Martin Edobor, George Nava

Rheumatology Crash Course

Objectives

• Go through GALS screen OSCE station• Cover key Rheum Topics• Practice SBA• General Advice

OSCE

Gals screen - Introduction1. Introduces self and states role

2. Consent and obtains patients name

3. Offers a chaperone

4. Exposure of patient (giving them privacy to do so)

5. Asks the following questions: What is your occupation? Do you have any pain

or stiffness in any joints, muscles

or your back? Do you have any difficulty climbing the stairs? Do you have any

difficulty washing or dressing?

6. General inspection for obvious scars, deformities, abnormal posture or muscle

wasting

7. GAIT: asks patient to walk away and turn and come back, checking for:

Smoothness of movement, abnormalities in gait, normal heel‐toe strike, normal

turning speed, normal

posture and arm swing

Gals screen - Gait

Gals screen – Spine (1)

SPINE: inspect from the back, looking for:

Any deformities such as scoliosis

Level shoulders and iliac crests

Normal cervical and lumbar lordosis

9. Checks muscle bulk in the paraspinals, shoulder girdle, gluteals, calves

10. Inspects the popliteal fossa and Achilles tendon

11. Feels supraspinatus and lightly squeezes trapezius to test for fibromyalgia

12. Runs finger down back to check for tenderness

13. Movements:

Side‐to‐side, rotate upper torso without moving hips

Press ear to shoulder

Open jaw and move side‐to‐side

Flex and extend neck, rotate head

Gals screen – Spine (2)

ARMS: General inspection, comment on deformity or erythema over the joints, Dupuytren’s or thenar wasting in the hand, pitting psoriasis in the nails16. Feels the temperature of the joints then squeezes across the metacarpals for tenderness17. Movements: Put hands behind head and push elbows back, touch the small of back, ‘pray’ with hands together thenback to back Flex and extend elbows, pronation and supination, flex and extend wrists18. Hands: Power grip Precision movements (thumb to each finger in turn)

Gals screen - Arms

LEGS: General inspection for muscle wasting, fasciculations, skin rashes,

nodules, swelling, deformity or

erythema over the joints, calluses or ulcers on soles

20. Feels the temperature of the joints then squeezes across the metatarsals for

tenderness

21. Movements:

Flex and extend hips, knees and ankles

Internal rotation at hips and knees

Inversion and eversion at the ankle

Crepitus and bulge test at the knee

Gals screen - Legs

22. Thanks patient, offers to help them dress

23. To complete my examination, would like to do a full neurological examination

of the upper and lower limbs and

a full cranial nerve exam

24. Presents findings in a clear and fluent manner, for example“This patient doesn’t have any pain or stiffness in any of his joints, muscles or back. He has no difficulty climbing stairs or washing and dressing. On general inspection, there were no obvious scars, deformities, abnormal posture or muscle wasting. On examination GAIT was smooth with no abnormalities. He displayed no weakness and normal range of movement in arms, leg and spine. This patient has a normal GALS screen”

Gals screen - Closing

Rheumatoid Arthiritis (1)A chronic symmetrical inflammatory polyarthititis with systemic involvement

Epidemiology: F>M 3:1, ages 30-50

Associated with HLA-DR4

Pathology: Autoantibodies (IgM) to Fc of IgG, forming immune complexes

which deposit in joints leading to inflamation.

Symptoms: Pain (decrease with activity), swelling, morning stiffness

>30mins,.

Joint Involvement: MCP, MIP, PIP, MTP SYMETRICAL!

Rheumatoid Arthritis (4)Systemic extra-articular involvement:

Skin: Rhumatoid nodules, synivotendonitis

Lungs: Effusions, nodules, fibroisis, Caplans syndrome: a combination of

pneumoconiosis and RA

Eyes: Sclertis (painful red eye), Episcleritis(non-painful red eye), Sorgjerns

syndrome

Neural: Mononeuritis multiplex, Carpal tunnel

Felty Syndrome: RA with splenomegaly and neutropenia

Normochromic normocytic anaemia

Rheumatoid Arthritis (5)Investigations

ESR, CRP Increased

Possible Anaemia

RF +ve in 70% of cases, Anti-ccp positive in >90% of cases

CXR –Swelling of soft tissue, erosions, joint narrowing, juxto-articular

osteopenia

Rheumatoid Arthritis (6)Management

2009 NICE guidelines it is recommend that patients with newly

diagnosed active RA start a combination of DMARDs (including

methotrexate and at least one other DMARD, plus short-term

glucocorticoids)

