PMR/GCA/polymyositis/dermatomyositis Flashcards

1
Q

Polymyalgia Rheumatica (PMR)

general

Etiology

A

Most common chronic inflammatory condition in older adults

Primarily affects proximal muscles and joints, causing disability

Closely associated with giant cell arteritis
Many consider the two disorders to be aspects of the same process
May occur independently or concomitantly
Polymyalgia rheumatica is more common than giant cell arteritis

Commonly affects:
Patients > 50 years of age
♀>♂

Incidence:
↑ with age
Peak incidence – 70-80 years
More likely to develop in patient with HLA-DR4 and after adenovirus or parvovirus B-19 infection

First described in 1888 and referred to as “senile rheumatic gout”

This not a myopathy

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2
Q

Polymyalgia rheumatica

Hallmark S/Sx’s

A

Hallmark symptoms:
Proximal muscle and joint pain
Most severe in the neck, shoulders, upper back, and buttocks
Develops over weeks to months
Synovitis, bursitis, tenosynovitis

Proximal muscle stiffness
Typically in the morning lasting > 60 minute
s
After prolonged sitting or reclining
Muscle tenderness

Shoulder involvement in 95% of cases

Synovitis: synovium of a joint becomes inflamed(swollen)
Bursitis: painful swelling of a small, fluid-filled sac called a bursa; bursa cushion the bones, tendons, and muscles near joints
Tenosynovitis: broad term describing the inflammation of the fluid-filled synovium within the tendon sheath

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3
Q

Polymyalgia rheumatica

Systemic symptoms

A

Fever
Night sweats
Fatigue
Anorexia
Weight loss
No weakness
Weakness may develop later (disuse atrophy)

Patients with complain of trouble with getting out of bed or performing daily activities
Often will have to “roll out of bed”

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4
Q

Polymyalgia rheumatica

Dx

A

No definitive test for polymyalgia rheumatica
Other possible causes for symptoms must be ruled out (diagnosis of exclusion)

Labs:
Inflammatory Markers:
ESR
Normal ESR for a patient’s age is best calculated as age/2 for men, and age+10/2 for women

Mean ESR is 65 mm per hour

>100 mm per hour raises concern for concomitant giant cell arteritis or underlying malignancy

CRP- not dependant on age
Often elevated when ESR is normal
More sensitive than ESR
Less affected by age

CK should be normal- there is not muscle destruction (not a myopathy)

Electromyography (EMG)…should this test be normal

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5
Q

Polymyalgia rheumatica

Normal ESR for a patient’s age is best calculated as

A

age/2 for men, and age+10/2 for women
Mean ESR is 65 mm per hour

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6
Q

Polymyalgia rheumatica

Tx

A

Corticosteroids
Prednisone 15-20 mg PO once daily
Dramatic improvement within hours to a day (supports the diagnosis)
If giant cell arteritis is also present, prednisone dosing will need to be higher
Taper prednisone to the lowest clinically effective dose as symptoms subside
Mean length of treatment is 1.8 years
Monitor for steroid complications (diabetes, hypertension, osteoporosis)

Methotrexate 10-15 mg PO once weekly
Used in patients who have frequent recurrences
Folic acid 1 mg daily

Use of bisphosphonates, specifically alendronate (Fosamax) and risedronate (Actonel), can increase bone mineral density and decrease vertebral fractures in patients on long-term glucocorticoid therapy

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7
Q

polymyalhia rheumatica

A
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8
Q

Giant Cell Arteritis

General

A

Also known as temporal arteritis
Most common type of vasculitis in adults
Affects medium and large arteries with a predilection for the extracranial carotid branches
More serious condition than polymyalgia rheumatica
Common affects:
♀>♂
Incidence:
↑ with age
Peak incidence - 70-80 years

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9
Q

GCA

Patho

A

Caused by the deposition of immune complexes in the involved arteries (those containing elastic tissue), resulting in a local immune response

Eliciting cause of this immune system reaction is not known
? Initiating viral pathogen

