Dr Clarke Essential Medicine - Rheumatology and Endocrinology

Feature of inflammatory arthropathy (S/S)

Symptoms:

Early morning stiffness

Worse with rest, better with movement

Signs:

Soft tissue swelling eg loss of valleys between Knuckles

(Raised ESR,CRP)

Features or rheumatoid arthritis

Symmetrical, inflammatory arthritis

Small joints of hands and feet

Also hips, elbows and knees

Young adults, F>M

HLA DR4 associate

What is rheumatoid factor

IgM against your own IgG

Lots of normal people have it

Lots of people with classic rheumatoid do not have it

High titres associated with progressive disease

Features of the rheumatoid hands (dorsal aspect)

Symmetrical, peripheral destructive arthropathy

Soft tissue swelling with spindling of proximal joints and loss of valleys between knuckles

Palmar subluxation and ulnar deviation at metacarpo-phalangeal joints

Nodules- otherwise "rheumatoid pattern"

Features of rheumatoid hands (Palms)

Palmar erythema

Wasting of thenar eminence- carpal tunnel syndrome

Fixed flexion contracture

Specific abnormalities- swan neck, button hole, z thumb

Due to "rheumatoid tenosynovitis"

What causes Positive prayer sign in early inflammatory arthropathy

Rheumatoid tends synovitis

What is seronegative rheumatoid

One third of patients are seronegative

Identical disease presentation to seropositive in most respects, but unlikely to have nodules or extra-

articular features

Most have some non-classical rheumatoid factors (IgG vs IgG)

40% have antibodies to cyclic citrillinated peptide (CCP); if so, more likely to be rapidly progressive

Investigations for suspected rheumatoid arthritis

FBC, CRP

Renal and liver function tests (baseline)

Rheumatoid factor

Anti-CCP abs (if positive, more likely to be rapidly progressive)

X-ray hands and feet (if erosions present at baseline, more likely to be rapidly progressive)

Refer even if all these are negative

DMARDS

"Treat to target" strategy

• Start conventional DMARD asap

• Three first line drugs: methotrexate, leflunomide and sulfasalazine

• May need "bridging treatment" with steroids

• Target: remission or low disease activity

• Intensive monthly monitoring until achieved

• CRP and disease activity score (DAS 28) at each visit

• If not winning, escalate dose

• If still not winning after 6 months, step-up to dual DMARD

• If still not winning and high level of disease activity, offer biological

What are the first line DMARDs

Methotrexate

Leflunomide

Sulfasalazine

When is bridging treatment offer while using DMARDs

What are the bridging drugs

If severe

Bridge with steroids (DMARDs take about a month to work)

What is the target with DMARDs

Remission/ low disease activity

Intensive monthly monitoring until achieved

(CRP and disease activity scoring (DAS28) at each visit)

What to do if DMARDs not working sufficiently

Escalate dose

After 6 months still not sufficient: dual DMARD

Still not sufficient: biological

DMARDS and the immune system mind map

How does methotrexate work

Once weekly

Anti proliferative / anti folate

Examples of TNF inhibitors

Infliximab

What is given with methotrexate

Folate - to decrease hematological/GI and hepatic side effects (given on a non methotrexate day)

Complications of methotrexate

GI SEs

Bone marrow suppression

Liver and renal toxicity (rare)

Pneumonitis/pulmonary fibrosis (rare)

What drug is contraindicated with methotrexate

Trimethoprim (both anti folate drugs)

Excretion can be inhibited by NSAIDs

What bloods need to be checked prior to methotrexate

FBC

EGFR

LFT

What test need to be done prior to starting biological

Tuberculin skin test/ interferon gamma release assay

CXR

+/- Hep B/C and HIV

Treat latent tuberculosis

Warm patient that risk of serious infection (2% per annum)

Atlantoaxial subluxation

Tendons at the top of the cervical spine can be weakened by rheumatoid tenosynovitis

If odontoid peg subluxation backwards over days or week = compress upper cervical cord = progressive spastic tetraparesis

Sudden = rapid output of inhibitory impulses down vagus +/- cardiac arrest

Differential diagnosis for rheumatoid hands

Rheumatoid arthritis

Psoriasis arthropathy

Systemic lupus

Osteoarthritis with inflammatory component

Types of psoriatic arthropathy

Oligo arthritis (70%)

Distal (15%)

Rheumatoid (15%)

Arthritis mutilans (telescoping; rare)

Sarcoiliitis (may be associated)

Treatment options for psoriatic arthropathy

Sulfasalazine

Methotrexate

TNF inhibitor (eg adalimumab)

Interleukin inhibitors (ixekizumab)

Janus kinase inhibitors (eg upadacitinib)

