Polymyalgia Rheumatica &Temporal Arteritis

Polymyalgia Rheumatica &Temporal Arteritis
Presentation by: 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

o Onset - abrupt or insidious
o Pain and stiffness shoulder and hip girdle
o Usually symmetrical
o Symptoms more common in the morning
o Gel phenomena (stiffness after prolonged inactivity)
o Constitutional symptoms - fatigue, malaise, depression, weight loss, low grade fever
o Arthralgias/arthritis (non inflammatory)
o No weakness (pain may limit strength)
o Muscle tenderness mild to moderate
o No muscle atrophy
o Decreased range-of-motion of joints on active motion usually due to pain
o May have signs and symptoms of giant cell arteritis

RISK FACTORS

o Age greater than 50
o Presence of giant cell arteritis

Differential Diagnosis

o Rheumatoid arthritis
o Other connective tissue disease
o Fibromyalgia
o Depression
o Polymyositis/dermatomyositis (check CPK, aldolase)
o Thyroid disease
o Viral myalgia
o Osteoarthritis
o Occult infection
o Occult malignancy (extensive search usually not necessary)
o Myopathy (steroid, alcohol, electrolyte depletion)

LABS

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

TREATMENT
Patient Follow -up
PROGNOSIS

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

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

SIGNS & Symptoms

o Onset can be acute or gradual
o 50% of patients have malaise and low grade fever
o 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
o Lesions blanch on pressure but later become petechial or purpuric
o Lesions appear in crops
o Angioedema of scalp, lips, eyelids, ears, dorsa of hands and feet, back, scrotum and perineum may be seen
* Two-thirds of patients experience arthritis:
o Large joints (knees and ankles) are most commonly involved
* One-half of patients experience GI symptoms:
o Colicky abdominal pain associated with vomiting is most common
o Occult or gross blood in stool
o Hematemesis
o Intussusception, obstruction or infarction rarely occurs
o Pancreatitis
* Renal involvement is less common
o Hematuria, with or without casts or proteinuria
* Other manifestations
o Seizures, neuropathies
o Hepatosplenomegaly
o Lymphadenopathy
o Cardiac involvement
o Pulmonary hemorrhage
o Rheumatoid-like nodules

DIFFERENTIAL DIAGNOSIS

o Hemorrhagic diathesis
o Septicemia
o Intussusception
o Acute appendicitis
o Acute glomerulonephritis
o Familial IgA nephropathy
o Polyarteritis nodosa
o Systemic lupus erythematosus
o Inflammatory bowel disease
o Subacute bacterial endocarditis
o Rocky Mountain spotted fever
o Thrombocytopenic purpura
Labs

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

TREATMENT
COMPLICATIONS

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

PROGNOSIS

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

Polymyalgia Rheumatica &Temporal Arteritis.ppt