Rodolfo Puccini

A case of adult periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome associated with endocapillary proliferative glomerulonephritis

PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously. The endocapillary proliferative glomerulonephritis (EPG) is a glomerular injury characterized by hypercellularity in glomerular lumen and is caused by post-infectious or autoimmune diseases. In this paper, we describe the case of a 35-year-old man affected by PFAPA and EPG. To our knowledge this association has never been reported in the literature before.

PROTEINURIA IN FOCAL SEGMENTAL GLOMERULOSCLEROSIS: ROLE OF CIRCULATING FACTORS AND THERAPEUTIC APPROACH

The clinical course of primary Focal Segmental Glomerulosclerosis (FSGS) is frequently complicated by nephrotic range proteinuria and progression to renal failure. The high recurrence rate of the disease in transplanted kidney suggests the hypothesis that such patients have a circulating factor that alters glomerular capillary permeability. In recent years some authors found that serum from patients with FSGS increases glomerular permeability to albumin and partially identified the permeability factor (PF) as a protein of 30–50 Kd m.w. The removal of this protein by means of Plasma Exchange (PE) or plasma Immunoadsorption by Protein A (IA) decreased proteinuria. In this report we provide preliminary data about the prevalence of PF and the therapeutic effect of its removal by IA, in 3 pts with recurrence in the transplanted kidney, and 4 with FSGS of the native kidneys. They were resistant to corticosteroids (CS) and immunosuppressive (IS) therapy. 10 IA sessions were performed in 4 weeks: if a remission was achieved IA was gradually tapered. The level of PF in the serum was measured by an in vitro assay to determine the glomerular permeability to albumin. The FSGS was histologically proven in all cases and the degree of evolution was evaluated. PF levels, serum creatinine, daily proteinuria and serum albumin were monitored. The 3 patients with recurrent FSGS had a normalization of the PF levels; 2 had a clinical remission. In FSGS of native kidneys PF was elevated in 3/4 cases; 1 had a clinical remission; 2 with extensive sclerohyalinosis and 1 without PF levels did not improve. Our results confirm that most patients with FSGS have high PF serum levels and suggest that its removal can be beneficial.

Acute and Subacute Effect of Rheopheresis on Microvascular Endothelial Function in Patients Suffering From Age‐related Macular Degeneration

This study was performed on seven patients affected by the atrophic form of age‐related macular degeneration (AF‐ARMD). The patients under investigation belonged to a larger study aimed at evaluating the efficacy of rheopheresis treatment (RT) on the visual function of AF‐ARMD patients. Following the protocol of the larger study, patients received RT twice a week, every two weeks, for a total of ten treatments, as well as high‐dose supplementation with zinc and vitamins A, E and beta‐carotene. Recruited patients underwent skin laser Doppler flowmetry coupled with skin iontophoresis of the endothelium‐dependent vasodilator acetylcholine (ACh) and a test of skin post‐ischemic reactive hyperemia, before and after the first RT (time 1: all seven patients) and the fifth RT (time 2: six patients). A significantly higher absolute (anova for repeated measures) and relative (percentage change from the baseline) skin blood flux response (SBFR) to ACh iontophoresis was observed after RT, compared to before RT at time 1 (679 ± 43% and 436 ± 78%, respectively; P < 0.05), as well as before RT at time 2 compared to before RT at time 1 (683 ± 74% and 436 ± 78%, respectively; P < 0.05). Absolute and relative SBFR to ischemia did not differ either after RT compared to before RT at time 1, or before RT at time 2 compared to before RT at time 1. These findings are consistent with an acute and subacute beneficial effect of RT on skin microvascular endothelial function in the studied AF‐ARMD patients.

Staphylococcus protein A-based extracorporeal immunoadsorption and thalidomide in the treatment of skin manifestation of dermatomyositis: a case report.

Cutaneous involvement in dermatomyositis can be the prevalent component of disease, failing to respond to adequate therapies for myositis. In this case report, we describe a patient affected by dermatomyositis, characterized by prevalent skin involvement, successfully treated with Staphilococcus protein A‐based extracorporeal immunoadsorption (Immunosorba, Fresenius Medical Care AG & Co. KGaA, Bad Homburg, Germany) and thalidomide. The patient showed panniculitic ulcerative lesions of the skin, difficult to treat because of side effects or ineffectiveness of various therapies. Skin manifestations rapidly improved after introduction of immunoadsorption; The association of thalidomide allowed a good maintenance of these results until the remission of skin lesions, despite several infective complications of some residual ulcers. Considering the difficulties in the management of our patient, combined therapy with Immunosorba and thalidomide has allowed a good clinical response. If confirmed, the observed beneficial effects suggest that protein A‐based immunoadsorption and thalidomide can represent an alternative option in dermatomyositis, especially when cutaneous manifestations are predominant.