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Lecturer: Craig Payne

This lecture covers:

* Polymyalgia rheumatica

* Vasculitis

* Haemophilic arthropathy

* Relatively common clinical syndrome associated with ‘aching’ in the elderly – self limiting.

* Affects 1/200-1000 over the age of 50 (very rare <50yrs).

* F>M.

* Closely associated with temporal/giant cell arteritis where they are both possibly on the same continuum.

Characterised by severe pain/aching and stiffness in proximal muscle groups (shoulders and hips).

Aetiology:
* Unknown. Strong genetic predisposition (HLA-DR4), but no immune abnormalities have been detected yet.

Clinical features:
- More common in elderly Caucasian woman.

- Onset may be acute or subacute/insidious – initial diagnosis may take several months.
- Severe pain/disability and stiffness in neck, pectoral and pelvic girdles (symmetrical and bilateral) – usually insidious in onset; morning stiffness; stiffness after inactivity; poorly localised tenderness over joints (especially hips and shoulders); pain at night - may awaken patient; muscle strength is unaffected; malaise; fever; depression; fatigue; weight loss; raised alkaline phosphatase and ESR.
- Up to 50% may have another diagnosed rheumatological condition --> PMR may go unrecognised or undiagnosed.
- Diagnosis is based on history and clinical examination (laboratory tests will rule out other causes of symptoms).

Treatment:
- Reassurance and education.
- Usually follows a benign course ? most complete resolution within 2 years, but some develop giant cell arteritis (could be up to 30%). ESR can be used to monitor progress and response to treatment.
- NSAID’s in first 4 weeks – only effective in up to 20%
- Low dose prednisone is usual drug of choice – immediate and dramatic response to small dose is often considered to be diagnostic.
- Physiotherapy – especially range of motion exercises; strengthening weakened muscles; management of unsteady gait.

Online Resources:

ePodiatry's links to polymyalgia rheumatica

Vasculitis

(Inflammatory Vascular Diseases/Necrotising vasculitis(polyarteritis nodosa)

* Uncommon, heterogeneous group of conditions characterised by immune mediated inflammation of blood vessels.

* Clinical features will vary depending on site and size of the involved vessels. Most common clinical presentation is a palpable purpura (non-blanchable, raised redness/erythema) – indicates extravasation of red blood cells.

* Most have musculoskeletal manifestations, but they are often overshadowed by involvement of other organ systems.

* Terminology in literature can be confusing and classification is difficult.

Vasculitis --> inflammation of arteries, veins and capillaries
Arteritis --> inflammation of arteries and arterioles

Temporal Arteritis/Giant Cell Arteritis/Cranial arteritis/Horton’s headache:
• inflammatory vascular syndrome that affects mainly the cranial arteries in those >50yrs (10x more common in those >80yrs); F>M; higher incidence in populations of Nordic origin (Scandinavians); HLA and familial studies are not clear on genetic role in aetiology; previous periods of infection have been implicated
• Usually abrupt onset (may be insidious) with headache, jaw & tongue claudication, ear canal pain, loss of vision
• Have similar features to polymyalgia rheumatica. Polymyalgia may be prodromal manifestation of temporal arteritis, but nature association is not fully understood
• Predilection for superficial temporal arteries, but can affect extracranial circulation (eg aortic arch syndrome; claudication of extremities)
• involvement of lower limb is rare with a number of cases described (LeHello et al, 2001)
• arthralgias due to synovitis in up to 10%
• American College of Rheumatology criteria for diagnosis is when 3 of the 5 criteria are present (new symptoms after age of 50; new headache; temporal artery abnormality; elevated ESR; abnormal artery biopsy)
• Self-limiting – up to 12 months, but some may have residual ocular damage.
• high dose corticosteroids main approach to treatment – usually dramatic effect within 72hrs

Aortic arch/Takayasu arteritis/Pulseless disease:
• more common in Japan and Mexico (rare in Europe and North America)
• usually young woman (average 10-30yrs) presenting with prolonged period of malaise, fever and weight loss
• later develop vascular headache, visual symptoms and upper extremity claudication. May have CVA
• 50% have arthralgias
• due to inflammation and granulomatous necrotising arteritis of aortic ? ischaemia.
• American College of Rheumatology criteria for diagnosis is when 3 of 6 criteria are present (age of onset <40 years; claudication of extremities; decreased brachial pulse; blood pressure difference between arms of > 10mmHG; bruit over subclavian arteries or aorta; abnormality on arteriogram)
• corticosteroids are effective in early stages. Later may need anticoagulants or vascular surgery

