A Rare Manifestation of a Rare Disease: The Importance of Thinking Outside the Box in a Patient With Complex Dermatomyositis

Milne, Megan; Sims, Catherine; Anderson, Daren; Johannemann, Andrew; Leverenz, David; Criscione-Schreiber, Lisa G; Ardalan, Kaveh

doi:10.1002/acr.24905

Cited by 3 publications

(2 citation statements)

References 30 publications

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“…In patients with significant gut involvement, when selecting a steroid-sparing medication, parental dosing (such as subcutaneous methotrexate) may be preferable. 44 Because muscle enzymes are an imperfect biomarker, and JDM flares can occur in the absence of rising muscle enzymes, pediatric rheumatologists use a validated tool to monitor disease activity: such as Manual Muscle Testing-8 (MMT8) and the Childhood Myositis Assessment Scale (CMAS). The CMAS is administered by the clinician or physical therapist routinely and consists of 14 maneuvers, each of which is scored on a scale from 0 to 4.…”

Section: Juvenile Dermatomyositismentioning

confidence: 99%

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Transition to Adult Rheumatology Care: A Disease-Specific Guide

Cintron,

Chang,

Sadun

2024

J Clin Rheumatol

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Young adults with childhood-onset rheumatic diseases are more frequently establishing and continuing care with adult rheumatologists. The transfer of care can be challenging for both the young adult patients and their adult rheumatologists, in large part due to differences between pediatric-onset rheumatic diseases and their adult-onset counterparts, or due to the rarity of some pediatric-onset rheumatic conditions. Other challenges are due to cultural differences between pediatric and adult medical care and to the young adult needing to increasingly perform self-management skills that were previously managed by parents or other caregivers. In this review, we will provide a summary of strategies for working effectively with young adults as they transition to adult care. We will then discuss a subset of childhood-onset rheumatic diseases—including juvenile idiopathic arthritis, localized scleroderma, autoinflammatory diseases, pediatric-onset systemic lupus erythematosus, juvenile-onset dermatomyositis, and autoimmune encephalitis—for which clinical manifestations, management, and prognosis frequently differ between pediatric onset and adult onset. Our aim is to highlight differences that make caring for this population of transitioning young adults unique, providing tools and knowledge to empower the adult rheumatologist to care for these young adults in ways that are evidence-based, effective, efficient, and rewarding.

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Section: Juvenile Dermatomyositismentioning

confidence: 99%

“…If intestinal vasculopathy is worsening, aggressive treatment is warranted, including high-dose IV steroids (often pulse doses) and IV immunoglobulin (IVIG). In patients with significant gut involvement, when selecting a steroid-sparing medication, parental dosing (such as subcutaneous methotrexate) may be preferable 44 …”

Section: Disease-specific Carementioning

confidence: 99%

Transition to Adult Rheumatology Care: A Disease-Specific Guide

Cintron,

Chang,

Sadun

2024

J Clin Rheumatol

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Nailfold microscopy in adult-onset dermatomyositis in association with myositis antibodies

Flatley,

Collins,

Lukowiak

et al. 2024

Arch Dermatol Res

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Dermatomyositis (DM) is an immune mediated inflammatory disease classically associated with muscular and cutaneous involvement. Existing studies have suggested characteristic nailfold findings may be observed in DM, indicating a potential role for nailfold microscopic examination in the diagnosis of DM. To that end, we performed a systematic review of literature pertaining to nailfold microscopic, capillaroscopic, and dermoscopic findings observed in patients with DM, with a secondary review of the association of nailfold microscopic findings with myositis-specific antibody (MSA) and myositis-associated antibody (MAA) status. Thirty-seven papers, representing 346 patients, met inclusion criteria. The most prevalent nailfold findings were evidence of increased vascular diameter (64.5%, n = 223) and decreased vascularity (57.5%, n = 199). Scleroderma-spectrum pattern and microhemorrhage or hemorrhage were both observed in 156 (45.1%) patients. Fifty-one patients had nailfold capillaroscopic findings reported in direct association with antibody positivity and demonstrated a range of nailfold findings, preliminarily suggesting associations between antibody-status and capillaroscopic findings. The results of our study align with existing data indicating the scleroderma-spectrum pattern and the pattern’s composite features are prevalent in patients with DM. Our review further demonstrates a variety of nailfold capillaroscopic findings are found in patients positive for MSAs and MAAs.

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Hydroxychloroquine/immunosuppressants

2023

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A Rare Manifestation of a Rare Disease: The Importance of Thinking Outside the Box in a Patient With Complex Dermatomyositis

Abstract: A 26-year-old man with dermatomyositis (DM) presented to the emergency department of an academic medical center with acute abdominal pain. He described diffuse abdominal pain since his initial diagnosis of DM a year prior.

Cited by 3 publications

References 30 publications

Transition to Adult Rheumatology Care: A Disease-Specific Guide

Transition to Adult Rheumatology Care: A Disease-Specific Guide

Nailfold microscopy in adult-onset dermatomyositis in association with myositis antibodies

Hydroxychloroquine/immunosuppressants

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