polymyalgia.h s

8/11/2019 Polymyalgia.H S

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Polymyalgia Rheumatica

A clinical syndrome characterized byaching and stiffness of the shoulder and

hip girdle muscles affecting older patients,associated with an elevated ESR, lastingover 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/yearPredominant age:60 or older.

Incidence increases with age (rare under

50 years old).Predominant sex:Females > Male (2:1)


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Signs & Symptoms

Onset - abrupt or insidious

Pain and stiffness shoulder and hip girdle

Usually symmetricalSymptoms 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 moderateNo muscle atrophy

Decreased range-of-motion of joints on

active motion usually due to painMay 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

FibromyalgiaDepression

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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TREATMENT

Physical therapy for range-of-motionexercises if necessary

Do not over exercise to cause exertionPrecautions 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 affectivedose 10-15 mg/d)

Usually dramatic (diagnostic) response.

May increase gradually to 20 mg if noresponse

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 18months to 2 years, if no recurrence of

symptoms.


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TREATMENT - Prednisone

Then attempt to taper by 1 mg every 2-4weeks until drug discontinued. Patient may,however, require steroids for 3 or moreyears.

Increase prednisone for recurrence ofsymptoms (relapse common)


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Patient Follow -up

Follow monthly initially and during taper ofmedication, every 3 months otherwise

Follow ESR as steroids tapered

Followup with patient for symptoms of giantcell arteritis. Educate patient to report suchsymptoms immediately (headache, visual and

neurologic symptoms)


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PROGNOSIS

Average length disease is 3 years (range 1-5years)

Exacerbation if steroids tapered too fast

Prognosis very good if treated (maygradually remit even if no treatment)

Relapse common


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Henoch-Schonlein Purpura


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Definition

A vasculitis of small vessels characterizedby 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 commonin winter.

Predominant age:Most occur between

2-8 years old, but can occur at any agePredominant sex:Male > Female (2:1)


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SIGNS & Symptoms

Onset can be acute or gradual

50% of patients have malaise and low grade

feverSkin lesions occur in all identified patients:

Lesions appear on lower extremities and buttocksbut may involve face, trunk and upper extremities

Begin as small wheals or erythematousmaculopapular


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SIGNS & Symptoms

Lesions blanch on pressure but later becomepetechial or purpuric

Lesions appear in crops

Angioedema of scalp, lips, eyelids, ears,dorsa of hands and feet, back, scrotum andperineum may be seen


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SIGNS & Symptoms

One-half of patients experience GIsymptoms:

Colicky abdominal pain associated withvomiting is most common

Occult or gross blood in stool

Hematemesis

Intussusception, obstruction or infarctionrarely 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

HepatosplenomegalyLymphadenopathy

Cardiac involvement

Pulmonary hemorrhageRheumatoid-like nodules


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DIFFERENTIAL DIAGNOSIS

Hemorrhagic diathesis

Septicemia

IntussusceptionAcute appendicitis

Acute glomerulonephritis

Familial IgA nephropathy

Polyarteritis nodosa


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DIFFERENTIAL DIAGNOSIS

Systemic lupus erythematosus

Inflammatory bowel disease

Subacute bacterial endocarditisRocky Mountain spotted fever

Thrombocytopenic purpura


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Labs

Not diagnostic

Sedimentation rate, white blood cell count

may be elevatedCoagulation 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 forarthritis and fever

Corticosteroids (prednisone 1-2 mg/kg/day)for severe GI symptoms and/or painfulangioedema. Corticosteroids do not alter theprogression of the lesions.


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COMPLICATIONS

Hypertension

Renal failure

Intestinal hemorrhageBowel obstruction or perforation

Death very rare


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PROGNOSIS

Disease may last for a few days withtransient arthritis; however, in many cases,the average duration is 4-6 weeks

Occasionally recurrent

25% of patients with initial renal involvementwill have persistently abnormal urine

sediment

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