Background Musculoskeletal (MS) involvement and clinically evident arthritis occurs in up to 65% of patients with Juvenile Systemic or localized Scleroderma (JSc). It may be the first manifestation preceding even the onset of Raynaud or skin manifestations; patients presenting with arthritis, tenosynovitis or enthesistis may suffer from Sc. On the other hand clinical examination often underestimates MS involvement in Sc. US and MRI can help distinguish arthritis with effusion from dry tenosynovitis of Sc, define whether loss of range of motion (LOM) derives only from skin thickening or from bone and joint involvement and monitor disease progression.
Objectives To describe the spectrum of MRI and US features in juvenile scleroderma with musculoskeletal involvement.
Methods We describe MRI and color Doppler MSUS findings of clinically affected (with arthritis and/or LOM and/or overlying skin with edema or sclerosis) lower or upper extremities from 4 males and 2 females;2 with systemic sc (ssc), 2 with linear scleroderma, 1 with generalized morphea, 1 with mixed morphea (median age 8,5 years, range 7–10,5; median time from symptom onset to MRI 11 months, range 2–24). MRI sequences;T1, fluid-sensitive, and T1-FS contrast-enhanced. Comparisons were made to uninvolved areas of the extremity, and the contralateral extremity. Findings guiding diagnosis and evaluation of disease extent are depicted in the Figure.
Results Thickening of the dermis and infiltration of the subcutaneous fat with increase in signal intensity on fluid sensitive sequences and contrast-enhanced T1w images and hypointense signal lesions on unenhanced T1w images (asterisks) was apparent in 4 patients. In 2 male patients with generalized scleroderma, clinical LOM of fingers and wrists preceded skin sclerosis by 2 months. Joint and tendon sheath synovitis, indicated by initial MSUS, was detected in fluid sensitive and T1w enhanced images (white arrows). The combination of tendon-sheath synovitis and muscular fascia thickening and enhancement (black arrows), and contractures (thin white arrow) very characteristic of scleroderma, helped identify sclerodermatous musculoskeletal involvement in the absence of skin induration. Focal bone marrow edema depicted as high signal intensity in fluid sensitive sequences (curved arrow) was found in 2 cases; 1 with generalized morphea without apparent overlying skin sclerodermatous lesion, 1 with linear scleroderma with atrophic lesions in all overlying structures
Conclusions Musculoskeletal imaging features of juvenile scleroderma involving the skin, fascia, musculature and bones reflect pathomorphologic changes of this rare disorder and enable a complete assessment of the disease extent, including depth of infiltration and disease activity.In the described cases implementation of MSUS and MRI in Juvenile scleroderma led to earlier definition of the diagnosis and assisted the evaluation of disease extension.
Zulian F et al. Arthritis and Rheumatism 2007 Mar 15;57(2):203–12.
Eusler E et al. Pediatr Radiol 2017 Jan 14.
Schannz S et al. RSNA 2011.
Torok K Pediatr Clin North Am. 2012 April;59(2):381–405.
Disclosure of Interest None declared