1.DMARDS

Methotrexate (s.e derange LFT’s renal, neutopenia, PF, teratogenic)

•Sulfasalazine (s.e infertility, G6PD heamolysis)

•Lefunimide

Others e.g Azathriopine, Gold

(Also add prednisolone)

The current indication for a TNF-inhibitor is an inadequate response to at least two DMARDs including methotrexate

4. Anti TNF alpha, use if DMARDS failed

e.g Entgeracept, Adelumimab , Infliximab

Side effects increase risk of cancer, infection, reversible refulx and demylinating disease.

If a diagnosis of RA is yet to be made, you can give your patient NSAIDS (if no contraindication) or a steroid injection

A 47 year old woman presents to A+E with dyspnoea. An X-ray confirms a large right sided pleural effusion. Fluid is then aspirated from the right lung. Clinical chemistry analysis showed a pleural fluid lactate dehydrogenase (LDH): serum LDH ratio of 0.76. Which of the following is the most likely cause of this patient’s pleural effusion?

• Meigs’ syndrome• Hypothyroidism• Constrictive pericarditis• Nephrotic syndrome• Rheumatoid arthritis

Pleural effusions

• Transudate– Increased venous pressure– Hypoproteinaemia– Hypothyroidism– Meigs syndrome

• Exudate– Increased capillary perm 2ndry to cytokines

• Infection• Inflammation• Malignancy

SBA question• A 53 year old woman with stable rheumatoid arthritis is to undergo an

elective cholecystectomy. Arthropathy is the only finding on clincial examination. Which of the following is the most important investigation prior to the procedure?

• A) Chest X-ray• B) Echocardiography• C) ABG• D) Cervical spine imaging• E) Uric acid levels

ANSWER

• C) 1 in 3 patients with RA have atlanto-axial subluxation.

• Hyperextension of C-spine during intubation risks cervical myelopathy

• Imaging and extra care during anaesthesia is necessary

Connective Tissue Diseases

Systemic Lupus Erythematosus (1)

SLE an “Systemic, inflammatory, multisystem disorder”

Autoimmune disease in which auto-antibodies and immune complexes

cause damage to organs.

Epidemiology: High prevalence in Afro-carribeans, Peak age of onset 20-

40 years

Aetiology: Hereditary , Genetic, Immune system disorder (Autoantibody

production + impaired tolerance), UV light , Drugs.

Clinical features• Musculoskeletal 90% :

-Symmetrical, small joint, arthralgia, - Jaccoud’s arthropathy –Joint

deformity resembling RA – rare

• Cutaneous 75%

-Malar Rash:

-Discoid Lupus

• Neurology 60%

-Cerebral Lupus

• Lungs 50%

-Recurent pleurisy and BILATERAL pleural effusions

- “Shrinking Lung syndrome’

• Renal 30% (Histological changes are frequent)

- Regular screening for urine for blood and protein required

‘ A RASH POIN MD’

• Arthralgia• Renal problems• Anti-nuclear antibodies• Serositis• Haematological disorder• Photosensitivity• Oral ulcers• Immunological disorder• Neurological disorder• Malar rash• Discoid rash

• >4 out of 11 needed

Investigations

Investigations• Bloods

FBC: Leucopenia, lymphopenia and/or thrombocytopenia

ESR: Raised

CRP: Normal

U+E: Urea and Creatinine raised in advanced renal disease

• Urine Testing: Protein in urine and RBC Caste

• Complement: Low C3 Low C4

Imaging

• CT– infarcts or haemorrhage with evidence of cerebral atrophy

• MRI – lesions in white matter

Serum Antinuclear Antibodies (ANA) Positive >95%

Double stranded DNA Specific for SLEPositive 60%

ENA: Anti-Ro and Anti-LA Detected

RF Positive in 40% of patients

Anticardiolipin antibodies Present in 35-45% of patients

Serological Syphilis Test False Positive 1/3rd

Immunoglobulins ↑ (IgG and IgA)

Investigation-Serology

A 32 year old pregnant woman with a history of malar rash, photosensitivity and joint pain in her hands is seen in antenatal clinic. An appropriate autoantibody screen is performed. Which of following autoantibodies are most associated with poor fetal cardiac function with respect to this patient?