Wegener Granulomatosis (WG):
• predominantly affects small arteries
• granulomatosis arteritis of upper and lower respiratory tracts associated with glomerulonephritis in kidneys and a necrotising vasculitis
• prevalence of around 3-8.5/1 000 000. Most white. M=F.
• fever, weight loss, malaise, respiratory symptoms (sinusitis, haemoptysis, chest pain, nasal discharges), nondestructive painful arthritis, peripheral neuropathy (mononeuritis multiplex and symmetric distal polyneuritis), ulcerative skin lesions, ocular problems
• American College of Rheumatology criteria for diagnosis is when 2 of 4 criteria is present (nasal or oral inflammation; abnormal chest x-ray; red cells in urine; granulomatous lesions on biopsy)
• Arthritis – in up to 75%; various patterns of joint involvement have been described; usually symmetrical polyarthritis, non-deforming and non-erosive.
• over half of those with WG have been reported to have peripheral neuropathy signs present before the WG was diagnosed (de Groot et al, 2001)
• prognosis is poor due to renal and respiratory problems, but early treatment improves prognosis
• treatment – corticosteroids, cyclophosphamide and immunosuppressives

Churg-Strauss vasculitis/allergic (eosinophilic) granulomatosis vasculitis:
• systemic necrotising vasculitis in those with pre-existing allergic rhinitis, sinusitis or asthma – affects small and medium sized arteries of, most often, the skin, peripheral nerves and lungs.
• M>F. Onset ages 20 to 40.
• main pathologic features are necrotising vasculitis, tissue infiltration with eosinophilia and extravascular granuloma formation
• present with worsening respiratory symptoms
• course is variable
• American College of Rheumatology criteria for diagnosis is when 4 of 6 symptoms are present (asthma, eosinophilia, neuropathy, pulmonary infiltrates, paranasal sinus abnormality, extravascular eosinophils)
• joint symptoms in 50% - polyarticular and migratory – joints swollen and effusions.
• Peripheral neuropathies are common

Polyarteritis nodosa (PAN):
• sometimes, historically, used as generic name for this group of vasculitis conditions
• widespread necrotising inflammatory arteritis ? can lead to life threatening multisystem disease.
• M 2-3x>F. Annual incidence of 5-10/1 000 000. Onset usually 40-60yrs.
• affects small and medium size arteries of skin, kidney, peripheral nerves, muscle and stomach – usually young men with no underlying disease
• often abrupt onset with fever, weight loss, joint pain (50-75%; similar to rheumatoid arthritis), skin lesions – palpable purpura/nodules (along course of artery), ulcerations and infarctions (25-50%), neurologic symptoms (includes a symmetric sensory and motor polyneuropathy), hypertension, renal disease (25%-80%)

Henoch-Schonlein purpura/Anaphylactoid purpura:
• disease of childhood (could also occur in adults) – consists of vasculitis limited to skin, gastrointestinal tract and kidneys that usually follows an upper respiratory tract infection
• abrupt onset – flu like symptoms. Within days --> purpura over tibia, ankles and feet; abdominal pain and GI bleeding.
• 75% develop joint symptoms - mostly knees and ankles – occasionally small joints of hands and feet – usually bilateral and symmetrical – usually only lasts up to a week
• usually self limiting – 80% make full recovery in 6 to 16 weeks
• NSAID’s may help

Online resources:

ePodiatry's online resources on vasculitis

Haemophilic arthropathy

• common feature of haemophilia – more common in knee, ankle and elbow
• haemorrhage induces a synovial proliferation, chronic inflammation --> release of degradative proteinases --> joint damage. Recurrent bleeding --> irreversible damage

• Stage 1 – acute haemoarthrosis in child when begin to walk – pain, tenderness, warmth, limited range of motion, distended joint capsule. Often have prodromal symptoms of stiffness and warmth in the joint.
• Stage 2 – subacute or chronic arthritis – repeated haemorrhage --> self perpetuating chronic proliferative synovitis; haemosiderin accumulates in joint tissues; cartilage is degraded – joint is chronically swollen, warm but not as painful as acute – range of motion is decreased; crepitus present; muscles atrophied ? may have significant disability
• Stage 3 – ‘end-stage arthropathy’ - chronic destructive arthropathy with joint instability, fibrous ankylosis, osteophytic overgrowth
• develop secondary osteoarthritis (from cartilage degradation) and increased risk for septic arthritis (consider infection if haemoarthrosis fails to respond after factor replacement)

X-ray – in acute stage --> only see soft tissue swelling and effusions. Later --> radiodense effusions (due to haemosiderin accumulating in synovial membrane), osteoporosis, bone erosions, joint space narrowing (from denudation of cartilage)

Treatment – medical management of haemophilia – need prompt replacement of deficient factor; ice packs; joint immobilisation in extended position (within tolerance); joint aspirations; analgesics; patient education on strategies for prevention of trauma.
Long term – exercises/braces to improve joint stability, muscle strengthening, synovectomy (if synovitis unresponsive to pharmacological approaches). Joint arthroplasty if end stage.

Functional foot orthoses have been should to be very effective in reducing the ankle pain associated with haemophilic arthropathy (Slattery & Tinley, 2001)

Online resources:

ePodiatry's links to other rheumatological diseases