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DERMATOMYOSITIS
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CLINICAL FEATURES
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DM can present at any age, including infancy Similar to most other autoimmune disorders, there is an increased incidence of DM in women compared to men11,19 Although the pathogenesis of childhood and adult DM is presumably similar, there are important differences in some of the clinical features and associated disorders Weakness can develop rather acutely (over several weeks), or insidiously (over months)13,20,21 Proximal leg and arm muscles are usually the earliest and most severely affected muscle groups Thus, the earliest patient complaints are often dif culty lifting their arms over their heads, climbing steps, and arising from chairs Distal muscles are also involved Children are more likely to present with an insidious onset of muscle weakness and myalgias that are often preceded by fatigue, low-grade
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TABLE 30 1 IDIOPATHIC INFLAMMATORY MYOPATHIES: CLINICAL AND LABORATORY FEATURES
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Disorder DM
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Sex F>M
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Age of Onset Childhood and adult
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Rash Yes
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Pattern of Weakness Proximal > distal
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Serum CK Increased (up to 50 normal)
Muscle Biopsy Perimysial and perivascular in ammation; MXA, MAC, Ig, and C deposition on vessels Endomysial in ammation
Cellular In ltrate CD4+ dendritic cells; B cells; macrophages
Response to IS Therapy Yes
Common Associated Conditions Myocarditis, interstitial lung disease, malignancy, vasculitis, and other connective tissue diseases Myocarditis, interstitial lung disease, and other connective tissue diseases Neuropathy; autoimmune disorders uncommon
Adult
Proximal > distal
Increased (up to 50 normal)
Yes CD8+ T cells; macrophages; plasma cells None or CD8+ T cells; minimal macrophages; plasma cells
Elderly (> 50 yr)
Proximal and distal; predilection for: nger/wrist exors, knee extensors
Normal or mildly increased (<10 normal)
Endomysial in ammation; rimmed vacuoles; amyloid deposits; EM: 15 18 nm tubulo laments
DM, dermatomyositis; PM, polymyositis; IBM, inclusion body myositis; F, female; M, male; IS, immunosuppressive; Ig, immunoglobulin; MAC, membrane attack complex; C, complement, CK, creatine kinase; MXA, interferon- - -inducible protein myxovirus resistance 1 From Amato AA, Barohn RJ Idiopathic in ammatory myopathies Neurol Clin 1997;15:615 648, with permission
INFLAMMATORY MYOPATHIES
TABLE 30 2 DIAGNOSTIC CRITERIA FOR INCLUSION BODY MYOSITIS
I Characteristic features inclusion criteria A Clinical features 1 Duration of illness >6 months 2 Age of onset >30 yr 3 Muscle weakness 4 Must predominantly affect proximal and distal muscles of the arms and legs and the patient muscle exhibit at least one of the following features: a Finger exor weakness b Wrist exor > wrist extensor weakness c Quadriceps muscle weakness (+ or < MRC grade 4) B Laboratory features 1 Serum creatine kinase < 12 times normal 2 Muscle biopsy a In ammatory myopathy characterized by mononuclear cell invasion of non-necrotic muscle bers b Vacuolated muscle bers c Either i Intracellular amyloid deposits (must use uorescent method of identi cation before amyloid excluded) or ii 15 1-nm tubulo laments by electron microscopy 3 Electromyography must be consistent with features of an in ammatory myopathy (however, long-duration potentials are commonly observed and do not exclude the diagnosis of inclusion body myositis [IBM]) C Family history: Rarely, IBM may be observed in families This condition is different from hereditary inclusion body myopathy without in ammation The diagnosis of familial IBM requires speci c documentation of mononuclear in ammatory cells invading non necrotic muscle bers by muscle biopsy in addition to vacuolated muscle bers and intracellular amyloid deposits or 15 18-nm tubulo laments II Associated disorders: IBM occurs with a variety of other, especially immune-mediated conditions An associated condition does not preclude a diagnosis of IBM if the diagnostic criteria are ful lled III Diagnostic criteria for IBM A De nite IBM 1 Patients muscle exhibit all muscle biopsy features including invasion of non-necrotic muscle bers by mononuclear cells, vacuolated muscle bers, and intracellular amyloid deposits or 15 18-nm tubulo laments 2 None of the other clinical or laboratory features are mandatory if the muscle biopsy features are diagnostic B Possible IBM: If the muscle biopsy shows only in ammation (invasion of non-necrotic muscle bers by mononuclear cells) without other pathological features of IBM, then a diagnosis of possible IBM can be made if the patient exhibits the characteristic clinical (A1,2,3,4) and laboratory (B1,3) features
With permission from Griggs et al Ann Neurol 1995;38:705 713
fevers, and a rash Dysphagia occurs in approximately 30% of patients with DM probably due to involvement of oropharyngeal and esophageal muscles3 Speech, chewing, and swallowing dif culties can arise secondary to involvement of the masseter muscle We have even seen dysarthria and speech as a result of involvement of the pharyngeal and the tongue muscles Sensation is normal, and muscle stretch re exes are preserved until a severe degree of weakness has developed DM is usually diagnosed earlier than other forms of myositis because of the characteristic rash, which typically accompanies or precedes the onset of muscle weakness1,3,20,22 However, the rash can develop years after the onset of weakness, which could lead to an erroneous diagnosis of PM Some patients have the characteristic rash but never develop weakness (the so-called amyopathic DM or DM sine myositis)23 Less well appreciated is the fact that rare patients who do not have an appreciable rash at the time present with weakness We have seen some patients with histopathological
features characteristic of DM who have developed the rash months or years after onset of weakness or not at all (adermatopathic DM or DM sine dermatitis) These patients would be erroneously classi ed as PM using Bohan and Peter criteria12,13,15 We suspect that there is a spectrum of DM Most patients have both skin and muscle involvement, but on either end of the spectrum are rare patients who have only muscle or skin involvement The classical skin manifestations include a purplish discoloration of the eyelids (heliotrope rash) often associated with periorbital edema and a papular, erythematous rash over the knuckles (Gottron papules) (Fig 30 1)22 In addition, an erythematous, macular, sunsensitive rash may appear on the face, neck and anterior chest (V-sign), shoulders and upper back (shawl sign), hips (holster sign), and extensor surfaces of elbows, knuckles, knees, and malleoli (Gottron sign) The nail beds often have dilated capillary loops occasionally with thrombi or hemorrhage The skin lesions can be subtle at
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