• Anti-dsDNA • Antinuclear • Antiphospholipid• Anti-Ro• Anti-La

Management

• Symptomatic treatment-NSAIDS-Antimalarials: e.g hydroxychloroquine

• Corticosteroids and immunosuppressive drugs-Single dose I.M corticosteroids for flares-immunosuppressive drugs for severe thrombocytopenia,

renal and cerebral diseases.

What is the best test to distinguish between a lupus flare and an infection in a patient with lupus?

Answer

ESR and CRP

ESR increased, CRP normal in flare

ESR and CRP both increased in infection

Drugs that worsen SLE

• Penicillamine – what is this?• Procainamide – what is this?• Hydralazine – what is this?

• All associated with drug induced lupus

• Which antibody is associated with drug induced lupus?

Sjogren’s Syndrome2 types:

Primary Sjogren’s: 9:1 F:M ratio

Secondary Sjogren’s:Associated with CT diseases: RA, SLE, Systemic Sclerosis

Classic Features1.Decreased tear production

—Dry eyes—Keratoconjunctivitis sicca

2.Decreased salivation—Dry mouth—Caries

Other features:—Arthritis, Raynaud’s , Interstitial Nephritis

Investigations• Measure the affected areas: Conjunctival dryness

• “Schirmer’s Test”: Filter paper under lower lid, measure distance along paper that tears are

absorbed 5mm in 5mins = +ve

• Blood tests:FBC: Increased Ig Autoantibodies: Anti-Ro + Anti-La(70%), RF+, ANA+(70%)

Treatment•No cure

•Symptomatic relief:Eye drops (artificial tears)Gels, frequent drinks, sugar free gum/pastilles etc.NSAIDs and immunosuppressants in severe systemic disease

A 42 year old woman presents to her GP with dry eyes, dry mouth and joint pain. She undergoes a Schirmer’s test which reveals a tear absorption rate of 3mm in 5 minutes. Concerning the diagnosis, which of following is most true?

• Patients are at an increased risk of non-Hodgkin’s lymphoma

• Women are three times more likely to be affected than men

• The peak onset of age is in the 6th decade of life• Rheumatoid factor is positive in 70% of patients• Is associated with anti-mitochondrial antibodies

Systemic Sclerosis

What is it?

• Chronic multisystem disease• Mainly affects skin and associated with Raynaud’s• F:M 3-5:1• Presents between age 30-50

• 2 main types:i. Limited cutaneous sclerodermaii. Diffuse cutaneous scleroderma

Clinical Features

1. Limited cutaneous scleroderma (60%)– Initially get Raynaud’s– Followed by slow skin changes - hands, feet and

forearms– Thickened skin– (Formerly known as CREST syndrome)– Calcinosis, Raynauds, Esophagitis, Sclerodactly,

Telangiectasia

Clinical Features

2. Diffuse cutaneous scleroderma (40%)– Initially get Raynaud’s– More rapid and widespread skin changes– Also affects:

1. Oesophagus (like limited type)2. Lungs: Pulmonary fibrosis , pulmonary vascular

disease, causing pulmonary HTN3. Kidneys: Acute or chronic renal failure4. Heart: pericarditis, pericardial effusion, myocarditis,

arrythmias

Investigations

• Blood tests:– FBC: normochromic, normocytic anaemia, raised ESR– Autoantibodies (NOT present in all):

• Limited cutaneous: anti-centromere• Diffuse cutaneous: anti-Scl70, anti-RNA pol I + III

• Radiology: XR, CT and barium swallow• Oesophageal manometry

Treatment

• No cure

• Symptomatic relief:– Immunosuppression with iv cyclophosphamide– ACE inhibitors or ATII receptor antagonists for HTN– Hand warmers/ Ca channel blockers for Raynaud’s

Overview

Polymyositis is a rare, subacute inflammatory myopathy.

OverviewOverview• Very rare (2-10/million); ♀ > ♂

• Subacute: weeks to months with typical delay before presentation

• Inflammation of striated muscle

• Aetiology complex and uncertain

Clinical featuresClinical features• Proximal muscle weakness:

– Progressive– Symmetrical

• Which sort of movements are initially affected?

