Rheumatology

Question 1

Describe Systemic Lupus Erythematosus, it’s features, investigations, and management

Answer 1

Multisystemic autoimmune disease may be idiopathic or drug induced. 9 times more common in women, usually women of child-bearing age of afro-caribbean or asian background. Associated with HLA B8, DR2, DR3.

SOAP BRAIN MD >3
Serositis - pleuritis/pericarditis
Oral ulcers/nasopharyngeal
Arthritis >1; Jaccoud's arthropathy
Photosensitivity
Blood disorders
Renal disorders
ANA +ve
Immunological disorders
Neurological
Malar rash
Discoid rash

Tests: ANA+ve, Anti dsDNA +ve, ESR raised, normal CRP, may also have anti-Ro, anti-La, anti-Sm, and Anti-RNP, Reduced C3 + C4
FBC, U&E, LFT, CRP
Skin/renal biopsy
Urine - casts/protein

Management
-Acute flares: IV cyclophosmide + high-dose predisolone
-Lupus nephritis: Cyclophosphamide or mycophenolate
BP control - ACE, a-blockers, CCB
-Maintenance: hydroxychloroquine, azathioprine, methotrexate, mycophenolate
-Rashes: sunblock and topical steroids
B cell depletion - rituximab, belimumab
Future - interferon a, IL6 inhibition, T cell targets

Question 2

What are the causes of drug-induced lupus?

Answer 2

skin and lung signs prevail can be due to >50 drugs.
isoniazid, hydralazine, procainamide, quinidine, chlorpromazine, minocycline, phenytoin.
antihistone antibodies in 100%

Question 3

Describe antiphospholipid syndrome

Answer 3

Can be primary or associated with SLE. Antiphospholipid antibodies causes CLOTs - Coagulation defect,
Livedo reticularis
Obstetric complications (recurrent miscarriage)
Thrombocytopenia. 
Thrombotic risk managed with low dose aspirin or warfarin if recurrent thrombosis.

Question 4

Describe Livedo reticularis

Answer 4

pink-blue mottling caused by capillary dilation and stasis in skin venules. May be a sign of SLE and Vasculitis.

Question 5

Describe Henoch-Schonlein Purpura

Answer 5

A small vessel vasculitis that presents with purpura over the buttocks and extensor surfaces typically affecting young males. May be glomerulonephritis, arthritis, and abdominal pain.

Question 6

Describe Vasculitis

Answer 6

An inflammatory disorder of blood vessel walls causing destruction or stenosis. It can affect any organ and may be primary or secondary due to SLE, RA, Hepatitis B/C, HIV. Categorised depending on size of vessel

Large: Giant Cell Arteritis, Takayasu’s Artertits

Medium: Polyarterits Nodosa, Kawasaki Disease

Small:
pANCA +ve: Microscopic polyangitis, glomerulonephritis, Churg-Strauss syndrome
cANCA +ve: Wegener’s Granulomatosis (GPA)

ANCA -ve: Henoch-Schonlein purpura, Goodpasture’s Syndrome and cryoglobulinaemia

Question 7

Describe Giant Cell Arteritis (GCA), its presentation, tests, and management

Answer 7

Large vessel Vasculitis, common in the elderly (consider takaysu’s if under 55yrs) associated with Polymylagia Rheumatica (PMR).

Presentation: headache, temporal artery and scalp tenderness, jaw claudication, amaurosis fugax or sudden blindness.

Test: increased ESR (typically over 47) + CRP (typically greater than 2.45mg/dL), increased platelets, increased ALP and decreased Hb.
Temporal biopsy 7-14 days of steroids (beware skip lesions) - up to 6 weeks

Management:

• Immediate Prednisolone 60mg/d PO due to risk of irreversible blindness.
• N methylprednisolone if impending visual loss
• aspirin 75mg OD

Question 8

Describe Polyarteritis Nodosa (PAN)

Answer 8

Medium vessel necrotising Vasculitis that causes aneurysms and thrombosis –> infarction in affected organs. Twice as common in men may be associated with Hepatitis B.

