Atypical polymyalgia rheumatica
Download
1 / 64

Atypical Polymyalgia Rheumatica - PowerPoint PPT Presentation


  • 107 Views
  • Uploaded on

Atypical Polymyalgia Rheumatica. A Case Report.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about ' Atypical Polymyalgia Rheumatica' - saddam


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
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.



Review of systems
Review of Systems strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

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

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


Allergies
Allergies strenuous activity, visual changes, jaw

  • None


Medications
Medications strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Hypertension

  • Depression

  • Migraine

  • Obesity


Past surgical history
Past Surgical History strenuous activity, visual changes, jaw

  • Knee surgery bilaterally

  • Cholecystectomy

  • Foot surgery

  • Breast cyst aspiration


Social history
Social History strenuous activity, visual changes, jaw

  • No smoking

  • Rare social alcohol

  • No illicit drug abuse

  • Lesbian

  • Works as a manager at Petsmart


Family history
Family History strenuous activity, visual changes, jaw

  • Unknown ( Adopted )


Physical examination
Physical Examination strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Vitamin D : 31

  • CCP : <1

  • RF: <5

  • ANA reflex : negative

  • CRP : 18.1


Clinical course
Clinical Course strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Polymyalgiarheumatica

  • Seronegative rheumatoid arthritis

  • Bursitis / tendonitis

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

  • Spondyloartropathy


Differential diagnosis continued
Differential Diagnosis Continued strenuous activity, visual changes, jaw

  • CPPD disease ( calcium pyrophosphate deposition disease )

  • Fibromyalgia

  • Hypothyroidism

  • Paraneoplastic syndrome

  • Infective endocarditis

  • Inflammatory Myopathy


Differential diagnosis continued1
Differential Diagnosis Continued strenuous activity, visual changes, jaw

  • Vasculitis

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


Seronegative rheumatoid arthritis
Seronegative strenuous activity, visual changes, jaw 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 strenuous activity, visual changes, jaw

  • Not bilateral

  • Usually does not have elevated WSR or CRP


Rs3pe syndrome
RS3PE Syndrome strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Proximal symptoms

  • Elevated WSR

  • Axial skeletal involvement

  • Edema

  • Constitutional symptoms

  • Enthesitis


Spondyloarthropathy continued
Spondyloarthropathy strenuous activity, visual changes, jaw Continued

  • Uveitis

  • Sacroillitis

  • HLA –B27


Cppd disease
CPPD Disease strenuous activity, visual changes, jaw

  • Calcium pyrophoshate deposition disease

  • Characteristic crystals on joint aspiration

  • Chondrocalcinosis


Fibromyalgia
Fibromyalgia strenuous activity, visual changes, jaw

  • Tender points

  • Widespread musculoskeletal pain

  • Aching

  • Fatigue

  • Normal WSR and CRP


Hypothyroidism
Hypothyroidism strenuous activity, visual changes, jaw

  • Aching

  • Stiffness

  • Arthralgias

  • Elevated TSH


Paraneoplastic syndrome
Paraneoplastic strenuous activity, visual changes, jaw Syndrome

  • Diffuse muscle and joint pain

  • Does not respond to steroids


Infective endocarditis
Infective strenuous activity, visual changes, jaw Endocarditis

  • Persistent fever

  • Heart murmur

  • Diffuse aching


Inflammatory myopathy
Inflammatory strenuous activity, visual changes, jaw Myopathy

  • Dermatomyositis or polymyositis

  • Proximal muscle weakness

  • Elevated CPK

  • Abnormal EMG

  • Myositis on biopsy


Vasculitis
Vasculitis strenuous activity, visual changes, jaw

  • Can mimic PMR

  • Positive ANCA

  • Upper respiratory involvement

  • Pulmonary hemorrhage

  • Renal disease

  • Neuropathy


Miscellaneous
Miscellaneous strenuous activity, visual changes, jaw

  • Parkinsons

  • Hyperparathyroidism

  • Drug induced

  • Depression


HCG strenuous activity, visual changes, jaw

  • 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
Polymyalgia strenuous activity, visual changes, jaw rheumatica

  • Aching in shoulders neck and hip girdle

  • Severe morning stiffness

  • Can be associated with giant cell arteritis


PMR strenuous activity, visual changes, jaw

  • 15-30% of cases develop GCA


PMR strenuous activity, visual changes, jaw

  • Usually occurs in adults greater than 50 years old

  • Average age of patients is 70


PMR strenuous activity, visual changes, jaw

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

  • Women are effected 2-3 times more than men


PMR strenuous activity, visual changes, jaw

  • Incidence is higher in northern regions

  • 113/100,000 in Norway

  • 13/100,000 in Italy


Pathogenesis
Pathogenesis strenuous activity, visual changes, jaw

  • PMR is associated with specific alleles of HLA DR4

  • Macrophages and CD4 T lymphocytes are found in synovial membranes


Clinical manifestations
Clinical Manifestations strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Systemic signs and symptoms in 40%

  • Malaise

  • Fatigue

  • Depression

  • Weight loss

  • Fever


Laboratory1
Laboratory strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Normocytic anemia can be seen

  • Negative ANA

  • Negative rheumatoid factor

  • Negative CCP

  • Elevated alkaline phosphatase can be seen


Imaging
Imaging strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Age 40-50 years

  • WSR less than 40


Treatment
Treatment strenuous activity, visual changes, jaw

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

  • Dexa scan


Treatment continued
Treatment Continued strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

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


Steroid treatment side effects
Steroid Treatment Side Effects strenuous activity, visual changes, jaw

  • Osteoporosis

  • Glucose intolerance

  • Hypertension


Management of steroid side efects
Management of Steroid Side strenuous activity, visual changes, jaw Efects

  • Dexa scan

  • Calcium and Vitamin D therapy

  • Prophylactic bisphosphonates in some cases

  • Monitor blood pressure , glucose, and A1C levels


Alternative therapies
Alternative Therapies strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • 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 strenuous activity, visual changes, jaw

  • Physical therapy

  • Low dose NSAIDs with PPIs for concurrent osteoarthritis


Prognosis
Prognosis strenuous activity, visual changes, jaw

  • 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


ad