Polymyalgia Rheumatica

&Temporal Arteritis
Anna Mae Smith, MPAS,
PA-C
Polymyalgia Rheumatica
A clinical syndrome characterized by
aching and stiffness of the shoulder and
hip girdle muscles affecting older patients,
associated with an elevated ESR, lasting
over 1 month and responsive to low dose
steroids

Epidemiology
Incidence/Prevalence in USA:
Approximately 50/100,000 patients over
age 50/year
Predominant age: 60 or older.
Incidence increases with age (rare under
50 years old).
Predominant sex: Females > Male (2:1)

Signs & Symptoms
Onset - abrupt or insidious
Pain and stiffness shoulder and hip girdle
Usually symmetrical
Symptoms more common in the morning
Gel phenomena (stiffness after prolonged
inactivity)
Constitutional symptoms - fatigue, malaise,
depression, weight loss, low grade fever

Signs & Symptoms
Arthralgias/arthritis (non inflammatory)
No weakness (pain may limit strength)
Muscle tenderness mild to moderate
No muscle atrophy
Decreased range-of-motion of joints on
active motion usually due to pain
May have signs and symptoms of giant cell
arteritis
RISK FACTORS
Age greater than 50
Presence of giant cell arteritis

Differential Diagnosis
Rheumatoid arthritis
Other connective tissue disease
Fibromyalgia
Depression
Polymyositis/dermatomyositis (check CPK,
aldolase)
Thyroid disease
Viral myalgia
Osteoarthritis

Differential Diagnosis
Occult infection
Occult malignancy (extensive search usually
not necessary)
Myopathy (steroid, alcohol, electrolyte
depletion)

LABS
ESR (Westergren) elevation greater than 50
Anemia - normochromic/normocytic
Creatine phosphdkinase (CPK)- normal
Rheumatoid factor (RF) - negative (5-10%
patients over 60 will have positive RF without
disease)
Mild elevations in liver function tests

TREATMENT
Physical therapy for range-of-motion
exercises if necessary
Do not over exercise to cause exertion
Precautions regarding steroid use
Instruct the patient about symptoms of giant
cell arteritis and to report them immediately

TREATMENT - Prednisone
10 mg/day initially (average initial affective
dose 10-15 mg/d)
Usually dramatic (diagnostic) response.
May increase gradually to 20 mg if no
response
Begin slow taper at 4-6 weeks by only 1 mg
every 1-4 weeks to a dose of 5-7.5 mg.
Continue at this dose for approximately 18
months to 2 years, if no recurrence of
symptoms.

TREATMENT - Prednisone
Then attempt to taper by 1 mg every 2-4
weeks until drug discontinued. Patient may,
however, require steroids for 3 or more
years.
Increase prednisone for recurrence of
symptoms (relapse common)

Patient Follow -up
Follow monthly initially and during taper of
medication, every 3 months otherwise
Follow ESR as steroids tapered
Followup with patient for symptoms of giant
cell arteritis. Educate patient to report such
symptoms immediately (headache, visual and
neurologic symptoms)

PROGNOSIS
Average length disease is 3 years (range 1-5
years)
Exacerbation if steroids tapered too fast
Prognosis very good if treated (may
gradually remit even if no treatment)
Relapse common

Henoch-Schonlein Purpura
Definition
A vasculitis of small vessels characterized
by nonthrombocytopenic, usually
dependent, palpable purpura, arthritis,
abdominal pain and nephritis

Epidemiology
Incidence/Prevalence in USA:
Incidence 14/100,000 in 2-14 year old age
range. Seasonal variation - more common
in winter.
Predominant age: Most occur between
2-8 years old, but can occur at any age
Predominant sex: Male > Female (2:1)


SIGNS & Symptoms
Onset can be acute or gradual
50% of patients have malaise and low grade
fever
Skin lesions occur in all identified patients:
Lesions appear on lower extremities and buttocks
but may involve face, trunk and upper extremities
Begin as small wheals or erythematous
maculopapular

SIGNS & Symptoms
Lesions blanch on pressure but later become
petechial or purpuric
Lesions appear in crops
Angioedema of scalp, lips, eyelids, ears,
dorsa of hands and feet, back, scrotum and
perineum may be seen


SIGNS & Symptoms
Two-thirds of patients experience
arthritis:
Large joints (knees and ankles) are most
commonly involved


SIGNS & Symptoms
One-half of patients experience GI
symptoms:
Colicky abdominal pain associated with
vomiting is most common
Occult or gross blood in stool
Hematemesis
Intussusception, obstruction or infarction
rarely occurs
Pancreatitis

SIGNS & Symptoms
Renal involvement is less common
Hematuria, with or without casts or
proteinuria

SIGNS & Symptoms
Other manifestations
Seizures, neuropathies
Hepatosplenomegaly
Lymphadenopathy
Cardiac involvement
Pulmonary hemorrhage
Rheumatoid-like nodules

DIFFERENTIAL DIAGNOSIS
Hemorrhagic diathesis
Septicemia
Intussusception
Acute appendicitis
Acute glomerulonephritis
Familial IgA nephropathy
Polyarteritis nodosa

DIFFERENTIAL DIAGNOSIS
Systemic lupus erythematosus
Inflammatory bowel disease
Subacute bacterial endocarditis
Rocky Mountain spotted fever
Thrombocytopenic purpura

Labs
Not diagnostic
Sedimentation rate, white blood cell count
may be elevated
Coagulation studies, platelet count and
complement determinations are normal
Serum IgA elevated in 50%
Urinalysis shows protein, red blood cells,
white blood cells if renal

TREATMENT
Anti-inflammatory agents may be used for
arthritis and fever
Corticosteroids (prednisone 1-2 mg/kg/day)
for severe GI symptoms and/or painful
angioedema. Corticosteroids do not alter the
progression of the lesions.

COMPLICATIONS
Hypertension
Renal failure
Intestinal hemorrhage
Bowel obstruction or perforation
Death very rare

PROGNOSIS
Disease may last for a few days with
transient arthritis; however, in many cases,
the average duration is 4-6 weeks
Occasionally recurrent
25% of patients with initial renal involvement
will have persistently abnormal urine
sediment