Atypical polymyalgia rheumatica
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Atypical Polymyalgia Rheumatica. A Case Report.

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Atypical Polymyalgia Rheumatica

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Atypical polymyalgia rheumatica

Atypical PolymyalgiaRheumatica


A case report

A Case Report

  • NG is a 47 year old female who developed severe shoulder and upper arm pain bilaterally as well as hip pain , wrist pain and severe morning stiffness in March 2011. She denied ever experiencing anything like this before. She did admit to losing 30 pounds intentionally after completing the HCG diet one month prior.


Atypical polymyalgia rheumatica

  • She denied fever, night sweats, rash, mucosal ulcers, strenuous activity, visual changes, jaw claudication, small joint pain, swelling and respiratory symptoms.


Review of systems

Review of Systems

  • General : see above

  • HEENT: no new headache

  • Endocrine: no cold intolerance, polydipsia or polyuria

  • Hematologic: no bruising or bleeding

  • Skin: no rash

  • Respiratory : no cough or dyspnea


Ros continued

ROS Continued

  • Cardiovascular: No chest pain, palpitations, or claudication

  • Gastrointestinal: No nausea, vomiting, diarrhea , constipation, or change in stools

  • Genitourinary: No change in menses, dysuria, frequency, hematuria, or foamy urine

  • Musculoskeletal: no active synovitis , but severe pain with ROM of shoulders


Rom continued

ROM Continued

  • Neurologic: No motor weakness, numbness, paresthesias, cognitive symptoms

  • Psychiatric: recent discontinuation of Zoloft which she had used since 2004


Allergies

Allergies

  • None


Medications

Medications

  • HCTZ 25 mg po daily

  • Metoprolol 12.5 po BID

  • KCL 10 meqpo daily

  • Recent HCG injections completed one month ago

  • Ibuprofen 200mg po QID


Past medical history

Past Medical History

  • Hypertension

  • Depression

  • Migraine

  • Obesity


Past surgical history

Past Surgical History

  • Knee surgery bilaterally

  • Cholecystectomy

  • Foot surgery

  • Breast cyst aspiration


Social history

Social History

  • No smoking

  • Rare social alcohol

  • No illicit drug abuse

  • Lesbian

  • Works as a manager at Petsmart


Family history

Family History

  • Unknown ( Adopted )


Physical examination

Physical Examination

  • Vital signs: BP 131/89, Temp 97.6, Weight 201.8, Height 5 feet 2 inches

  • Head : normal, No temporal artery tenderness

  • Eyes: normal

  • ENT: normal

  • Lymphatic : no nodes

  • Skin: normal


Pe continued

PE Continued

  • Chest: Clear to A & P

  • Cardiovascular : Normal ( no murmurs or rubs and pedal pulses normal)

  • Abdomen : obese ,non tender , no mass, or organomegaly

  • Back: No significant findings

  • Neurologic : normal


Pe continued1

PE Continued

  • Musculoskeletal : Severe pain with ROM of the shoulders and hips bilaterally. Some tenderness of the wrists but no active synovitis, and no synovitis of the hands, or feet, or knees


Laboratory

Laboratory

  • CMP : normal except AST 64

  • Magnesium : normal 2.2

  • Phosphate : normal 3.6

  • CBC : normal WBC 10.4, Hgb 14, PLT 131

  • WSR : 28

  • CPK : 53

  • TSH : 2.66


Lab continued

Lab Continued

  • Vitamin D : 31

  • CCP : <1

  • RF: <5

  • ANA reflex : negative

  • CRP : 18.1


Clinical course

Clinical Course

  • The patient was empirically treated with Prednisone 20 mg po daily . She had a “miraculous” response after the first dose. On her follow up visit she was asymptomatic and her CRP fell to 2.2 . Her prednisone has been slowly tapered subsequently and she continues to do well.