Intimal hyperplasia and luminal obstruction leads to ischemic manifestations
May be localized, multifocal, or widespread

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10
Q

GCA

S/Sx

A

Headache (85%)
Most common initial and frequent symptom
Involves the temporal and occipital region(s)

Burning or boring-type pain
Tenderness of arteries (temporal artery)
Swollen, nodular and/or erythematous

Jaw claudication
Weakness in the muscles of mastication (inflammation of the maxillary artery)
Onset with chewing and relieved by rest

Visual symptoms
Diplopia, blurring, or amaurosis fugax → blindness
Loss of vision occurs within hours to days
Contralateral eye is often affected within one to two weeks

Jaw claudication and visual symptoms are ischemic warning signs

Amaurosis fugax: painless temporary loss of vision in one or both eyes

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11
Q

GCA

Systemic symptoms:

A

Fever
Fatigue
Anorexia
Weight loss

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12
Q

GCA

Suspicion of Dx

A

Suspicion based on history and physical examination

Suspected in patients> 50 if any of the following develops, especially if they also have symptoms of systemic inflammation:
A new type of headache
Any new symptom or sign compatible with ischemia of an artery above the neck
Jaw muscle pain during chewing
Temporal artery tenderness
Unexplained subacute fever

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13
Q

GCA

Dx

A

Inflammatory markers
ESR
A normal ESR does not exclude giant cell arteritis
CRP

Temporal artery biopsy
Obtained within one week of starting treatment
Performed on the patient’s most symptomatic side
Sample of 1-2 cm; up to 5 cm, increase the yield

Negative biopsy does not rule out disease as temporal arteritis does not affect arteries in a continuous fashion; if suspicion remains high, obtain a color-duplex US of the temporal artery or an MRI with contrast of the cranial vessels

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14
Q

GCA

Tx

A

Medical emergency due to neuro-ophthalmic complications
Referral to rheumatology

Corticosteroids
Uncomplicated disease
Prednisone 40-60 mg daily

Complicated disease (visual disturbances)
methylprednisolone (Solu-Medrol) 500-1,000 mg IV daily for 3-5 days, then Prednisone 60 mg PO daily

If symptoms lessen,prednisone can be tapered gradually
Most patients require at least 2 years of treatment with corticosteroids

Prevention of long-term corticosteroid adverse events
PPI for gastrointestinal protection
Antiresorptive drug (bisphosphonate) to increase bone mass and prevent osteoporosis

To reduce exposure to corticosteroids (adjunctive agent)
Methotrexate
Tocilizumab
– interleukin-6 inhibitor

Low-dose aspirin
Decrease cerebrovascular complications
81 mg PO daily

If there is symptomatic relapse with tapering of steroids, immediately increase the steroid dose and start tocilizumab or a DMARD until symptoms resolve; these patients will need a longer steroid taper

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15
Q

GCA

A
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16
Q

Polymyositis (PM)

General

A

Autoimmune inflammatory myopathy caused by T cell-mediated (CD8 T cells) muscle injury
Tends to present between 30-60 years; smaller peak around age 15

♀>♂
Etiology is unknown - Idiopathic
Possible genetic predisposition
HLA-A1, HLA-B8, HLA-DR3
Environmental factors
Infections: HIV, Hepatitis B & C
Drug-induced: ACE inhibitors, D-penicillamine, Hydralazine
Associated medical conditions
Malignancy
Connective-tissue disorders

17
Q

Polymyositis

Muscle weakness:

A

Develops gradually over weeks to months
Symmetric, involving proximal muscles
Particularly evident when:
Climbing stairs
Brushing hair
Rising from a seated position
Reaching for overhead objects
Holding up the head
Pharyngeal weakness causing dysphagia

Associated myalgias occur in a small percentage of patients
Complaints of aches or cramps

Distal muscle involvemnt and facial muscle involvement can with advanced

small amount of pain. WEAKNESS.

