A Cross-sectional Comparison of Magnetic Resonance Imaging Findings and Clinical Assessment in Patients With Morphea

Abstract

scale) as reported by both physicians and patients, and the skin domains of both the Functional Assessment of Cancer Therapy-Bone Marrow Transplant (FACT-BMT; 0-4 scale for 47 items [maximum score, 188]) and Lee Symptom Scale (LSS; 0-4 scale for 5 categories [maximum score, 20]) as reported by patients. Results |The cohort consisted of 7 patients with NoGVHDP−, 9 with NoGVHDP+, 9 with ET, 8 with ST, and 13 with ET/ST. Clinical Severity Score responses were available for 34 patients (28 adult and 6 pediatric), FACT-BMT responses for 31 adults, and LSS responses for 34 adults (Tables 1 and 2). 3 Patients with ET/ST reported the greatest clinical severity (mean, 7.0/10) on CSS, the most skin bother (mean, 3.6/4) on FACT-BMT, and the highest total LSS (14.0/20). Using the CSS, physicians overall rated patients with skin of color to be less severely affected than patients rated themselves (mean, 2.8/10 vs 3.7/10). In particular, self-rated severity of NoGVHDP+ was higher than the physician-rated severity (mean, 2.4/10 vs 0.1/10). Despite having no active cGVHD, the NoGVHDP+ group self-rated their skin disease as more severe than patients with ST (mean, 2.4/10 vs 1.1/10).The total FACT-BMT scores were similar among patients with NoGVHDP+ (mean, 101.0/188) and those with ST (mean, 108.0/188). Similarly, in all LSS categories (color, rash, thickness, sores, and itch), patients with ET were more bothered by their disease than patients with ST (mean, 13/20 vs 5.4/20).

Morphea: progress to date and the road ahead

“…In fact, studies have shown that MRI can reveal clinically occult musculoskeletal involvement (82)(83)(84), demonstrating subclinical extension of lesions beyond visible margins (41). MRI has also been shown to demonstrate active disease that would otherwise be misclassified as inactive based on using the LoSCAT alone (41). Given how crucial it is to accurately assess activity when managing morphea patients, this further underscores the utility of MRI in conjunction with clinical evaluation for deep morphea.…”

“…Deep morphea lesions often have very subtle surface changes, and palpation can be more important than visual inspection to appreciate the extent of these lesions. It also may be difficult to fully evaluate activity in these deeper lesions, and given that unchecked morphea activity can lead to permanent functional sequelae, patients with these deeper manifestations of morphea may benefit from MRI to determine disease activity and damage (40,41). Although involvement of joints and areas underlying areas of morphea is the most common presentation, patients may also experience sacroiliitis, generalized synovitis, and inflammatory arthritis.…”

“…Recent studies have indicated that MRI may have a role as an objective outcome measure in morphea, particularly in the case of deep cutaneous or soft tissue involvement. High resolution MRI can demonstrate inflammation, sclerosis, and atrophy in morphea lesions, and has been shown to be a useful adjunct to clinical examination when it comes to accurate assessment of disease activity and extent (41). Figure 3 demonstrates MRI findings in morphea.…”

“…This is particularly relevant for deeper morphea lesions extending to the subcutis, fascia, and muscle, which often present with subtle cutaneous manifestations. In fact, studies have shown that MRI can reveal clinically occult musculoskeletal involvement (82)(83)(84), demonstrating subclinical extension of lesions beyond visible margins (41). MRI has also been shown to demonstrate active disease that would otherwise be misclassified as inactive based on using the LoSCAT alone (41).…”

Imaging More than Skin-Deep: Radiologic and Dermatologic Presentations of Systemic Disorders

“…Scleroderma, or systemic sclerosis, is an autoimmune fibrosing disorder, consisting of two subsets: diffuse cutaneous systemic sclerosis (dSSc; a systemic disease characterized by widespread involvement of any organ system with a prevalence of 20/100,000 and peak incidence in females between 30 and 50 years old), and limited cutaneous systemic sclerosis (lcSSc; a disease characterized by manifestations of the CREST syndrome (calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly ( Figure 3 A), and telangiectasia). Cases without skin changes but other systemic manifestations have been reported as well and are described as systemic sclerosis sine scleroderma [ 22 , 23 , 24 ]. The skin is the main organ involved in scleroderma, and disease subsets are differentiated by the degree of skin involvement [ 23 ].…”