Search

Idiopathic retroperitoneal fibrosis (IRF) is a rare disease often associated with autoimmune disorders. Whether IRF is associated with Hashimoto's thyroiditis (HT) is poorly understood and only addressed by case-reports. We evaluated the prevalence of HT in a large IRF cohort and in matched controls.We studied 73 consecutive patients with new-onset IRF and 71 controls. The association between HT and IRF was cross-sectionally evaluated in a referral center. Longitudinally, thyroid function tests were also performed. Serum concentrations of FT4, TSH, and anti-thyroperoxidase antibodies (AbTPO) were evaluated together with thyroid ultrasound (US). Lymphocytic infiltrates were characterized in thyroid nodule fine needle aspirates (FNAB). In patients undergoing thyroidectomy, thyroid histology was also reviewed.A higher prevalence of AbTPO positivity (P0.03) and US findings suggestive of autoimmune thyroiditis (US-AIT) (P0.0001) were found in IRF patients compared to controls. In the logistic regression analysis, the risk of AbTPO-diagnosed HT and that of US-AIT was significantly higher in IRF patients than in controls (ORs, 3.56, 95% CI 1.48-8.59, P=0.004 and 4.74, 95% C.I., 2.34-9.61, P0.0001 in AbTPO-diagnosed HT and US-AIT, respectively). Thyroid histology in IRF patients showed either classical or the fibrous variant of HT. At the end of the follow-up (median, 45 and 36 months in patients and controls, respectively), 25% of IRF patients and 3% of controls were receiving l-thyroxine.IRF patients have a higher risk of HT compared to controls. Thyroid function should be monitored in patients with IRF.

Retroperitoneal fibrosis (RPF) is an uncommon disease characterized by a fibrous reaction that takes place in the peri-aortic retroperitoneum and often entraps the ureters causing obstructive uropathy. RPF is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic disorders such as Erdheim–Chester disease. Idiopathic RPF is an immune-mediated disease, which can either be isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibro-inflammatory disorder recently renamed as IgG4-related disease. The differential diagnosis between idiopathic, IgG4-related and secondary RPF is crucial, essentially because the therapeutic approaches – especially of idiopathic vs. secondary RPF – can be dramatically different. This review focuses on the clinical, laboratory and imaging features of the different RPF forms, and also provides an overview of the available treatment options.

Pretransplant donor biopsy (PTDB)-based marginal donor allocation systems to single or dual renal transplantation could increase the use of organs with Kidney Donor Profile Index (KDPI) in the highest range (e.g. >80 or >90), whose discard rate approximates 50% in the United States. To test this hypothesis, we retrospectively calculated the KDPI and analyzed the outcomes of 442 marginal kidney transplants (340 single transplants: 278 with a PTDB Remuzzi score

Objectives We report a case of orofacial granulomatosis successfully treated with low-level laser therapy (LLLT). Study design LLLT was delivered through a diode laser device (Lasemar Eufoton, Trieste, Italy) with the use of a 4-cm defocalized lens and power of 1 W. Treatment was administered in sessions of 5 irradiations of 1 minute each, with a 1-minute interval between 2 subsequent irradiations (power density 0.08 W/cm 2 ; fluence/application: 4.8 J/cm 2 ; fluence/session: 24 J/cm 2 ). Laser therapy was repeated 12 times (3 times per week). Results After 2 weeks of LLLT applications, the patient reported an improvement of symptomatology as well as a decrease of labial swelling. Complete healing was observed after 1 month. The patient was followed for 2 years. No recurrence of swelling was observed during the follow-up. Conclusions Advantages of the LLLT approach include an absence of side effects, analgesic properties (usually reported after first application), and good patient compliance.

Background context Fungal spondylodiscitis is often because of Candida albicans or other common Candida species, whereas unusual strains such as Candida sake are often thought to be nonpathogenic. Purpose To report the first case of spondylodiscitis caused by C sake and its outcome after antimycotic therapy; as the disease occurred in a patient undergoing hemodialysis (HD), we also discuss the potential conditions related to the uremic state and to HD itself, which may predispose to spondylodiscitis. Study design/setting Case report. Methods Report of the patient's clinical findings and review of the literature concerning spondylodiscitis in HD patients and infections caused by C sake . Results The patient was a 48-year-old woman with end-stage renal disease (ESRD) undergoing HD who presented with back pain; spine computed tomographic (CT) scans showed lumbar spondylodiscitis with large bilateral abscesses in the psoas muscles, with an imaging appearance resembling that of Pott's disease. Surprisingly, C sake was isolated from the cultures of the liquid obtained by CT-guided aspiration of both abscesses, and fluconazole therapy was strikingly effective in inducing abscess regression and healing of spondylodiscitis. Conclusions In patients with chronic disorders such as ESRD, spondylodiscitis can also be caused by rare fungal strains that are usually thought to be nonpathogenic; a correct diagnostic workup is essential, as such forms can promptly respond to common antimycotic agents.

