POLYMYALGIA RHEUMATICA (PMR)

•PMR is a clinical syndrome of muscle pain and stiffness with an increased ESR.

• It is not a true vasculitis but there is a close association with giant cell arteritis.• The prevalence is approximately 20 per 100 000 over the age of 50. •The mean age of onset is 70. •Women are affected more often than men in a ratio of 3:1.

Clinical features of PMR• The cardinal features are muscle stiffness and pain,

symmetrically affecting the proximal muscles of the neck, upper arms and, less commonly, the buttocks and thighs.

• There is marked early morning stiffness, often with night pain.

• Constitutional features of weight loss, fatigue, depression and night sweats also occur.

• On examination there may be stiffness and painful restriction of active shoulder movement but passive movements are preserved.

• Muscles may be tender to palpation but there should not be muscle-wasting; if there is, then primary muscle or neurological disease is more likely

CONDITIONS THAT MAY MIMIC POLYMYALGIA RHEUMATICA

• Fibromyalgia • Hypothyroidism • Cervical spondylosis • Rheumatoid arthritis • Inflammatory myopathy (particularly inclusion

body myositis) • Systemic vasculitis • Malignancy

Investigations in PMR

• ESR is elevated above 40 mm/hour In the majority of patients

• Very occasionally the ESR is low, usually in the acute situation where there has not been sufficient time for it to rise. In this situation the CRP may be elevated prior to the ESR.

• There may be a normochromic, normocytic anaemia

Management of PMR

• The only effective treatment is corticosteroids.• prednisolone should be started at a dose of 15 mg daily.

• The majority of patients should have a dramatic response within 72 hours.

• If there is no response by 72 hours or an incomplete response by 7 days, then the diagnosis is not PMR.

• If there has been a good response to prednisolone, the daily dose should be reduced to 10 mg after 4 weeks and then by 1 mg per month, assuming that symptoms remain controlled.

Most patients need steroids for an average of 12-18 months and osteoporosis prophylaxis with bisphosphonates should be considered.

Some patients require steroid-sparing agents such as methotrexate or azathioprine, particularly if prednisolone cannot be withdrawn at 2 years or is needed at doses greater than 7.5 mg daily.

Approximately 15-20% of patients develop features of

giant cell arteritis at some point in the course of their disease.

TAKAYASU'S ARTERITIS

• Takayasu's disease is a chronic inflammatory granulomatous panarteritis of elastic arteries.

• The vessels most commonly involved are the aorta and its branches, and the carotid, ulnar, brachial, radial and axillary arteries. Pulmonary arteries are occasionally affected.

• It is more common in women (female:male ratio 8:1) with a typical onset at the age of 25-30 years.

• It has a world-wide distribution but is most common in Asia. • The aetiology is unknown.

• .The usual presentation is with claudication and systemic symptoms of fever, arthralgia and weight loss.

• Clinical examination may reveal loss of pulses, bruits hypertention and aortic incompetence.

• Laboratory investigations are usually non-specific, with high ESR and normocytic, normochromic anaemia.

• Diagnosis is usually based on angiographic findings of coarctation, occlusion and aneurysmal dilatation.

Diagnostic Criteria for TA

1-Age less than 40 years.2-Claudication of extremeties.3-Decreased brachial artery pulse.4-BP difference more than 10 mmHg between arms.5-Bruit over subclavian arteries & aorta.6-Arteriogram abnormalities: occlution or narrowing in aorta or main branches.

Must have 3\6 criteria for diagnosis.

Treatment• Most patients respond to initial high-dose oral prednisolone (1-2 mg/kg daily). • Additional therapy with methotrexate or cyclophosphamide is

usually required.

• Reconstructive vascular surgery should be avoided during periods of active inflammation but may benefit selected patients, especially those with hypertension secondary to aortic or renal lesions

• The 5-year survival rate is 80%.∼

Takayasu’s is LARGE Vessel Dz

Medium – Sized Arteritis1- Classical polyarteritis nodosa.2- Kawasaki disease.

CLASSICAL POLYARTERITIS NODOSA (PAN)

• Classical PAN is a necrotising vasculitis characterised by transmural inflammation of medium-sized to small arteries.

• PAN is a rare disorder with an annual incidence of 2 per million in most populations.

• All age groups can be affected, with a peak incidence in the fourth and fifth decades, and a male:female ratio of 2:1.

• Hepatitis B is a risk factor, and the incidence of PAN is higher in the areas, where hepatitis B infection is endemic

Clinical presentation is with myalgia, arthralgia, fever and weight loss in combination with manifestations of multisystem disease.

• The most common skin lesions are palpable purpura, ulceration, infarction and livedo reticularis .

• In 70% of patients arteritis of the vasa nervorum leads to neuropathy which is typically symmetrical and affects both sensory and motor function.

• Severe hypertension and/or renal impairment may occur due to multiple renal infarctions.

glomerulonephritis is rare .

• Diagnosis is confirmed by finding multiple aneurysms and smooth narrowing of either the mesenteric, hepatic or renal systems on angiography.

• Tissue biopsy may be definitive (muscle or sural nerve), even in the absence of angiographic abnormality.

Treatment• Treatment for hepatitis B-related disease is to remove the

source of the antigen, i.e. antiviral therapy. • Corticosteroids and cyclophosphamide are the treatment of

choice for idiopathic disease. • Mortality is less than 20%, although relapse occurs in up to

50% of patients

Severe Skin Ulceration in PAN

Polyarteritis NodosaMicro “Berry” aneurysms

Kawasaki Disease

• Kawasaki disease is an acute systemic disorder of childhood that predomintely occurs in Japan( 800 cases per million in children under the age of 5 ).

• The disease resembles a viral exanthem or stevens – Johnson syndrome.

• Although the causative trigger is unknown, it has been associated with Mycoplasma and HIV infection in some cases.

• The clinical features often develop abruptly.

Features of Kawasaki Disease*

• Fever persisting > 5 days• Bilateral conjunctival congestion• Erthema of lips, buccal mucosa and tongue• Acute non-purulent cervical lymphadenopathy• Polymorphous exanthema• Erythema of palms and soles(oedema followed by

desquamation)• Coronary dilatation* Five out of six clinical features, or four out of six clinical

features with evidence of Coronary dilatation, are required for diagnosis.

• Cardiovascular complications include myocarditis, pericarditis, coronary aneurysms, transient coronary artery dilatation, myocardial infarction due to coronary thrombosis.peripheral vascular insufficieny and gangerene.

• Investigations that favour KD include :• polymorphonuclear leucocytosis, thrombocytosis, raised ESR

and CRP and circulating antiendothelial cell antibodies.

• Treatment is with aspirin (5 mg/kg daily for 14 days) and intravenous gammaglobulin (400 mg/kg daily for 4 consecutive days).

• Steriods should be avoided because of the risk of worsening the coronary artery dilatation .

• coronary artery changes are usually monitored weekly by two-dimensional echo for 4 weeks, by which stage most children have recovered.

• The overall mortality is less than 2%• Relapse is rare , but if there is coronary artery involvement

long –term follow –up is necessory.