Radiographic changes in rheumatoid arthritis

SPADES

S - soft tissue swelling

P - peri articular osteoporosis

A - absent osteophytes

D - deformity

E - erosions

S - subluxation

Radiographic changes in osteoarthritis

LOSS

L - loss of joint space (cartilage thinning)

O - osteophytes

S - subchondral cysts

S - subchondral sclerosis

Extra articular manifestation of rheumatoid

FACEBOOKS

F - feltys syndrome

A - Atlantoaxial subluxation

C - Caplin syndrome and pulmonary nodules

E - effusions

B- blood (normochromic normocytic)

O - olecranon bursitis

O - oral dryness (sicca syndrome)

K - kidneys (amyloid, gold and penicillamine)

S - sensory neuropathy and scleromalacia

Feltys syndrome

RA, splenomegaly, neutropenia

Caplans syndrome

rheumatoid nodules in the lungs

Olecranon Bursitis

inflammation of the bursa located over the olecranon process of the elbow

Features of scleroderma

Hands:

Sclerodactylty

Telangiectasia

Raynauds

Evidence ulceration (knuckles, finger tips)

Prayer test

Subcutaneous calcifications

Skin tethering:

Face , upper arms, chest (diffuse cutaneous scleroderma)

Telangiectasia

Mouth opening reduced; (microstomia)

Test with three finger test

Pathophysiology of vasculitis in scleroderma

Intimal vasculitis

Spectrum of systemic sclerosis

CREST syndrome

Calcinosis, Raynaud's, esophageal dysmotility, Sclerodactyly, Telangiectasia

Features of diffuse cutaneous scleroderma

More extensive with internal organ involvement

Mask like facies, wrinkled skin, microstomai

Tethering of skin over nose

Interstitial pulmonary fibrosis (restrictive ventilatory defect)

Renal involvement (hypertension, impaired renal function)

Atonic oesophagus (reflux and aspiration)

Three questions to ask in the patient with suspected scleroderma

Do you hands change colour in the cold (white, blue, red)

Do you get breathless? (Scleroderma affecting chest wall or interstitial fibrosis)

Do you get indigestion or heart burn

Scleroderma treatment

Digital ulcer: bosentan or IV prostanoids

Skin: methotrexate

GI: PPI

Renal crisis: ACE-I

Pulmonary hypertension: bosentan or sildenafil

Raynauds: nifedipine

Key points in rheumatology

Examine the hands means- examine the hands and the elbows and the ears

Common exam cases: rheumatoid arthritis and scleroderma

Don't forget to check hand function

Name the hormones which are insulin antagonists

Adrenaline

Glucagon

Cortisol

Growth hormone

(Ie when there is less insulin in the body, these hormones come into action)

Signs of hypoglycaemia

Low blood pressure

Tachycardia

Pale, cold, clammy

Seizures

Thodds paraesis

ABCD approach to unconscious patient with hypoglycaemia in hospital

Airway:

Guedel airway to prevent obstruction by tongue

Breathing:

give oxygen

Circulation:

check pulse and BP;

get IV access;

send bloods and check bedside glucose stick testing.

Glucose <4mmol/l;

Give IV glucose and then IM glucagon

Recheck glucose in 10 minutes. (IV glucose eg 200ml 10% dextrose over 15 minutes)

Disability:

check response eg response to voice, pain or unresponsive

D2:

don't ever forget glucose in an unconscious patient

Neuroglycopenia

deficiency of sugar that interferes with normal brain activity

<3mmol/l

Ketoacidosis diagnostic triad

Hyperglycaemia (>11mmol/l or known diabetic)

Ketonuria (blood ketones >3mmol/l)

Acidosis (pH <7.3, or bicarbonate <15mmol/l)

Lower limit of normal for blood glucose

4mmol/l

(Four is floor)

Features of ketoacidosis

Dehydration (hypokaelamic):

Tachycardia and hypotension

Kussmaul respiration-acidosis

Sweet ketone smell

Vomit + abdominal pain

Signs of precipitating cause (eg infection, MI, etc)

Prinicples of treatment for diabetes.