Presentation: systemic features; skin, renal, cardiac, neuro, GI, GU

Tests: increased WCC, mild eosinophilia, anaemia, increased ESR + CRP. Renal biopsy/ renal or mesenteric angio - rosary bead aneurysm

Rx: BP control and corticosteroids/cyclophosphamide (if severe); plasma exchange.
If hepB- antivirals

Question 9

Describe Microscopic Polyangitis

Answer 9

It is a p-ANCA +ve small (medium) vessel vasculitis. presents with rapidly progressive glomerulonephritis +/- Pulmonary haemorrhage.

Treatment: prednisolone 15mg/day PO

Question 10

Describe Polymyalgia Rheumatica (PMR)

Answer 10

Linked with Giant Cell Arteritis but not a true Vacsculitis. Typical patient >50yrs presenting with bilateral aching, tenderness and morning stiffness of shoulders and proximal limb muscles

Tests: raised CRP and normal or raised ESR. 30% have increased ALP. CK should be normal and can help distinguish from myopathy. Normocytic normochromic anaemia

Mx: prednisolone 15mg/d PO (most need for >2years) so give PPI and bisphosphonates

Differentials: RA, polymyositis, hypothyroidism, osteroarthritis, malignancy, spinal stenosis.

Question 11

Describe Takayasu’s Arteritis

Answer 11

Large vessel vasculitis rare outside of Japan that affects the aorta and its branches. Often affects women age 20-40yrs. Aortic arch is often affected giving symptoms such as dizziness, visual changes, weak arm pulses.

Complications include aortic regurgitation, aortic dissection, ischaemic stroke, ischaemic heart disease. Also increased BP due to renal artery stenosis.

Question 12

Describe Kawasaki Disease and its symptoms.

Answer 12

Medium vessel vasculitis of children similar to PAN. Median age is 10 months. Can lead to coronary artery aneurysm and infarction.

Symptoms: CRASH + burn
Conjunctivitis (bilateral non-purulent)
Rash (erythematous to desquamation)
Adenopathy (cervical LN)
Strawberry tongue
Hands and feet inflammation
Fever >5 days

Rx IVIG within 1st 10 days and aspirin

Question 13

Describe Churg-Strauss Syndrome

Answer 13

pANCA +ve triad of adult-onset asthma, eosinophilia, and vasculitis affecting the lungs, nerves, heart and skin.

Question 14

Describe Wegner’s Granulomatosis (Granulomatosis with Polyangitis GPA), its symptoms, and management.

Answer 14

cANCA +ve necrotizing small vessel vasculitis. It mostly affects upper respiratory tract, lungs and kidneys.

Symptoms: Upper airways disease is common with nasal obstruction, ulcers, epistaxis, or destruction of nasal septum causing saddle nose deformity. Pulmonary involvement may cause cough, haemoptysis, pleuritis.

Management:

• steroids
• cyclophosphamide in steroid-resistant.
• consider plasma exchange in severe cases.

Question 15

Describe Goodpasture’s Disease

Answer 15

ANCA -ve small vessel vasculitis affecting kidneys and lungs. Characterised by acute glomerulonephritis + lung symptoms (haemoptysis) caused by anti glomerular basement membrane antibodies.

Question 16

What is cyroglobulinaemia, what conditions is it associated with?

Answer 16

Condition in which blood contains cyroglobulins (proteins that become insoluble at low temperatures).

They may be present in Mycoplasma Pneumonia, Post-streptococcal Glomerulonephritis, Multiple Myeloma, SLE, RA, Hepatitis B+C, HIV.

Question 17

Describe Sjogrens syndrome its features, tests and treatment

Answer 17

It is a chronic inflammatory autoimmune disorder which may be primary (Onset 40-50yrs 9 times more common in women) or sencondary associated with connective tissue disease (RA, SLE, systemic sclerosis). There is lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary glands.

Features: decreased tear production, dry eyes, decreased salivation, parotid swelling, other glands are affected causing vaginal dryness. Systemically raynauds may present and lung, liver, joint and kidney involvement. It is associated with other autoimmune disease.

Tests: Anti-Ro (40%) and La +ve (26%), ANA +ve (75%), RF+ve (38%)

Managment: artificial saliva and tears, frequent drinks. NSAIDs and hydroxychloroquine for arthralgia, immunosupression for severe systemic involvement

Question 18

Describe Rheumatoid Arthritis, its presentation, signs, extra-articular manifestations, investigations and management.

Answer 18

RA is a chronic systemic inflammatory disease, characterised by a symmetrical, peripheral polyarthritis. It is twice as common in women.

