Polymyalgia Rheumatica

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Polymyalgia Rheumatica Temporal Arteritis

• Anna Mae Smith, MPAS,
• PA-C

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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

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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 (21)

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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
  

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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

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RISK FACTORS

• Age greater than 50
• Presence of giant cell arteritis

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Differential Diagnosis

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

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Differential Diagnosis

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

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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

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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
  

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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.

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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)
  

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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)

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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

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Henoch-Schonlein Purpura
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Definition

• A vasculitis of small vessels characterized by
  nonthrombocytopenic, usually dependent, palpable
  purpura, arthritis, abdominal pain and nephritis
  

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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 (21)

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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
  

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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
  

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SIGNS Symptoms

• Two-thirds of patients experience arthritis
• Large joints (knees and ankles) are most commonly
  involved
  

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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

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SIGNS Symptoms

• Renal involvement is less common
• Hematuria, with or without casts or proteinuria

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SIGNS Symptoms

• Other manifestations
• Seizures, neuropathies
• Hepatosplenomegaly
• Lymphadenopathy
• Cardiac involvement
• Pulmonary hemorrhage
• Rheumatoid-like nodules

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DIFFERENTIAL DIAGNOSIS

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

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DIFFERENTIAL DIAGNOSIS

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

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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
  

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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.

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COMPLICATIONS

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

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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