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Atypical Polymyalgia Rheumatica
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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 She denied ever experiencing anything like this before. She did admit to losing 30 pounds intentionally after completing the HCG diet one month prior.
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She denied fever, night sweats, rash, mucosal ulcers, strenuous activity, visual changes, jaw claudication, small joint pain, swelling and respiratory symptoms.
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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
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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
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ROM Continued Neurologic: No motor weakness, numbness, paresthesias, cognitive symptoms Psychiatric: recent discontinuation of Zoloft which she had used since 2004
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Allergies None
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Medications HCTZ 25 mg po daily Metoprolol 12.5 po BID
KCL 10 meq po daily Recent HCG injections completed one month ago Ibuprofen 200mg po QID
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Past Medical History Hypertension Depression Migraine Obesity
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Past Surgical History Knee surgery bilaterally Cholecystectomy
Foot surgery Breast cyst aspiration
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Social History No smoking Rare social alcohol No illicit drug abuse
Lesbian Works as a manager at Petsmart
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Family History Unknown ( Adopted )
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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
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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
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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
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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
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Lab Continued Vitamin D : 31 CCP : <1 RF: <5
ANA reflex : negative CRP : 18.1
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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 Her prednisone has been slowly tapered subsequently and she continues to do well.
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Differential Diagnosis
Polymyalgia rheumatica Seronegative rheumatoid arthritis Bursitis / tendonitis RS3PE syndrome ( Remitting seronegative symmetrical synovitis with pitting edema ) Spondyloartropathy
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Differential Diagnosis Continued
CPPD disease ( calcium pyrophosphate deposition disease ) Fibromyalgia Hypothyroidism Paraneoplastic syndrome Infective endocarditis Inflammatory Myopathy
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Differential Diagnosis Continued
Vasculitis Miscellaneous ( Parkinson’s disease Hyperparathyroidism, Drug induced-HCG, Depression)
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Seronegative Rheumatoid Arthritis
Symmetric polyarthritis of small joints of hands and feet Does not respond to low dose steroids Can mimic Polymyalgia rheumatica Lower WSR and CRP than PMR
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Bursitis Not bilateral Usually does not have elevated WSR or CRP
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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
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Spondyloarthropathy Proximal symptoms Elevated WSR
Axial skeletal involvement Edema Constitutional symptoms Enthesitis
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Spondyloarthropathy Continued
Uveitis Sacroillitis HLA –B27
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CPPD Disease Calcium pyrophoshate deposition disease
Characteristic crystals on joint aspiration Chondrocalcinosis
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Fibromyalgia Tender points Widespread musculoskeletal pain Aching
Fatigue Normal WSR and CRP
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Hypothyroidism Aching Stiffness Arthralgias Elevated TSH
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Paraneoplastic Syndrome
Diffuse muscle and joint pain Does not respond to steroids
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Infective Endocarditis
Persistent fever Heart murmur Diffuse aching
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Inflammatory Myopathy
Dermatomyositis or polymyositis Proximal muscle weakness Elevated CPK Abnormal EMG Myositis on biopsy
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Vasculitis Can mimic PMR Positive ANCA Upper respiratory involvement
Pulmonary hemorrhage Renal disease Neuropathy
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Miscellaneous Parkinsons Hyperparathyroidism Drug induced Depression
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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
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Polymyalgia rheumatica
Aching in shoulders neck and hip girdle Severe morning stiffness Can be associated with giant cell arteritis
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PMR 15-30% of cases develop GCA
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PMR Usually occurs in adults greater than 50 years old
Average age of patients is 70
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PMR Prevalance is 700 per 100,000 over the age of 50
Women are effected 2-3 times more than men
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PMR Incidence is higher in northern regions 113/100,000 in Norway
13/100,000 in Italy
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Pathogenesis PMR is associated with specific alleles of HLA DR4
Macrophages and CD4 T lymphocytes are found in synovial membranes
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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
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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
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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
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Clinical Manifestations Continued
Systemic signs and symptoms in 40% Malaise Fatigue Depression Weight loss Fever
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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
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Laboratory Continued Normocytic anemia can be seen Negative ANA
Negative rheumatoid factor Negative CCP Elevated alkaline phosphatase can be seen
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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
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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 )
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Atypical Presentations
Age years WSR less than 40
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Treatment Baseline lab testing ( glucose ,UA ,creatinine, alkaline phosphatase and calcium ) Dexa scan
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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
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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
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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
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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
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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
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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
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Relapse Continued An alternative approach to relapse is methylprednisolone mg im
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Steroid Treatment Side Effects
Osteoporosis Glucose intolerance Hypertension
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Management of Steroid Side Efects
Dexa scan Calcium and Vitamin D therapy Prophylactic bisphosphonates in some cases Monitor blood pressure , glucose, and A1C levels
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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
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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 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
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Other Therapy Physical therapy
Low dose NSAIDs with PPIs for concurrent osteoarthritis
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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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