Monitoring the microcirculation in the diagnosis and follow-up of systemic sclerosis patients: Focus on pulmonary and peripheral vascular manifestations.

Systemic sclerosis (SSc) is a connective tissue disease, characterized by vascular damage and progressive fibrosis, affecting the skin and internal organs. The vascular changes include functional and structural abnormalities in the microcirculation, which play a central role not only in diagnosis but also in the prognosis and follow-up of systemic sclerosis patients. Nailfold videocapillaroscopy (NVC) is a safe, validated, noninvasive, inexpensive, reliable, and reproducible method that allows for the evaluation of structural changes in scleroderma microangiopathy. However, capillary blood flow/perfusion cannot be measured by NVC under standard conditions and, consequently, must rely on various laser techniques and thermography for the assessment and quantification of cutaneous blood perfusion. Other emerging technologies, such as optical Doppler tomography and spectroscopy, may be used to evaluate the skin flow. This review updates current knowledge on the use of microvascular evaluation techniques in SSc, including complications such as digital ulcers and pulmonary arterial hypertension.

Nephrogenic systemic fibrosis: an unusual scleroderma-like fibrosing disorder.

Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder, recently described in patients with advanced chronic kidney disease, usually after exposure to gadolinium (Gd)-based contrast agents, characterized by progressive fibrotic involvement mainly of the skin. At clinical examination, the cutaneous findings of NSF may partly resemble those of systemic sclerosis. However, the different topographic distribution of the skin thickening and hardening, usually involving the limbs and trunk, whilst sparing the face, the lack of serologic abnormalities and the distinctive histopathological findings allow this new disease entity to be distinguished from systemic sclerosis and other scleroderma-like fibrosing disorders (scleromyxedema, scleredema, eosinophilic fasciitis, etc.). Herein, we describe what, to best of our knowledge, is the first, biopsy proven, Italian case of NSF, which highlights the issue of the differential diagnosis between NSF and scleroderma-like fibrosing disorders.