Gastric aspiration

Rehydration

Insulin replacement

Potassium replacement

The potassium paradox in ketoacidosis

• Osmotic diuresis takes potassium

• Dehydration: renin..angio..aldosterone- further potassium loss

• Total body K+ always low eg 200mmol deficit

• But serum K+ is occasionally high or normal at presentation (serum potassium is high because there is a pre renal AKI = no excretion + anuria)

What should be done in the first hour in regards to ketoacidosis (PANICS)

P - Potassium- measure hourly: omit if anuria suspected or serum level >5.5mmol/l

A - Acidosis: check venous pH and ketone levels

N - "Normal saline": 500ml over 15 minutes if systolic <90mm; otherwise 1litre in first hour

I - Insulin by infusion (weight based, fixed rate, soluble insulin: 0.1 units/kg/hour)

C - Catheter and cultures; urine, blood etc

S - Stomach aspiration if drowsy; endotracheal tube first if no gag reflex

Examination of diabetic foot

Inspect- including heels

Palpate- capillary refill and pulses

Light touch- finger, cotton wool or monofilament

Avoid pin prick testing (risk of infection introduction)

Vibration sense

Ankle jerks

Buzz words "Loss of protective sensation"

Triad for Graves' disease

Goitre

Eye signs (lid retraction + lid lag)

Thryotoxicosis

Oligoclonal IgG antibodies in Graves' disease

Peripheral thryoid signs

• Lid retraction and / or lid lag

• Clubbing and onycholysis

• Fine tremor, moist palms

• Tachycardia, atrial fibrillation

• Biceps reflex

• Slow relaxation- hypothyroid

• Proximal myopathy

• Pre-tibial myxoedema = Thyroid dermopathy (non-pitting)

Graves eye disease features

Oedema (periorbital and chemosis)

Proptosis (best assessed from the side)

Retraction (autonomically mediated)

Exposure keratopathy

Ophthalmoplegia (upgaze palsy)

Graves' disease treatment options

Carbimazole low dose

Treat and block

Surgery

Radioiodine

Features of hypothryoidism

Gruff voice, slow cerebration

Coarse facial features

Dry cold scaly skin

Slow pulse and slow relaxation biceps jerk

May have goitre (Hashimoto's disease)

May have myxoedema (soft tissue swelling= oedema of the myxos)

Typical face of a person with hypothyroidism

Pale or yellow appearance "Peaches and cream" complexion Puffy face with periorbital oedema Dry,coarse hair

Diffuse hair loss

Loss of lateral eyebrow hair

(poor reliability)

Thickened dry flaking skin

Deep hoarse voice

Myxoedema in hypothryoidism

Severe hypothyroidism

with swelling of subcutaneous tissues

Typically around eyes and the backs of the hands

Often has a purplish tinge Tight rings

Tight rings

Cushing's syndrome features (catabolic, glucocorticoid and mineralocorticoid effects)

Catabolic:

Myopathy

Striae

Bruising

Osteoporosis

Glucocorticoid:

Diabetes

Obesity

Mineralocorticoid:

Hypertension

Hypokalaemia

Examination schematic for cushings patient

SWEDISH Mnemonic

S - spinal tenderness

W - weight (central obesity)

E - easy bruising

D - diabetes

I - interscapular fat pad

S - striae

H - hypertension

Causes of Cushing syndrome

Iatrogenic Cushing (from corticosteroid therapy)

Adrenocortical adenoma (secretes excess cortisol)

ACTH-secreting pituitary adenoma

Paraneoplastic Cushing (due to ACTH secretion by tumors)

Investigations for hypercortisolaemia

24 hour urinary free cortisol

Loss of diurnal rhythm

Low dose dexamethasone test (no suppression of endogenous cortisol)

How differentiate adrenal from pituitary disease (cushings)

ACTH levels high in pituitary (cushings)

ACTH level low in adrenal (cushings)

(ACTH assay is tricky though and high dose dexamethasone test is to have poor reliability)

Acromegaly

enlargement of the extremities

Hand features of acromegaly

Increase size

Thenar eminence: wasting if carpal tunnel syndrome

Check sensation in median distribution

Hyperhydrosis (palms)

Bogginess of palms

Skin fold thickness increased in active disease

Facial features of acromegaly

Prominent supraorbital ridges,

Big ears, nose and lips

Macroglossia

Prognathism (from the size) (pro- gnathism= translates as "forward gnashers" ie protrusion of jaw due to overgrowth of the mandible; also leads to wide separation of the teeth)

Ask patient "Show me your gums" to demonstrate wide separation of teeth and that lower jaw teeth are in front of upper teeth (reversal of normal)

Mnemonic for acromegaly

Boggy sweaty ABCDEF

Boggy sweat = active

A - arthropathy

B - BP

C- carpal tunnel

D - diabetes

E - englahred tongue, heart, thyroid

F - field (bitemporal hemianopia)

How is acromegaly confirmed

Insulin like growth factor levels

Failure of suppression of GH levels during oral glucose tolerance test

(GH is an insulin antagonist and is normally suppressed by glucose)

Average hourly growth hormone levels

Examination schematic for acromegaly

Rx for acromegaly

Surgery

Radiotherapy

Medical treatment( in prep for surgery)

What screening test is NB in acromegaly

Colonoscopy from age 40