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P.212 From normal to active scleroderma pattern: nailfold videocapillaroscopy as a mirror of disease progression in systemic sclerosis

jsrd · 2026-06-05 · canonical JSON source

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Introduction Systemic sclerosis (SSc) is a complex autoimmune connective tissue disorder characterized by vascular dysfunction, immune activation, and progressive fibrosis. Nailfold videocapillaroscopy (NVC) has emerged as a valuable diagnostic and monitoring tool, providing insights into microvascular changes that often precede clinical manifestations. The evolution of capillaroscopic patterns may reflect disease activity and predict organ involvement. We present a case of rapidly progressive SSc where serial NVC examinations closely paralleled clinical deterioration and immunological changes.Material and Methods A case report of a patient followed at our Rheumatology clinic.Results A 62-year-old man was referred to Rheumatology for a 2-month history of symmetric polyarthritis affecting wrists and small hand joints, partially responsive to high-dose corticosteroids, generalized hand swelling, and Raynaud’s phenomenon. Physical examination revealed facial telangiectasias and minimal skin thickening distal to proximal interphalangeal joints. Initial investigations demonstrated mildly elevated erythrocyte sedimentation rate (41 mm/h) and ANA 1/640 speckled pattern, while extended autoimmune panel, including SSc-specific antibodies, was negative. Baseline NVC was normal. Treatment with oral methotrexate 10mg weekly and vasodilator therapy resulted in mild clinical improvement despite poor therapeutic compliance.Approximately eighteen months later, the patient developed worsening hand swelling with more pronounced sclerodactyly, facial skin thickening, and microstomia. Repeat laboratory studies revealed strong anti-Scl70 antibody positivity, and concurrent NVC showed isolated haemorrhages in the right 4th finger.Six months subsequently, skin thickening progressed to involve the forearms, accompanied by increased telangiectasias. The patient complained of fatigue, weight loss and worsening inflammatory arthralgias despite successive methotrexate dose escalations. Cardiopulmonary assessment revealed nonspecific interstitial pneumonia-pattern interstitial lung disease on high-resolution chest CT. Repeat NVC demonstrated megacapillaries in 4th and 5th fingers bilaterally and hemorrhages involving left 3rd to 5th fingers and right 4th and 5th fingers, consistent with active scleroderma pattern. Following multidisciplinary discussion, rituximab therapy was initiated.Conclusions This case demonstrates rapid SSc progression with concurrent immunological evolution from seronegative to strongly anti-Scl70 positive status. Serial NVC examinations provided objective evidence of microvascular deterioration, transitioning from normal to active scleroderma pattern in parallel with clinical worsening and organ involvement. The correlation between capillaroscopic findings and disease progression underscores NVC’s value as a non-invasive monitoring tool in SSc management, potentially guiding therapeutic decisions and predicting organ complications in rapidly progressive disease.Abstract P.212 Figure 1Photographs from Inspectis@ Nailfold Digital Capillaroscopy illustrating the evolution of videocapillaroscopic changes in the patient. From left to right: A - right 4th finger, without relevant abnormalities (May/2023); B - right 4th finger, with microhemorrhages (Dec/2024); C and D- right 4th finger and left 5th finger, with microhemorrhages, dilated capillaries, and megacapillaries (May/2025)