Rheumatology 2

polymyositis, dermatomyositis, proximal, enzymes, mononuclear, necrosis

Idiopathic Inflammatory Myopathies

• Includes ______________ (PM), _______________ (DM), and multiple types of myositis

• Common clinical features

  • Progressive, symmetrical, ___________ muscle weakness, elevation of muscle ___________, muscle histology showing ___________ inflammatory cell infiltrates and muscle fiber ___________, and EMG abnormalities

T, myofibers, CD8, 1, necrosis

Polymyositis (PM)

• Pathogenesis

  • Endomysial _ cells surround and invade ___________, macrophages, dendritic cells, and plasma cells

  • Clonally expanded ___-positive cytotoxic T cells invade muscle fibers that express MHC class _, which leads to fiber _________

microangiopathy, autoantibodies, complement, ischemia

Dermatomyositis

• Pathogenesis

  • A ______________ affecting both skin and muscle

  • _____________ cause injury to endothelial cells, which activates the ___________ cascade, and leads to capillary injury and muscle _________

proximal, malar, Shawl, heliotrope, periungal, gottron, scalp

Dermatomyositis

• S/S

  • Symmetric _________ muscle weakness

  • Dusky red rash in ______ distribution that mimics SLE

    • Facial erythema beyond the malar distribution

    • “_______ or V sign”

  • Periobital edema and purplish color over the eyelids (__________ rash)

  • _________ erythema and dilation of nailbed capillaries

  • _____________ sign: scaly patches over dorsum of PIP and MCP joints

  • ______ involvement may mimic psoriasis

weakness, leg, arm, dysphagia, atrophy

Polymyositis (PM)

• S/S

  • Symmetric proximal muscle __________

    • Gradual and progressive

    • Upper and lower ext, neck

    • ___ weakness often precedes ___ weakness

  • Pain and tenderness of affected muscles

  • ___________

  • Muscle ___________ and contractures late in disease

extramuscular, autoantibody, >, PM

Idiopathic Inflammatory Myopathies

• Differing Features

  • Association with ___________ manifestations and ____________ profiles

• Epidemiology for PM and DM

  • F _ M for PM and DM

  • Childhood or adult onset (__ rare in children

  • Peak incidence in 4th-6th decades

subacute, weakness, deltoids, hip, neck, mild, cancer

Clinical Features of PM and DM

• ___________ presentation (weeks/months)

• Symmetric proximal muscle _________ (cardinal feature)

  • ___________, ___ flexors, _____ flexors most commonly

    • Difficulty washing/combing hair, rising from a seated position, climbing stairs, lifting head off pillow

• No pain to ____ myalgias only

• Associated with ________ (DM > PM)

Gottron Papules

What DM skin finding is being described?

• Erythematous/violaceous papules over bony prominences

  • Dorsal aspect of MCP and IP joints most common

• Can scale and/or ulcerate

Heliotrope eruption

What DM skin finding is being described?

• Erythematous/violaceous eruption on periorbital skin

• ± eyelid edema

shawl, V, nailfold, calcinosis cutis

Other Skin findings in DM

• Photodistributed erythema (______ and _ sign)

• Poikiloderma

• __________ changes

• Scalp involvement

• ____________ ________

dysphonia, reflux, interstitial, myocarditis

Extramuscular S/S of PM and DM

• General

  • Fatigue, weight loss, hoarseness, ____________ (pharyngeal weakness), Raynaud’s

• GI

  • Dysphagia, ________, dysmotility of the bowel

• Pulm

  • Respiratory muscle weakness causing ventilator insufficiency, ____________ lung disease

• Cardiac

  • ___________, CHF, MI

• MSK

  • Arthritis/arthralgias

DM

Which condition is more associated with the following cancers?

• Ovarian

• Lung

• Pancreatic

• Breast

• Colorectal

• Stomach

• Lymphoma

• Adeno > Squamous > Lymphoid

PM

Which condition is more associated with the following cancers?

