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Atypical Polymyalgia Rheumatica. A Case Report NG is a 47 year old female who developed severe shoulder and upper arm pain bilaterally as well as hip.

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Presentation on theme: "Atypical Polymyalgia Rheumatica. A Case Report NG is a 47 year old female who developed severe shoulder and upper arm pain bilaterally as well as hip."— Presentation transcript:

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 2.2. 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 % 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 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


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