• Distal weakness may occur later

• Ocular and other facial muscles spared even in advanced, untreated cases

Clinical Features

• Respiratory, oesophageal & laryngeal muscles can be affected

• Weakness is associated with wasting.

• Isolated Polymyositis is incredibly rare. More commonly it is associated with either:– Systemic autoimmune or connective tissue disease– Viral or bacterial infection

• Systemic symptoms including Raynaud’s phenomenon.

InvestigationsInvestigations

1 CK – raised (up to 50-fold)

2 Electromyography (EMG) show abnormalities.

3 Muscle biopsy. This is the definitive test

Management

• Goal is to improve muscle strength.

• First-line is 3 month trial of oral Prednisolone. This fails in 75% of patients

• If this fails, other immunosuppressive drugs used.e.g. methotrexate, azathioprine, mycophenolate

• If immunosuppression fails, rethink diagnosis. (Repeat biopsy!)

Any questions?

Take-home messages

SLE is a systemic, inflammatory, multisystem disorder. It is an autoimmune disease in which auto-antibodies and immune complexes cause damage to organs.

Sjogren’s Syndrome is a chronic inflammatory condition due to immunologically mediated destruction of epithelial exocrine glands

Systemic Sclerosis is a chronic multisystem disease. It mainly affects skin and is associated with Raynaud’s

Polymyositis is a subacute inflammatory myopathy of a proximal-to-distal progressive symmetrical pattern

Gout

• What do you know?

Gout

• Hyperuricaemia and uric acid crystals• Predilection for MTP joint (70%)

• Presenting features?• Risk factors

Gout Risk Factors

• Alcohol• Drugs – which ones?• High purine diet• High cell turnover• CKD

Ix

• MUST RULE OUT SEPTIC ARTHRITIS• Joint aspirate – negatively birefringent needle

shaped crystals under polarised light• X-ray• U+Es• Serum uric acid – may be normal in acute attack

A 67 year old man with gastro-oesophageal reflux disease (GORD) presents with an acutely painful swollen right metatarsophalangeal joint. Joint aspiration reveals the presence of needle shaped crystals that are negatively birefringent when viewed under polarised light. Which of the following is the most appropriate initial treatment option in this patient?

• Colchicine• Indomethacin• Allopurinol• Steroids• Febuxostat

Rx

• NSAIDs /colchicine/ steroids for acute phase

• Allopurinol/ Febuxostat/ Rasbicurase for chronic phase

• What if patient is on azathioprine?

Azathioprine toxicity

• Nausea • Fatigue• Rash• Hair loss• BONE MARROW SUPPRESSION

• Need to measure thiopurine S-methyltransferase levels before you start a patient on AZT

Pseudogout

• What do you know?

Pseudogout

• Calcium pyrophosphate crystals

• Tends to affect larger joints

• Risk factors?

Pseudogout risk factors

• Hypomagnesaemia• Hypophosphataemia• HAEMOCHROMATOSIS• WILSON’S DISEASE• HYPERPARATHYROIDISM

Ix• MUST RULE OUT SEPTIC ARTHRITIS

• Polarised light microscopy of joint fluid• X-ray• Consider secondary causes (esp if under 60)

• Calcium• Magnesium• Iron studies• Caeruloplasmin

Practice SBA’s1. Which one of the following systems is most commonly affected in

SLE?

A. JointsB. SkinC. ChestD. KidneyE. Heart

Clinical features• Musculoskeletal 90% :

-Symmetrical, small joint, arthralgia, - Jaccoud’s arthropathy –Joint

deformity resembling RA – rare

• Cutaneous 75%

-Malar Rash:

-Discoid Lupus

• Neurology 60%

-Cerebral Lupus

• Lungs 50%

-Recurent pleurisy and BILATERAL pleural effusions

- “Shrinking Lung syndrome’

• Renal 30% (Histological changes are frequent)

- Regular screening for urine for blood and protein required

2.You review the hands of a 60-year-old man who is complaining of 'arthritis' in his hands:

A. Rheumatoid arthritisB. Systemic SclerosisC. Systemic Fungal infectionD. Psoriatic Arthritis E. Reiter's Syndrome

3. A 49 year old man suddenly experiences severe pain in his left ankle while walking. His past medical history is unremarkable, except for a recent course of antibiotics for gastroenteritis. Clinical examination reveals an inability to plantar flex the left foot. Which of the following antibiotics was the patient most likely to have been taking?