Presentation:

• Typically presentation is symmetrical, swollen, painful and stiff small joints of the hands and feet. It is associated with early morning stiffness.
• May present suddenly with widespread arthritis
• May present as a recurring mono/polyarthritis of various joints (palindromic RA)
• May present with persistent mono-arthritis
• May present systemically with initially few joint problems

Signs: Early signs include swollen MCP, PIP, wrist or MTP joints (often symmetrical). Later joint damage occurs and there may be ulnar deviation of the fingers and dorsal wrist subluxation. Boutonniere and swan-neck deformities of the fingers or z-deformity of the thumb.

Extra-articular manifestations: nodules on elbows and lungs, lymphadenopathy, vasculitis, fibrosing alveolitis, pleural/pericardial effusions, Raynaud’s, carpel tunnel, osteoporosis, amyloidoss, splenomegaly (Felty’s syndorme = RA + Splenomegaly + neutropenia)

Investigations: RF+ve (~70%), high titre associated with severe disease, X-ray of hands may show LOSE:
-Loss of joint space
-Osteopenia (juxta-articular)
-Soft-tissue swelling
-Erosions
Anti-cyclic citrullinated peptide antibodies (anti-CCP) are highly specific for RA.
FBC may show anaemia of chronic disease and increased platelets. ESR and CRP are also raised.

Management: Disease activity measured using DAS28 aiming to reduce score to less than 3.

• early used of DMARDS and biological agents improve long term outcomes. NSAIDs are good for symptom relief but have no effect on disease progression.
• Sulfasalazine and Hydroxychloroquine are usually 1st line combination therapy with replacement of sulfasalazine with methotrexate if disease not controlled.
• Biological agents may be used if failure to respond to two DMARDs and as DAS28 score >5.1
• TNF-alpha inhibitors are first line e.g. infliximab, etanercept, adalimumab usually given in combination with methotrexate though etanercept and adalimumab can be used in those intolerant of methotrexate.
• B-cell depletion e.g. rituximab is next used in combination with methotrexate when above therapy fails.
• IL-1 and IL-6 inhibition e.g. tocilizumab (IL-6) can be used in combination with methotrexate when above steps have failed.
• T-cell disruption agents such as abatacept may be used in patients with severe RA who have not responded TNF-alpha inhibitors or rituximab.

Question 19

Describe scleroderma (aka systemic sclerosis) the types and management

Answer 19

Systemic sclerosis features scleroderma (skin fibrosis) and vascular disease, it can be split into two main types:

Limited cutaneous systemic sclerosis: formally known as CREST syndrome:
-Calcinosis of subcutaneous tissues
-Raynaud’s,
-oEsophageal and gut dysmotility
-Sclerodactyly (swollen tight digits)
-Telangiectasia
Skin involvement is ‘limited’ to the face, hands and feet. It is associated with anticentromere antibodies in 70-80%. Pulmonary hypertension is often present subclinically.

Diffuse cutaneous systemic sclerosis may involve skin of the whole body and is associated with early organ fibrosis i.e. lung, cardiac, GI and renal. Associated with antitopoisomerase-1 (Scl70) antibodies in 40% and anti-RNA polymerase antibodies in 20%. Requires annual ECG and spirometry.

Management: There is no cure. Immuosuppresive regimens e.g. IV cyclophosphamide are used for organ involvement or progressive skin disease. Monitor BP and renal function, and ACE-i help reduce the risk of renal problems.

Question 20

What is Froment’s test?

Answer 20

A test for ulnar nerve function, patient makes a fist and then grips a piece of paper between thumb and index finger. Examiner attempts to pull the paper away. If the patient can hold the paper ulnar nerve is intact if the patient flexes the thumb to compensate for inability to hold paper this is considered to be a failed test and indicative of ulnar nerve palsy.

Question 21

What is Allen’s test?

Answer 21

This test should be performed before attempting an ABG. It is a test for abnormal circulation in the hand.
-The hand is elevated and the patient is asked to make a fist and squeeze tightly for 30 seconds.
-Pressure is applied to the ulnar and radial artery.
-Still elevated the hand is opened and should appear blanched
-Pressure is removed from the ulnar artery and colour should return within 7 seconds.
If it does not this is a positive and colour does not return to the hand appropriately then ulnar artery supply to the hand is insufficient and an ABG should not be attempted on the radial artery.