Differential diagnosis

Differential Diagnosis

  • Polymyalgiarheumatica

  • Seronegative rheumatoid arthritis

  • Bursitis / tendonitis

  • RS3PE syndrome ( Remitting seronegative symmetrical synovitis with pitting edema )

  • Spondyloartropathy


Differential diagnosis continued

Differential Diagnosis Continued

  • CPPD disease ( calcium pyrophosphate deposition disease )

  • Fibromyalgia

  • Hypothyroidism

  • Paraneoplastic syndrome

  • Infective endocarditis

  • Inflammatory Myopathy


Differential diagnosis continued1

Differential Diagnosis Continued

  • Vasculitis

  • Miscellaneous ( Parkinson’s disease Hyperparathyroidism, Drug induced-HCG, Depression)


Seronegative rheumatoid arthritis

Seronegative Rheumatoid Arthritis

  • Symmetric polyarthritis of small joints of hands and feet

  • Does not respond to low dose steroids

  • Can mimic Polymyalgiarheumatica

  • Lower WSR and CRP than PMR


Bursitis

Bursitis

  • Not bilateral

  • Usually does not have elevated WSR or CRP


Rs3pe syndrome

RS3PE Syndrome

  • Remitting seronegative symmetrical synovitis with pitting edema

  • Sudden onset of polyarthitis

  • Negative rheumatoid factor

  • Distal joint involvent

  • Some response to steroids

  • May be paraneoplastic


Spondyloarthropathy

Spondyloarthropathy

  • Proximal symptoms

  • Elevated WSR

  • Axial skeletal involvement

  • Edema

  • Constitutional symptoms

  • Enthesitis


Spondyloarthropathy continued

Spondyloarthropathy Continued

  • Uveitis

  • Sacroillitis

  • HLA –B27


Cppd disease

CPPD Disease

  • Calcium pyrophoshate deposition disease

  • Characteristic crystals on joint aspiration

  • Chondrocalcinosis


Fibromyalgia

Fibromyalgia

  • Tender points

  • Widespread musculoskeletal pain

  • Aching

  • Fatigue

  • Normal WSR and CRP


Hypothyroidism

Hypothyroidism

  • Aching

  • Stiffness

  • Arthralgias

  • Elevated TSH


Paraneoplastic syndrome

Paraneoplastic Syndrome

  • Diffuse muscle and joint pain

  • Does not respond to steroids


Infective endocarditis

Infective Endocarditis

  • Persistent fever

  • Heart murmur

  • Diffuse aching


Inflammatory myopathy

Inflammatory Myopathy

  • Dermatomyositis or polymyositis

  • Proximal muscle weakness

  • Elevated CPK

  • Abnormal EMG

  • Myositis on biopsy


Vasculitis

Vasculitis

  • Can mimic PMR

  • Positive ANCA

  • Upper respiratory involvement

  • Pulmonary hemorrhage

  • Renal disease

  • Neuropathy


Miscellaneous

Miscellaneous

  • Parkinsons

  • Hyperparathyroidism

  • Drug induced

  • Depression


Atypical polymyalgia rheumatica

HCG

  • No clinical reports linking HCG to rheumatologic syndromes

  • HCG diets are popular currently

  • No clinical evidence that HCG is better than placebo in curbing appetite


Polymyalgia rheumatica

Polymyalgiarheumatica

  • Aching in shoulders neck and hip girdle

  • Severe morning stiffness

  • Can be associated with giant cell arteritis


Atypical polymyalgia rheumatica

PMR

  • 15-30% of cases develop GCA


Atypical polymyalgia rheumatica

PMR

  • Usually occurs in adults greater than 50 years old

  • Average age of patients is 70


Atypical polymyalgia rheumatica

PMR

  • Prevalance is 700 per 100,000 over the age of 50

  • Women are effected 2-3 times more than men


Atypical polymyalgia rheumatica

PMR

  • Incidence is higher in northern regions

  • 113/100,000 in Norway

  • 13/100,000 in Italy


Pathogenesis

Pathogenesis

  • PMR is associated with specific alleles of HLA DR4

  • Macrophages and CD4 T lymphocytes are found in synovial membranes


Clinical manifestations

Clinical Manifestations

  • At least 30 minutes of stiffness in the morning

  • Trouble dressing and rising from or turning in bed

  • Pain in shoulders in 70-95% of patients

  • Pain in hips and neck in 50-70% of patients

  • The pain worsens with movement


Clinical manifestations continued

Clinical Manifestations Continued

  • Synovitis and bursitis

  • 50% of patients have distal synovitis in knees and wrists

  • Swelling and tenosynovitis can be seen in hands wrists and ankles

  • Carpal tunnel syndrome can be seen in 10-15% of patients


Clinical manifestations continued1

Clinical manifestations Continued

  • Decreased ROM in shoulders neck and hips

  • Muscle tenderness in not a prominent feature

  • Shoulder tenderness is more due to synovial or bursal inflammation