Polymyositis – weakness, normally without pain; muscle destruction
Polymyalgia rheumatica – painful muscles, without weakness; no muscle destruction

18
Q

Polymyositis

Other S/Sx

A

Arthralgias or arthritis:
Symmetric

Joints commonly involved:
Knees
Wrists
Hands

Raynaud’s phenomenon may be present

Constitutional symptoms:
Weight loss
Fatigue
Low-grade fever

19
Q

Polymyositis

Labs and Dx

A

Labs:
CBC
Leukocytosis
Thrombocytosis

Inflammatory markers
↑ Erythrocyte sedimentation rate (ESR)
↑ CRP
ANA
positivity
Nonspecific and not diagnostic
Seen in of patients

Muscle enzymes
↑↑ CK – 50x normal; level is a good indicator of disease activity

Myositis-specific antibody testing:
Anti-signal recognition protein (anti-SRP) antibodies
Anti-Jo-1 antibodies

Electromyography (EMG):
Helps to support the diagnosis

Muscle biopsy:
Confirms the diagnosis

know your antibodies

20
Q

Polymyositis

Tx

A

High-dose systemic glucocorticoids (1st-line)
50% of patients with PM will not respond to steroids alone
Therapy is usually needed for 9–12 months
Slowly tapered off
Levels of muscle enzymes are monitored to determine response to therapy

Immunosuppressive therapy
Methotrexate, azathioprine
Used in conjunction with glucocorticoid therapy

IV immunoglobulin (IVIG)
Patients with severe life-threatening weakness (severe dysphagia, respiratory insufficiency)

21
Q

Polymyositis - complications

Malignancy

A

Usually detected within the 1st year of diagnosis
Observed in men > 60 years of age with severe systemic involvement
Common cancers: non-Hodgkin’s lymphoma, and bladder, lung, breast, ovarian, stomach, pancreatic, prostate, and colorectal cancer

Patients diagnosed with PM should be evaluated for possible underlying malignancy

22
Q

Polymyositis

Respiratory complications:

A

Interstitial lung disease → pulmonary hypertension and cor pulmonale
Aspiration pneumonia due to dysphagia

23
Q

Polymyositis

Adverse effects of medical therapy:

A

Corticosteroid therapy
Osteoporosis and compression fractures
Insulin resistance → diabetes mellitus
Hypertension

Immunosuppression therapy
Opportunistic infections
Upper respiratory tract infections and pneumonia
Skin infections

24
Q

Dermatomyositis (DM)

general
Typical sx

A

An autoimmune inflammatory myopathy resulting from immune-complex deposition in the muscle capillaries

Patients present with symptoms similar to those observed in PM

Also exhibit characteristic cutaneous manifestations (heliotrope rash, Gottron’s sign, Gottron’s papules)

Diagnosis is based on clinical presentation and antibody evaluation, and may be confirmed with biopsy

Management includes systemic glucocorticoids, immunosuppressants, and screening for malignancy

25
Q

Dermatomyositis

Cutaneous Manifestations

A

Heliotrope rash:
Pathognomonic feature
Erythematous/violaceous rash around the upper eyelids
Periorbital edema

Gottron’s papules:
Pathognomonic feature
Symmetric
Erythematous/violaceous papules
Located on the dorsal metacarpophalangeal and interphalangeal joints

Facial erythema:
“Butterfly” pattern that mimics the malar rash of lupus
Involves the nasolabial folds

Gottron’s sign:
Erythematous/violaceous macules or patches
Often on extensor surfaces (hands, elbows, knees, and ankles)

Nail changes:
Dilated capillary loops at the proximal nailfold
Periungal erythema
Cuticle hypertrophy

Calcinosis cutis (common in children)
Deposition of calcium within the skin

26
Q
dermatomyositis
A
27
Q

Polymyositis VS polymyalgia rheumatica

A

Polymyositis – weakness, normally without pain; muscle destruction

Polymyalgia rheumatica – painful muscles, without weakness; no muscle destruction