Question 22

What is Finklesteins test?

Answer 22

It is a test used to identify DeQuervains tenosynovitis in people with wrist pain. The therapist grasps the thumb and ulnar deviates the hand sharply pain in the distal radial side of the forearm is +ve test. A modified version involves the patient making a fist grasped around the thumb and ulnar deviating but the former is more sensitive when performed correctly.

Question 23

Describe osteoarthritis, its signs and symptoms, tests and mangement

Answer 23

It is the commonest joint condition, three times more common in women, typically presenting >50yrs.

Signs and symptoms:

• Localised disease (usually knee or hip) typically pain on movement and crepitius, worse at the end of the day with background pain at rest.
• Generalised disease, commonly affected joints are the DIP, thumb carpo-metacarpal joints and the knees. There may be joint tenderness, decreased range of movement and mild synovitis. Nodal swellings (bouchards nodes at PIP and Herbedens nodes at DIP)

Tests: Radiograph shows LOSS:

• Loss of joint space
• Osteophytes
• Subarticular sclerosis
• Subchondral cysts

Management: Exercise and weight loss if obese. Analgesia Regular paracetamol +/- topical NSAIDs. Capsicum cream may be tried. Steroidal injections in severe cases. In hip or knee joint replacement if substantial impact on quality of life.

Question 24

Describe Septic Arthritis, its risk factors, investigations and treatment

Answer 24

Consider septic arthritis in any acutely inflamed joint, as it can destroy a joint in under 24h. The knee is affected in >50% of cases. Hip commonly affected in kids, Kochers criteria allows differentiaton from irritable hip (1 point for each, non weight bearing, ESR over 40, Fever over 38.5, WBC over 12, 3 or 4 indicates 95% probability of sepsis)

Risk factors: pre-exisiting joint disease (especially RA), diabetes, immunosuppresion, chronic renal failure, recent joint surgery, prosthetic joints, IV drug abuse, age >80yrs

Investigations: Urgent joint aspiration for synovial fluid microscopy and culture.

Treatment: If in doubt start broad spectrum IV antibiotics until sensitivities known.

Question 25

Describe Gout its causes, investigations, treatment and prevention

Answer 25

Gout typically presents with an acute monoarthropathy, with severe joint, inflammation, >50% occur at the MTP joint of the big toe (podagra). May affect other joints and may present as polyarthropathy. It is caused by deposition of monosodium urate crystals in and near joints and is associated with raised plasma urate. In the long term there may be deposition of urate as tophi in tendons and pinna of ear.

Causes: Hereditary, alcohol excess, diuretics, leukaemia, cytotoxics.

Investigations: Polarised light microscopy of synovial fluid shows negatively birefringent needle-shaped urate crystals.

Treatment: High dose NSAIDs or colchicine is slower but better if NSAIDs are contraindicated.

Prevention: weight loss, decrease alcohol intake, low-dose aspirin increase serum urate. If plasma urate increased 3 weeks after attack allopurinol may be used If more than 2 attacks in a year (SE include rash, fever, decreased WCC).

Question 26

Describe Psuedogout its risk factors, tests and management

Answer 26

Similar to gout, it causes an acute monoarthropathy, typically of larger joints and it is due to calcium pyrophosphate crystals.

Risk factors: Old age, hyperparathyroidism, haemochromatosis, hypophosphataemia.

Tests: polarised light microscopy of synovial fluid shows weakly birefrigent rhomboid-shaped crystals. It is associated with soft-tissue calcium deposition on radiographs.

Management: Aspiration, rest, intra-articular steroids. Methotrexate and hydroxychloroquine have a role in chronic psuedogout.

Question 27

What are the main types of Spondyloarthritides and name some of the common features

Answer 27

Types: Ankylosing Spondylitis, Enteric arthropathy, Psoriatic arthritis, reactive arthritis

Features:

• seronegativity (RF -ve)
• HLA B27 association
• Axial arthritis, pathology in spine and sacroiliac joints
• asymmetrical large joint oligoarthritis or monoarthritis
• enthesitis, inflammation of the site of insertion of tendon or ligament in to bone e.g plantar fasciitis, Achilles tendonitis, costochondritis
• dactyliitis inflammation of an entire digit due to soft tissue oedema and joint inflammation
• extra-articulate manifestations e.g. Anterior uveitis, psoriatic my rashes, oral ulcers, IBD.

