfasalazine** or hydroxychloroquine, lefunomide (also teratogenic)

fourth line - need to have failed previous 2 treatments to be prescribed (because of cost)

biologics e.g

- infliximab - antiTNF-alpha

- rituximab (only if seropositive RA) = anti-cd20, stop production of B cells (therefore antibodies). Side effect = immunosuppression. 2 infusions 2 weeks apart. Can increase risk of severe covid.

- JAK inhibitor (tablet)

physiotherapy and hand therapy

sulfasalazine

- give this to women of childbearing age

- careful if also allergic to aspirin

- can cause oligospermia (resolves on stopping the drug)

- Stevens-Johnsons syndrome

- may colour tears , so you get stained contact lenses

what do you need to screen for before starting biologic therapy

biologic therapy can reactivate TB especially, anti-TNFa

RHEUMATOID ARTHRITIS complications

Osteoporosis

Rheumatoid nodules

Dry eyes and mouth

Infections

Carpel tunnel syndrome

Hardened and blocked arteries - CVD

Inflammation and scarring of the lung tissue

Non- hodgkin Lymphoma

Secondary amyloid

Felty's syndrome (triad: neutropenia, RA, splenomegaly)

methotrexate:

- advice for pregnancy

- significant side effects

- Methotrexate should be avoided during pregnancy due to the risk of teratogenicity. A washout period of 6 months is recommended preconception

- hepatotoxicity, bone marrow suppression and pneumonitis

SEPTIC ARTHRITIS definition

infection of the joint

SEPTIC ARTHRITIS aetiology

most commonly: staph aureus

young adults who are sexually active, Neisseria gonorrhoeae is the most common organism

IV drug users: pseudomonas aeruginosa

SEPTIC ARTHRITIS features

- Acute onset of tender, swollen joint

- Reduced range of joint movement

- Systemic symptoms such as fever, malaise, or chills

SEPTIC ARTHRITIS investigations

1. joint aspiration for MC&S - shows neutrophils >90% and very high WCC

2. bloods

3. blood culture

SEPTIC ARTHRITIS management

- IV antibiotics guided by local guidelines

- joint washout under general anaesthesia to remove infected material

- Physiotherapy following the resolution of acute infection to restore joint function

- sepsis 6

SEPTIC ARTHRITIS complications

1. Osteomyelitis: infection of the bone

2. Chronic arthritis: persistent joint inflammation

3. Ankylosis: joint fusion resulting in immobility

SJOGREN'S SYNDROME definition

an autoimmune condition where exocrine glands producing tears and saliva are destroyed

SJOGREN'S SYNDROME investigations

1. RF - rheumatoid factor positive in nearly 50% of patients

2. ANA positive in 70%

3. anti-Ro (SSA) antibodies in 70% of patients with PSS

4. anti-La (SSB) antibodies in 30% of patients with PSS

5. Schirmer's test: filter paper near conjunctival sac to measure tear formation

6. histology: focal lymphocytic infiltration

7. Anti-dsDNA suggests Sjogren's syndrome is secondary to SLE if strongly positive

8. Anti-CCP may suggest associated rheumatoid arthritis

SJOGREN'S SYNDROME symptoms

- dry eyes, perhaps more susceptible to eye infections

- dry mouth, - poor swallowing, poor dental health - increases risk of decay (caries)

- dry skin

- vaginal dryness

- raynauds phenomenon

- systemic symptoms - fatigue, arthralgia, arthritis

SJOGREN'S SYNDROME complications

- complication of pregnancy: risk of congenital heart block and neonatal lupus

- increased risk of B cell lymphoma, most commonly MALT lymphoma

SJOGREN'S SYNDROME treatment

conservative

- humidifying home and work environments

- reducing screen time

medical

- oral pilocarpine to treat eye and mouth dryness

- lubricating eye drops

SYSTEMIC LUPUS ERYTHEMATOSUS definition

-Autoimmune disease characterised by the proliferation of antinuclear antibodies. These form immune complexes which can deposit anywhere in the body.

- more common in young women

- relapsing remitting course

- more common in afrocaribbeans/asians

SYSTEMIC LUPUS ERYTHEMATOSUS investigations

- ANA = most sensitive

- anti dsDNA = most specific (antidsDNA is a subtype of ANA)

- anti-smith = also specific

- Us and Es (to check kidney function)

- clotting screen - (if anti-phospholipid syndrome - paradoxical increase in APTT)

- low levels of C3 and C4 (because of consumption) - this is used to monitor flares!

