1. Atypical Polymyalgia Rheumatica

2. 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.

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

4. 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

5. 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

6. ROM Continued
Neurologic: No motor weakness, numbness, paresthesias, cognitive symptoms
Psychiatric: recent discontinuation of Zoloft which she had used since 2004

7. Allergies
None

8. 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

9. Past Medical History
Hypertension
Depression
Migraine
Obesity

10. Past Surgical History Knee surgery bilaterally Cholecystectomy
Foot surgery
Breast cyst aspiration

11. Social History No smoking Rare social alcohol No illicit drug abuse
Lesbian
Works as a manager at Petsmart

12. Family History
Unknown ( Adopted )

13. 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

14. 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

15. 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

16. 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

17. Lab Continued Vitamin D : 31 CCP : <1 RF: <5
ANA reflex : negative
CRP : 18.1

18. 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.

19. Differential Diagnosis
Polymyalgia rheumatica
Seronegative rheumatoid arthritis
Bursitis / tendonitis
RS3PE syndrome ( Remitting seronegative symmetrical synovitis with pitting edema )
Spondyloartropathy

20. Differential Diagnosis Continued
CPPD disease ( calcium pyrophosphate deposition disease )
Fibromyalgia
Hypothyroidism
Paraneoplastic syndrome
Infective endocarditis
Inflammatory Myopathy

21. Differential Diagnosis Continued
Vasculitis
Miscellaneous ( Parkinson’s disease Hyperparathyroidism, Drug induced-HCG, Depression)

22. 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

23. Bursitis
Not bilateral
Usually does not have elevated WSR or CRP

24. 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

25. Spondyloarthropathy Proximal symptoms Elevated WSR
Axial skeletal involvement
Edema
Constitutional symptoms
Enthesitis

26. Spondyloarthropathy Continued
Uveitis
Sacroillitis
HLA –B27

27. CPPD Disease Calcium pyrophoshate deposition disease
Characteristic crystals on joint aspiration
Chondrocalcinosis

28. Fibromyalgia Tender points Widespread musculoskeletal pain Aching
Fatigue
Normal WSR and CRP

29. Hypothyroidism
Aching
Stiffness
Arthralgias
Elevated TSH

30. Paraneoplastic Syndrome
Diffuse muscle and joint pain
Does not respond to steroids

31. Infective Endocarditis
Persistent fever
Heart murmur
Diffuse aching

32. Inflammatory Myopathy
Dermatomyositis or polymyositis
Proximal muscle weakness
Elevated CPK
Abnormal EMG
Myositis on biopsy

33. Vasculitis Can mimic PMR Positive ANCA Upper respiratory involvement
Pulmonary hemorrhage
Renal disease
Neuropathy

34. Miscellaneous
Parkinsons
Hyperparathyroidism
Drug induced
Depression

35. 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

36. Polymyalgia rheumatica
Aching in shoulders neck and hip girdle
Severe morning stiffness
Can be associated with giant cell arteritis

37. PMR
15-30% of cases develop GCA

38. PMR Usually occurs in adults greater than 50 years old
Average age of patients is 70

39. PMR Prevalance is 700 per 100,000 over the age of 50
Women are effected 2-3 times more than men

40. PMR Incidence is higher in northern regions 113/100,000 in Norway
13/100,000 in Italy

41. Pathogenesis PMR is associated with specific alleles of HLA DR4
Macrophages and CD4 T lymphocytes are found in synovial membranes

42. 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

43. 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

44. 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

45. Clinical Manifestations Continued
Systemic signs and symptoms in 40%
Malaise
Fatigue
Depression
Weight loss
Fever

46. 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

47. Laboratory Continued Normocytic anemia can be seen Negative ANA
Negative rheumatoid factor
Negative CCP
Elevated alkaline phosphatase can be seen

48. 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

49. 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 )

50. Atypical Presentations
Age years
WSR less than 40

51. Treatment
Baseline lab testing ( glucose ,UA ,creatinine, alkaline phosphatase and calcium )
Dexa scan

52. 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

53. 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

54. 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

55. 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

56. 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

57. 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

58. Relapse Continued
An alternative approach to relapse is methylprednisolone mg im

59. Steroid Treatment Side Effects
Osteoporosis
Glucose intolerance
Hypertension

60. Management of Steroid Side Efects
Dexa scan
Calcium and Vitamin D therapy
Prophylactic bisphosphonates in some cases
Monitor blood pressure , glucose, and A1C levels

61. 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

62. 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

63. Other Therapy Physical therapy
Low dose NSAIDs with PPIs for concurrent osteoarthritis

64. 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