Dermatomyositis with normal muscle enzyme concentrations. A single-blind study of the diagnostic value of magnetic resonance imaging and ultrasound

Stonecipher, Marcus R.; Jorizzo, Joseph L.; Monu, Johnny U. V.; Walker, Francis O.; Sutej, Paul G.

doi:10.1001/archderm.130.10.1294

Cited by 55 publications

(34 citation statements)

References 27 publications

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“…US revealed hyperechogenicity and MRI demonstrated high signs on T2-weighted images in several muscle groups of the patients with active myositis. 27 Limited data are available from studies correlating clinical findings and US assessment for the monitoring of patients with IIM. The first longitudinal study published evaluated by US 12 patients with juvenile DM and PM with mean disease duration of 10 years.…”

Section: Discussionmentioning

confidence: 99%

Relevance of ultrasonography in assessing disease activity in patients with idiopathic inflammatory myopathies

et al. 2017

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Aim: Idiopathic inflammatory myopathies (IIM) comprise a group of rare and heterogeneous diseases difficult to diagnose and follow up. Precise measures for assessing disease activity are not available at the moment. Our objective was to evaluate the usefulness of ultrasonography (US) as a monitoring tool in IIM. Method:The study evaluated IIM patients diagnosed and followed up from 2005 to 2015 in our department. Fifteen patients with a mean age of 52.2 AE 22.09 years and mean disease duration of 4.6 AE 3.20 years were included. Physical examination including muscle strength tests, laboratorial analysis and a selective muscle US assessment were performed for each patient at a scheduled visit.Results: Nine of the 15 patients were in clinical remission and US assessment revealed a preserved muscle pattern. Symmetrical proximal muscle atrophy was found on US in one patient with longstanding polymyositis (PM) with proximal weakness. Inflammation and focal or generalized muscle edema were present on US in the remaining five patients with muscular weakness suggesting active disease. One of these patients in acute flare presented with atrophy changes in addition to edema. Early untreated myositis presented in one patient with moderate power Doppler (PD) signal. Conclusion:As far as muscle US assessment is concerned, a single specific pattern was not observed in our study. A mixture of muscle edema and atrophy was detected depending on disease activity and duration. US findings seem to correlate well with disease activity, suggested by clinical data, and may be a useful tool to complement patient evaluation.

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Section: Discussionmentioning

confidence: 99%

Relevance of ultrasonography in assessing disease activity in patients with idiopathic inflammatory myopathies

et al. 2017

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“…Even in patients with normal results on EMG and muscle biopsy, muscles may be affected, as can be demonstrated by ultrasound, magnetic resonance imaging, and P-31 magnetic resonance spectroscopy. While metabolic deficiencies are detectable during both rest and exercise in paticnts with myopathic DM, these metabolic deficiencies may become apparent only during exercise in patients with ADM (21)(22)(23).…”

Section: Discussionmentioning

confidence: 99%

A young woman with a photosensitive pruritic rash on her face and upper trunk

Zuber

John

Pfreundschuh

et al. 1996

Arthritis & Rheumatism

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A 38-year-old woman prcscntcd in March 1991 with a violaceous periorbital rash ( Figure 1) and an erythematous rash on her facc, uppcr trunk, arms, and legs, which was intermittently pruritic. She denied having muscle weakness, myalgias, arthralgias, or dyspnea on exertion. The patient did not have any symptoms involving other organs. On examination, cruptions over the dorsa of hcr hands and Gottron's papules on the knuckles and proximal interphalangeal joints (Figures 2 and 3) wcrc dctcctcd. Proximal and distal musclc strength was normal. Electromyography (EMG) showcd no evidence of an inflammatory myopathy. Thc crcatinc kinasc Icvcl, aldolasc level, crythrocyte sedimentation rate, and C-reactive protein level were all within normal limits. Antinuclear antibodies (ANA) wcrc present at a titer of 1:160 with a speckled pattern. Antibodies to Sm, small nuclcar RNP (snRNP), SS-A, SS-B, doublcstranded DNA (dsDNA), Scl-70, and PM-Scl were all absent.Skin biopsy rcvcaled a superficial perivascular lymphocytic infiltrate with vacuolar interface changes. Necrotic, apoptotic keratinocytes were present along the epidermal basement mernbranc zonc. A slight cpidcrmal hypcrplasia with focal atrophy and compact orthokeratosis were also present. No musclc biopsy was pcrformed bccausc of the lack of specific symptoms. Investigation for a tumor in the lungs, mammae, stomach, and colorectum yielded negative results. Therapy and clinical courseTherapy with prednisolonc at 40 mgiday was begun, which improved the skin rash rcmarkably. The Submitted for publication July 3, 1995; accepted in revised form March 13, 1996. dosage was tapered to 10 mgiday. During the last 5 years thc following stcroid-sparing agents were administered consecutively, along with the prcdnisolonc: hydroxychloroquinc 100 mgiday, azathioprine 150 mgiday, and dapsone 100 mg/day. However, each time the daily prednisolone dosage was reduced to -6 -1 0 mg, the disease flared; this happened an average of twice per year. Sunscrccns and topical steroids alone did not control the disease. After 4M years of disease, the paticnt does not have any symptoms of muscle weakness or myalgias. She is doing well and participates in sports twice a week. DiscussionThe symptoms of a violaceous periorbital rash, crythcmatous rash on the face, and Gottron's papules on Figure 1. Erythematous rash and violaceous periorbital rash in the patient, a 38-year old woman.

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“…The lack of muscle weakness rather than the lack of laboratory abnormalities has been proposed as the primary criterion for ADM [6]. Some patients with myopathic DM have normal levels of serum muscle enzymes, but muscle inflammation in these patients can be detected with magnetic resonance imaging (MRI) or ultrasound [20,21]. Although weakness is absent in amyopathic patients, fatigue is reported by at least 50% of patients [6,22] and must be distinguished from frank weakness.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Four of these 13 patients had abnormal biopsy results consistent with DM and eventually developed clinically apparent myopathy. The imaging modalities of ultrasound and MRI offer noninvasive alternatives that are useful in the evaluation of muscle disorders [20,21]. These approaches have been applied to studies in patients with ADM.…”

Section: Anatomic and Histologic Abnormalities In Skin And Musclementioning

confidence: 99%

“…The period of follow-up was not noted; it is possible that some of these patients eventually developed clinically apparent weakness. MRI and ultrasound were used in another study to examine seven patients with ADM [21]. Ultrasound scans were abnormal in two patients and MRI scans showed inflammation in four patients.…”

Section: Anatomic and Histologic Abnormalities In Skin And Musclementioning

confidence: 99%

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Amyopathic dermatomyositis

Olsen¹,

Park²,

KingJr³

2001

Curr Rheumatol Rep

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Amyopathic dermatomyositis is a variant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities. It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the observation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensitive measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although juvenile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur. Determining whether a patient has amyopathic rather than myopathic disease may have prognostic implications.

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Dermatomyositis with normal muscle enzyme concentrations. A single-blind study of the diagnostic value of magnetic resonance imaging and ultrasound

Cited by 55 publications

References 27 publications

Relevance of ultrasonography in assessing disease activity in patients with idiopathic inflammatory myopathies

Relevance of ultrasonography in assessing disease activity in patients with idiopathic inflammatory myopathies

A young woman with a photosensitive pruritic rash on her face and upper trunk

Amyopathic dermatomyositis

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