Lung Transplantation as Successful Treatment of End-stage Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report.

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition, characterized by predominantly upper-lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls that leads a clinical progression to respiratory failure. This condition may not be as rare as it seems to be, because nowadays the increasing awareness among specialists is raising the number of new diagnoses. Limited data are available about the prognosis, both for secondary and idiopathic forms. Nevertheless, the idiopathic form seems to be rapidly progressive and no treatment can control the disease, which is why management is challenging. Since the disease was characterized, PPFE cases have been reported in the literature, but most have been secondary rather than idiopathic. Of these, few have successfully undergone lung transplantation as a treatment of end-stage respiratory failure. We here report a successful case of a 38-year-old man affected by idiopathic PPFE who underwent bilateral lung transplantation after extracorporeal membrane oxygenation bridging for an abrupt transition to critical clinical conditions. After a complex postoperative course and a first year characterized by acute rejection, the patient is alive at 5 years with a good quality of life. Our experience confirms that lung transplantation would be a valuable treatment option in case of end-stage idiopathic PPFE cases.

[Association between serum amyloid A (SAA) in salivary glands and high levels of circulating beta 2-microglobulin in patients with Sjögren syndrome]. / Associazione fra presenza di Serum Amyloid A (SAA) nelle ghiandole salivari e alti livelli circolanti di beta 2-microglobulina in pazienti affetti da sindrome di Sjögren.

OBJECTIVES: The presence of secondary amyloidosis is a complication of different rheumatic diseases. We investigated the presence of Serum Amyloid A (SAA), marker of secondary amyloidosis, in salivary glands of patients (pts) with Sjögren Syndrome (SS) and correlated it to biohumoral parameters. MATERIALS AND METHODS: 141 pts with sicca syndrome who fulfilled 3 items of the European Criteria for SS by Vitali et al underwent biopsies of labial salivary glands, that were scored according to Chisholm and Mason index and evaluated for the presence of SAA. All pts were evaluated for ANA, ENA, rheumatoid factor, gamma-globulins, IgA, IgG, IgM, C3, C4, beta 2-microglobulin, erythrosedimentation rate, C reactive protein. RESULTS: Forty out of 141 pts, showed sialoadenitis (SL) with focus score 3-4 (definite SS), and 101 pts showed SL with focus score 1-2. Fourteen out of 101 pts (13.8%) with score 1-2 and 12/40 pts (30%) with definite SS were positive for SAA, respectively. SS pts were further divided in group A (positive for SAA) and group B (negative for SAA). These groups were compared to detect if differences could exist in biohumoral parameters: group A showed higher levels of biohumoral parameters than group B, but the difference was significant only for beta 2-microglobulin: 2653+610 ng/ml versus 1848+440 ng/ml; p< 0.025. CONCLUSION: Secondary amyloidosis is a complication of SS. In pts with SAA in salivary glands were detected high levels of beta 2-microglobulin, that could be considered a factor predicting the development of amyloidosis in SS.