  • Subjective weakness


Clinical manifestations continued2

Clinical Manifestations Continued

  • Systemic signs and symptoms in 40%

  • Malaise

  • Fatigue

  • Depression

  • Weight loss

  • Fever


Laboratory1

Laboratory

  • WSR greater than 40 in 78-93%

  • Elevated CRP is more common than elevated WSR

  • CRP is greater than 5 in 99%

  • 90% of patients with a normal WSR had an elevated CRP


Laboratory continued

Laboratory Continued

  • Normocytic anemia can be seen

  • Negative ANA

  • Negative rheumatoid factor

  • Negative CCP

  • Elevated alkaline phosphatase can be seen


Imaging

Imaging

  • MRI shows inflammation of extra-articular synovial structures:

  • Tenosynovial sheaths and bursas

  • Subacromial and subdeltoid bursitis

  • Ultrasound shows the same in 96% of patients


Diagnosis

Diagnosis

  • Age greater than 50

  • Bilateral aching and morning stiffness greater than 30 minutes

  • WSR greater than 40

  • Prompt response to steroids ( 50-70% of patients are better in 3 days )


Atypical presentations

Atypical Presentations

  • Age 40-50 years

  • WSR less than 40


Treatment

Treatment

  • Baseline lab testing ( glucose ,UA ,creatinine, alkaline phosphatase and calcium )

  • Dexa scan


Treatment continued

Treatment Continued

  • 15 mg prednisone daily ( range 10-20 mg )

  • Improvement can be noted after the first dose

  • 50-70% reduction in pain and stiffness within 3 days

  • Doses greater than 15mg daily are associated with more side effects

  • If no response consider GCA


Maintenance therapy

Maintenance Therapy

  • Maintain dose for 2-4 weeks that suppressed symptoms

  • If on greater than 15 mg daily of prednisone taper in 5mg daily decrements every 2-4 weeks

  • If on 15mg daily taper in 2.5 mg daily decrements every 2-4 weeks


Maintenance therapy continued

Maintenance Therapy Continued

  • When at a daily dose of 10 mg reduce the dose no faster than 1mg monthly

  • This approach allows therapy for one year


Monitoring response to therapy

Monitoring Response to Therapy

  • WSR and CRP generally normalize with prednisone therapy

  • If they do not consider GCA or malignancy

  • Monitor WSR and CRP every 2-3 months


Relapse

Relapse

  • Occurs in 25-50% of patients

  • If off steroids resume dose at original dose of steroids that control was achieved with

  • If on prednisone increase the dose to the lowest dose that controlled the symptoms ( a 1-2 mg increase may be adequate )

  • In patients who relapse multiple times the dose reduction interval should be every 2-3 months


Relapse continued

Relapse Continued

  • Distinguish recurrent symptoms of PMR from those secondary to prednisone taper alone:

  • Rest increases stiffness and aching in PMR

  • Rest improves symptoms in steroid withdrawal


Relapse continued1

Relapse Continued

  • An alternative approach to relapse is methylprednisolone 40-120 mg im


Steroid treatment side effects

Steroid Treatment Side Effects

  • Osteoporosis

  • Glucose intolerance

  • Hypertension


Management of steroid side efects

Management of Steroid Side Efects

  • Dexa scan

  • Calcium and Vitamin D therapy

  • Prophylactic bisphosphonates in some cases

  • Monitor blood pressure , glucose, and A1C levels


Alternative therapies

Alternative Therapies

  • No agent has been proven effective as a steroid sparring agent when used in combination

  • Methotrexate has been used in combination with high dose prednisone in patients with GCA , but this is controversial

  • TNF inhibitors and infliximab were not helpful


Alternative therapies continued

Alternative Therapies Continued

  • Etanercept therapy shows some benefit but there is no evidence that it can be a substitute for prednisone

  • In patients who could not reduce prednisone below 7.5-10 mg daily or had severe steroid side effects one small study ( 6 pts ) used Etanercept 25 mg twice weekly for 24 weeks with good results


Other therapy

Other Therapy

  • Physical therapy

  • Low dose NSAIDs with PPIs for concurrent osteoarthritis


Prognosis

Prognosis

  • Most patients have a self limited course that lasts from months to a few years

  • Some require therapy for 2-3 years

  • 10% will relapse within 10 years following a course of therapy

  • There is no increase in mortality from PMR itself


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