Polymyalgia Rheumatica (PMR) Temporal Arteritis (TA)
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Transcript of 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