Polymyalgia Rheumatica (PMR) Temporal Arteritis (TA)

Polymyalgia Rheumatica (PMR)

Temporal Arteritis (TA)

Diagnostic Criteria- PMR (healey 1984)

1. Age> 50 yrs

2. Pain corresponding to proximal muscles of the limbs. 2 out of 3 regions: neck, shoulder & pelvic girdle. Symptoms> 1 month

3. Morning stiffness> 1 hr

4. Elevated ESR (>40 mm/hr)

5. Rapid response to low dose of steroids

Diagnostic Criteria- TA (ACR 1990)

1. Age> 50 years

2. Recent localized headache

3. Temporal artery abnormality

4. Elevated ESR

5. Temporal biopsy positive for vasculitis

* Diagnosis ≥ 3

Epidemiology

Central+north of Europe, north America

Mainly Caucasian

Women>men X2-3

Older age 90%> 60 years

Annual prevalence: TA: 6.7/105

PMR: 53/105

Etiology

Genetic tendency (familial+ethnic). HLA-DR 4

Infectious agent? (Clusters of cases+annual variation)

Immune response involving 2 processes:

a. An immune response with presence of active

mononuclear cells in peripheral blood (IL-6) +systemic

symptoms.

b. Granulomatotic inflammation with involvement of CMI

against Ag in the inner elastic membrane or near

Pathology

1. Lymphocytic infiltration (CD4) near the elastica

2. Thickening of the intima without prominent infiltration

3. Massive infiltration: lymphocytes+macrophages+giant cells+granulomas

4. Necrosis of the arterial wall

5. Thrombosis of blood vessels in inflamed areas

6. Recanalization

Arteries Involved:

- Superficial temporal, Vertebral, Posterior Ciliary, Ophtalmic

- Central retinal, Carotid, Subclavian, Brachial, Aorta,

Clinical Features

The onset usually insidious

I. Constitutional symptoms

II. TA

III. PMR

I. Constitutional Symptoms

1. Fatigue, depression

2. Anorexia, weight loss (50%, 6 Kg)

3. Fever (50%)

15% of cases with FUO age>65 yrs TA

II. Temporal Areteritis

1. Headache- often localized to temples or occiput (2/3).

On physical exam: Nodular, thickened, tender vessel +absent or weak pulse

2. Ophthalmic manifestation: 20-50%.

a. Diplopia

b. Ptosis

c. Visual loss: 6-10%. Usually sudden, painless and permanent.

Fundus: Anterior>Posterior ischemic optic neuropathy

II. Temporal Areteritis

3. Neurological manifestation: 30%

a. Peripheral neuropathy

b. Involvement of carotid or vertebrobasilar TIA, CVA

4. Jaw claudication

5. Sore throat

6. Hoarseness

II. Temporal Areteritis

7. Occlusion of large as: 10-15%.

a. Claudication of the hands.

On exam: Bruits + weak pulses

b. Raynaud phenomenon

c. Aneurysm, dissection of thoracic aorta- late

III. Polymyalgia Rheumatica

1. Arthralgia/myalgia. Usually starts in neck, shoulder

+pelvic girdle

Symetrical

Infrequent distal involvement

On exam: muscle strength usually unimpaired

2. Morning stiffness

3. Night pains

4. Tenderness of periarticular structures: capsule ,bursa, tendon

5. +Mild & transient synovitis usually knees, wrists, SCJ

Relationship between PMR & TA

1. Similar Age & sex & ethnic distribution

2. In both + systemic features

3. 10-15% of PMR have positive temporal biopsies

4. Good response to steroids

5. 40-60% of TA patients +PMR

6. 20-40% of TA patients PMR is the presenting symptoms

* PMR may precede, coincide with or follow TA

Investigations

1. ESR

2. Anemia-normocytic, mild to moderate

3. ILFT- 30%, ALP (70%)

4. RF, ANA- negative

5. Doppler of temporal as

6. CT, MRI if involvement of large as is suspected

7. Temporal biopsy

Differential Diagnosis

1. Malignancy

a. Solid tumors

b. Hematological malignancy: MM, Leukemia, Lymphoma

2. Joint diseases

a. OA, especially +cervical involvement

b. RA

c. Collagen vascular diseases

Differential Diagnosis

3. Muscles diseases

a. Polymyositis

b. Myopathy

4. Chronic infections

a. SBE

b. osteomyelitis

5. Hypothyroidism

6. Parkinson disease

7. Functional

Treatment

1. Steroids

a. Initial dose

PMR: 10-20 mg of prednisone per day

TA: 40-60 mg per day

+Eye involvement: Pulse IV methylprednisolone 1 gr X 3 ds

b. Gradual decease of steroids dose

2. Steroid sparing agents: Methotrexate