• Lung

• Non-Hodgkins lymphoma

• Bladder

muscle, CK, aldolase, ANA, muscle biopsy, endomysial, perifascicular

Diagnosis of PM and DM

• Labs

  • Elevated _______ enzymes → __, LDH, __________

  • Autoantibodies

    • ___ (80% of pts)

    • Myositis specific (30-40% of pts)

    • Myositis Associated

  • EMG

  • _______ ________ (Definitive Dx)

    • PM → __________ inflammation

    • DM → ____________ and perivascular inflammation

Anti-Jo-1, Anti-Mi-2, Anti-155/140, Anti-signal

Myositis Specific Antibodies

• ____-__-_ (antisynthetase)

  • PM or DM with Interstitial lung disease, arthritis, mechanic’s hands

• ____-__-_

  • DM with rash more than myositis

• ____-___/___

  • Cancer-associated myositis

• ____-_________

  • Severe, acute necrotizing myopathy

steroids, strength, function, year, decrease, methotrexate, hydroxychloroquine

Treatment of DM and PM

• _________

  • Initial Treatment

  • Show to improve __________ and preserve muscle __________

  • High dose then taper over a _____ (depends on response)

  • Monitor muscle enzymes (should _________)

  • 80% of pts improve with steroids

• DMARD

  • ______________ or azathioprine most often used

  • Sometimes added initially along with steroids

  • If fail steroids

• Anti-malarial

  • _______________ useful for skin lesions

pain, stiffness, 50, >, unknown, GCA

Polymyalgia Rheumatica (PMR)

• Symptom complex that includes ____ and __________ of the shoulder and hip girdles and proximal extremities

• Epidemiology

  • Age > __ y/o (peak between 70-80 y/o)

  • F _ M

• Pathogenesis

  • Mostly __________

  • Similar to ___

acute, bilateral, mornings, shoulders, ROM

Polymyalgia Rheumatic Symptoms

• Aching/Stiffness

  • Can be _____/abrupt

  • __________

  • Severe in _________ (lasting > 1 hr)

  • __________, neck, hip girdle, torso

  • Limited ___

• GCA

  • Ask/look for s/s of GCA

clinical, normal, US, MRI, steroids, quick, years

Polymyalgia Rheumatica Diagnosis

• Mostly __________

• Labs

  • CBC w/ diff → may see normocytic anemia

  • ESR/CRP → often elevated

  • CK → usually _______

• Imaging

  • __ or ___ → often show bursitis and/or tenosynovitis

• Treatment

  • Low dose _________ (10-20 mg/day)

    • Response usually ______

    • Most can taper off after 1-2 _______

fatigue, soft, female, stress, regulation

Fibromyalgia

• Controversial, chronic MSK syndrome characterized by widespread joint and muscle pain, _________, and tender points

• Considered _____ tissue rheum condition

• Epidemiology

  • Majority are ________

  • Peak between 20-50 y/o

• Pathogenesis/Etiology

  • Unknonw

  • Thought to be genetic susceptibility and environmental triggers altering gene expression

  • Alterations in neuroendocrine and _______ responses

  • Alterations in pain ___________

depressed, pain, fatigue, sleep, cognitive

Fibromyalgia Multi-system characters

• ___________ mood

• Widespread chronic ____

• Severe _________

• _________ dysfunction

• Decreased well-being

• Stiffness

• _________ impairment

• Headaches

Depression, IBS, fatigue, localized

Fibromyalgia Concomitant Conditions

• __________ (25-60%)

• ___ (50-80%)

• Migraines (50%)

• Chronic ____________ Syndrome (70%)

• Myofascial pain (possible ____________ form of FM)

WPI, SSS, generalized, 3

Fibromyalgia Diagnosis

• ACR 2016 Fibromyalgia Diagnostic Criteria

  • Wide-spread Pain Index (___) >7 and Symptom Severity Scale (___) >5 OR WPI 4-6 and SSS >9

  • ______________ pain, defined as pain in at leas four of five regions, is present

    • Left upper, right upper, left lower, right lower, axial

  • Symptoms have been present at a similar level for at least _ months

  • A dx of FM is valid regardless of other diagnoses that the pt may have. A dx of FM does not exclude the presence of other clinically important illnesses

individualized, comorbidities, exercise, CBT, SNRI, anticonvulsant, tricyclic, analgesics

Fibromyalgia Treatment

• ___________ and multidisciplinary

• Nonpharmacologic

  • Education

  • Address _____________

  • ___________ → can be hard b/c pain and fatigue

  • Cognitive behavioral therapy (___)

• Pharmacologic

  • ____ (duloxetine, milnacipran)

  • Alpha-2-ligand ___________ (pregabalin)

  • ____________ antidepressant (amitriptyline)

  • Other options → fluoxetine, gabapentin, naltrexone

• ____________ haven’t shown benefit