- CRP may be normal or raised

- ESR raised

SYSTEMIC LUPUS ERYTHEMATOSUS commonly affected demographic

- afro-caribbeans

- women

SYSTEMIC LUPUS ERYTHEMATOSUS risk factors for getting the disease

Smoking

Ultraviolet light

Silica exposure

Epstein-Barr virus

SYSTEMIC LUPUS ERYTHEMATOSUS triggers for flare ups

Oestrogen exposure (e.g. pregnancy, the combined oral contraceptive pill). (this is also why lupus decreases after menopause)

Infections

Emotional stress

Physical stress e.g. surgery, injury

Excessive ultraviolet light exposure

SYSTEMIC LUPUS ERYTHEMATOSUS what score do we use to classify it

EULAR/ACR score

*patients need to have positive ANA antibodies and a score of 10 or more on the EULAR/ACR score

SYSTEMIC LUPUS ERYTHEMATOSUS features

- Fatigue

- Weight loss

- respiratory: pleurites, pleural effusions

- Dermatological: scalyMalar rash (butterfly shaped). Spares nasolabial folds

- cardiovascular - Libman sacks endocarditis

- Musculoskeletal: Arthralgia

- Haematological: anaemia,

- Renal symptoms: lupus nephritus - needs constant monitoring. (classified into 6 stages based on histology). You get a 'Full house' immunofluorescence pattern.

- Neuro: seizures, anxiety and depression, increased risk of stroke

- Complications with pregnancy

- Gastro-intestinal: peritonitis

- Haem: cytopenias

- mouth ulcers

remember: pericarditis, peritonitis, pleuritis

SYSTEMIC LUPUS ERYTHEMATOSUS complications

the complications of long-term steroid use!

SYSTEMIC LUPUS ERYTHEMATOSIS management

conservative

- sun protection

- cap

- not using hormonal contraception

medical

- first line: hydroxychloroquine (side effect is damage to retina, so also need regular eye checks)

- if additional joint issues/constitutional sx: add on steroids, NSAIDs,

- immunosuppressants,

drug induced lupus features

- resolves on stopping the drug

antibodies

- ANA positive

- ant histone antibodies present in 90%

drug induced lupus

sulfonamide

hyddralazine

isoniazid

phenytoin

procainamide

discoid lupus what is it

- basically lupus that mainly affects the skin

SYSTEMIC SCLEROSIS definiton

a condition characterised by hardened, sclerotic skin and other connective tissues

SYSTEMIC SCLEROSIS types of patterns

1. limited (central) systemic sclerosis

- anti-centromere antibodies

- CREST syndrome: calcinosis, Raynaud's phenomenon, oesophageal dysmotility, scleroderma (thickening of skin) - causing shiny skin and telangiectasia (red patches)

2. diffuse systemic sclerosis

- associated with anti scl-70 antibodies

- more lung involvement (affects connective tissue in lung)

SYSTEMIC SCLEROSIS complications

scleroderma renal crisis

this is more common in diffuse systemic sclerosis

it causes:

-AKI

- severe hypertension

also

pulmonary hypertension

more common in limited systemic sclerosis

drug induced lupus causes

Most common causes

procainamide

hydralazine

Less common causes

isoniazid

minocycline

phenytoin

VASCULITIS types

- small vessel (granulomatosis with polyangitis - GPA, microscopic polyangitis, churg-strauss syndrome, henoch-schonlein purpura)

- medium vessel (e.g kawasaki, polyarteritis nodosa, buerger's disease)

- large vessel (e.g giant cell arteritis, takayasu arteritis)

VASCULITIS - small vessel: granulomatosis with polyangitis (GPA) what is it

- a disease where a persons b cells targets granules which are released by their neutrophils (granulomatosis) with an autoantibody called c-ANCA

- this causes free radicals to be released which damages endothelial cells and affects blood flow

this then blood supply to

1. nasopharynx - can cause saddle nose deformity

2. lungs - can cause difficulty breathing, and ulcers -> causing bloody cough

3. kidneys - decreased urine output, increased blood pressure (because kidneys cant maintain function to decrease BP). Can cause rapidly progressive glomerulonephritis,

(classic triad of upper respiratory tract, lower respiratory tract and kidneys)

VASCULITIS - small vessel: granulomatosis with polyangitis (GPA) demographic

middle aged males

VASCULITIS - small vessel: granulomatosis with polyangitis (GPA) treatment

1. corticosteroids

2. cyclophosphamide

VASCULITIS - small vessel: microscopic polyangitis (MP) what is it

very similar disease to GPA except

- doesn't affect nose (only kidney and lungs)

- the antibodies are called p-ANCA not c-ANCA

VASCULITIS - small vessel: churg-strauss syndrome aka eosinophilic granulomatosis with polyangiitis or EGPA what is it

- a types of small vasculitis which

affects nose, kidney, lung + GI (stomach churns)

- antibodies are p-ANCA

- raised eosinophil count (churg Strauss is associated with asthma)

VASCULITIS - small vessel: churg-strauss syndrome aka eosinophilic granulomatosis with polyangiitis or EGPA management

High dose corticosteroids which are weaned after some years (to prevent long term damage)