• Erythromycin• Metronidazole• Amoxicillin• Cefalexin• Ciprofloxacin

4. A 57-year-old man presents to his GP due to pain in his right knee. An x-ray shows osteoarthritis. He has no past medical history of note. What is the most suitable treatment option for the management of his pain?

A.Oral Doclofenac with omeprazoleB.Oral glucosamineC.Oral DiclofenacD.Oral IbuprofenE.Oral paracetamol

5. You refer a 24-year-old female to rheumatology with intermittent pain and swelling of the metacarpal phalangeal joints for the past 3 months. An x-ray shows loss of joint space and soft-tissue swelling. Rheumatoid factor is positive and a diagnosis of rheumatoid arthritis is made. What initial management is she most likely to be given to help slow disease progression?

A.InfliximabB.MethotrexateC.SulfasalazineD.Methotrexate + sulfasalazine + short-course of prednisoloneE.Diclofenac

Rheumatoid Arthritis (6)Management

2009 NICE guidelines it is recommend that patients with newly

diagnosed active RA start a combination of DMARDs (including

methotrexate and at least one other DMARD, plus short-term

glucocorticoids)

1.DMARDS

Methotrexate (s.e derange LFT’s renal, neutopenia, PF, teratogenic)

•Sulfasalazine (s.e infertility, G6PD heamolysis)

•Lefunimide

Others e.g Azathriopine, Gold

(Also add prednisolone)

6. A 25-year-old man presents complaining of dysuria and pain in his left knee. Three weeks previously he had suffered a severe bout of diarrhoea. What is the most likely diagnosis?

A.Reactive ArthritisB.Disseminated gonococcal infectionC.Bechet’s diseaseD.Ulcerative colitisE.Rheumatoid arthritis

7. A 38 year old intravenous drug user presents with an acutely painful swollen left knee and a temperature of 38.30C. Joint aspiration reveals a turbid, yellow fluid which is sent for culture. Which of the following is the most appropriate initial empirical treatment?

• Intravenous flucloxacillin and intravenous benzylpenicillin

• Oral metronidazole• Oral amoxicillin and oral erythromycin• Intravenous cefotaxime and intravenous

gentamycin• Oral flucloxacillin and oral penicillin V

8 A 72 year old lady presents with a two year history of right hip pain. She mentions that it is particularly troublesome after she has walked to the shops. X-ray shows loss of joint space and subchondral sclerosis and cysts. Which of the following is the most effective treatment for this lady?

• Physiotherapy• Weight loss• Joint replacement• NSAIDs• Glucosamine and chondroitin sulphate

9. Which one of the following is least associated with systemic lupus erythematous?

A.Anti-nulcear antibodiesB.Anti-Sm AntibodiesC.Elevated ESRD.Elevated C3 And C4 levelsE.Elevated anti-dsDNA titres in active disease

SLE: ANA is 99% sensitive - anti-Sm & anti-dsDNA are 99% specific

C3, C4 are low in SLE not elevated

10. You are doing the annual review of a 50-year-old woman who has rheumatoid arthritis. Which one of the following complications is most likely to occur as a result of her disease?

A. Chronic lymphocytic leukaemiaB. HypertensionC. Colorectal CanerD. Type 2 diabetes mellitusE. Ischaemic heart disease

11 A 38 year old woman with known rheumatoid arthritis presents with a history of shortness of breath, easy bruising and recurrent chest infections. On clinical examination there is splenomegaly. Which of the following is the most likely explanation for this presentation?

• Sulphasalazine• Methotrexate• Penicillamine• Felty’s syndrome• All of the above

12 A 50 year old woman undergoes a DEXA bone scan which reveals a T score of -2.6. Taking the history reveals a strong family history of breast cancer and a 30 pack year smoking history. Which of the following treatment options is most appropriate for this patient?

• Alendronate• Strontium Ranelate• Vitamin D• Calcitonin• Raloxifene

Can you any name anti-osteoporotic medications and their side effects?

• Anti-SSA• Anti-topoisomerase-I• Anti-centromere• Anti-liver kidney microsomal• Anti-Jo1