Atypical Polymyalgia Rheumatica

1. Atypical PolymyalgiaRheumatica

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 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.

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 meqpo 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 2.2 . Her prednisone has been slowly tapered subsequently and she continues to do well.

19. Differential Diagnosis • Polymyalgiarheumatica • 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 Polymyalgiarheumatica • 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. Polymyalgiarheumatica • 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 40-50 years • WSR less than 40