Question 28

Describe Restless legs syndrome, its symptoms, associations, and management.

Answer 28

Symptoms: compelling desire to move legs, worse at night, and relieved by movement, unpleasant leg sensations worse at rest. More common in women.

Associations: iron deficiency, uraemia, pregnancy, DM, polyneuropathy, RA, COPD.

Management:

• exclude cramps, positional discomfort, and local leg pathology
• dopamine agonists are commonly used
• anticonvulsants, opioids and benzodiazepines may have a role e.g. Carbmazepine

Question 29

Describe Ankylosing Spondylitis its symptoms, investigations, and management.

Answer 29

A chronic sero-negative spondyloarthropathy which primarily involves the axial skeleton (I.e. Sacroilitis and spondylitis). Present earlier in men.

Symptoms: The typical patient is a man less than 30yrs old with gradual onset low back pain, worse at night, with spinal morning stiffness relieved by exercise. Pain radiates from sacroiliac joints to hips and buttocks, and usually improves by the end of the day. There is progressive loss of spinal movement. Other symptoms include enthesitis e.g. Plantar fasciitis, Achilles tendinitis.

Investigations: Clinical diagnosis supported by imaging. X-ray may show signs of sarcoilitis look for irregularities, erosions or sclerosis. Vertebral syndesmophytes are characterstic and calcification of ligaments can lead to bamboo spine. May also be associated with normocytic anaemia, raised ESR and CRP. HLA B27+VE

Management:

• Conservative I.e. Excercise, physio,
• Pharmocological I.e NSAIDs, TNFalpha blockers if severe and NSAIDs fail. Local steroid injections provide temporary relief.
• Sugrical I.e. hip replacement if hip involvement and rarely spinal osteoporosis.

Question 30

Describe Psoriatic Arthritis, it’s symptoms, investigations, and management.

Answer 30

A sero-negative spondyloarthropathy which occurs in 10-40% with psoriasis and can present before skin changes.

Symptoms: Paterns include symmetrical polyarthritis like RA, lone DIP joints, asymmetrical oligoarhthtis, spinal (similar to AS). Associated with nail changes in 80%.

Investigations: X-ray show erosive changes with pencil in cup
deformity in severe cases.

Management:
-NSAIDs, Sulfasalzine, methotrexate, ciclosporin, Anti-TNF if fail.

Question 31

Describe Fibromyalgia, its features, and it’s management

Answer 31

Fibromyalgia and chronic fatigue syndrome are part of a diffuse group of overlapping syndromes, sharing similar demographic and clinical characteristics, in which chronic symptoms of fatigue and widespread pain feature prominently.

Features: Diagnosis depends on pain that is chronic I.e. More than 3 months, and widespread I.e. Involves both left and right sides, above and below the waist and the axial skeleton, in the absence of inflammation, and the presence of pain on palpating of at least ‘11/18 tender points’. Additional features include morning stiffness (80%), fatigue (80%), poor concentration, low mood and sleep disturbance.

Management:

• first rule out more sinister causes e.g. RA, PMR, vasculitis, hypothyroidism, myeloma.
• explain that is a relapsing remitting syndrome, reassure there is no serious underlying pathology.
• CBT and graded exercise programmes improve function capacity and develop coping strategies.
• Pharmcological intervention include low dose amitriptyline 10-20mg at night, and pregabaln (150-300mg/12hr PO).

Question 32

Relapsing polychondritis

Answer 32

repeated episodes of inflammation and deterioration of cartilage. This most commonly affects the ears, however can affect other parts of the body such as the nose and joints.

Key features:
Ears: auricular chondritis, hearing loss, vertigo
Nasal: nasal chondritis
Ocular: episcleritis, scleritis, iritis, and keratoconjunctivitis sicca
Joints: arthralgia
Less commonly: cardiac valcular regurgitation, cranial nerve palsies, peripheral neuropathies, renal dysfunction

Diagnosis:
Various scoring systems based on clinical, pathological, and radiological criteria

Treatment
Induce remission: steroids
Maintenance: azathioprine, methotrexate, cyclosporin, cyclophosphamide