Central nervous system (CNS) lymphoma is a rare posttransplant lymphoproliferative disorder (PTLD), which usually has a poor outcome. To date, no specific conditions predisposing to this complication have been identified. We here describe the case of a renal transplant patient who was initially diagnosed as having Epstein-Barr virus (EBV)-associated leukoencephalopathy and ultimately developed EBV-positive CNS lymphoma. The patient was a young lady who, 2 years after transplantation, presented with focal neurological and electroencephalographic abnormalities and diffuse white matter lesions on brain magnetic resonance imaging. EBV-DNA was detected in the cerebrospinal fluid (CSF) by polymerase chain reaction. After acyclovir therapy and immunosuppressive drug tapering, the symptoms and electroencephalographic abnormalities subsided, and EBV-DNA disappeared from the CSF. Ten years later, a bulky cerebral mass was found. After excision, a diagnosis of EBV-positive, Hodgkin-like monomorphic B-cell PTLD was made. This case illustrates the potential pathophysiological relationships between EBV infection, leukoencephalopathy and CNS lymphoma; although a long time elapsed from the initial neurological illness to CNS lymphoma, a link between these two conditions cannot be excluded. Therefore, a careful long-term follow-up of EBV-related encephalopathy is advisable.

BACKGROUND: Churg-Strauss syndrome (CSS) is a rare disorder characterized by asthma, eosinophilia, and systemic vasculitis. Renal involvement is not regarded as a prominent feature, and its prevalence and severity vary widely in published reports that usually refer to small series of selected patients. METHODS: We examined the prevalence, clinicopathologic features, and prognosis of renal disease in 116 patients with CSS. RESULTS: There were 48 men and 68 women with a mean age of 51.9 years (range, 18 to 86 years). Signs of renal abnormalities were present in 31 patients (26.7%). Rapidly progressive renal insufficiency was documented in 16 patients (13.8%); urinary abnormalities, 14 patients (12.1%); and chronic renal impairment, 1 patient. There were 3 additional cases of obstructive uropathy. Sixteen patients underwent renal biopsy, which showed necrotizing crescentic glomerulonephritis in 11 patients. Other diagnoses were eosinophilic interstitial nephritis, mesangial glomerulonephritis, and focal sclerosis. Antineutrophil cytoplasmic antibody (ANCA) was positive in 21 of 28 patients (75.0%) with nephropathy versus 19 of 74 patients without (25.7%; P < 0.001). In particular, all patients with necrotizing crescentic glomerulonephritis were ANCA positive. After a median follow-up of 4.5 years, 10 patients died (5 patients with nephropathy) and 7 patients developed mild chronic renal insufficiency. Five-year mortality rates were 11.7% (95% confidence interval, 3.9 to 33.3) in patients with nephropathy and 2.7% (95% confidence interval, 0.7 to 10.7) in those without (P = 0.10). CONCLUSION: Renal abnormalities are present in about one quarter of patients with CSS. The prevailing picture is ANCA-associated necrotizing crescentic glomerulonephritis; however, other forms of nephropathy also may occur. Outcome and long-term follow-up usually are good.

Background Chronic periaortitis includes idiopathic retroperitoneal fibrosis, inflammatory aneurysms of the abdominal aorta, and perianeurysmal retroperitoneal fibrosis. It is considered to be due to advanced atherosclerosis, but is often associated with systemic autoimmune disorders. Methods We studied 16 consecutive patients who were diagnosed with chronic periaortitis by computed tomography. Each patient underwent a physical examination, routine laboratory tests, measurement of autoantibodies, thyroid echotomography, and chest radiography. Aortic wall or periaortic retroperitoneal samples from 9 patients who underwent surgery were available for histologic examination and immunohistochemical characterization of the inflammatory infiltrate. Results Twelve patients had constitutional symptoms, 14 had an elevated erythrocyte sedimentation rate, and 13 had an elevated C-reactive protein level. Antinuclear antibodies were positive in 10 patients. Three patients had autoimmune thyroiditis, and 1 had seropositive rheumatoid arthritis. Antineutrophil cytoplasmic antibodies were positive in 3 patients who presented with rapidly progressive renal failure. Pathologic examination of the aortic and periaortic specimens revealed moderate to severe inflammatory infiltration, mainly consisting of B cells and CD4 + T cells. Vasculitis with fibrinoid necrosis involving the aortic vasa vasorum and the small and medium retroperitoneal vessels was found in seven of the nine histologic samples. Conclusion These clinical and pathologic features support the hypothesis that, at least in some patients, chronic periaortitis is a systemic autoimmune disease, perhaps involving a vasculitic process of small and medium vessels.

The figures illustrate the computed tomographic findings in 2 patients with idiopathic retroperitoneal fibrosis (IRF) who developed obstruction of the renal veins and peri-renal collateral circles late in the course of their disease. The involvement ab extrinseco of the renal veins is an overlooked feature of IRF. It often progresses slowly and only rarely leads to clinically overt thrombosis; collateral circles thus develop and, as in our patients, may be a late manifestation of the disease.