IgG4-Related Disease: Results From a Multicenter Spanish Registry.

IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients.To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients.A retrospective multicenter study was performed. Twelve hospitals across Spain included patients meeting the current 2012 consensus criteria on IgG4-RD diagnosis.Fifty-five patients were included in the study, 38 of whom (69.1%) were male. Median age at diagnosis was 53 years. Thirty (54.5%) patients were included in the Histologically Highly Suggestive IgG4-RD group and 25 (45.5%) in the probable IgG4-RD group. Twenty-six (47.3%) patients had more than 1 organ affected at presentation. The most frequently affected organs were: retroperitoneum, orbital pseudotumor, pancreas, salivary and lachrymal glands, and maxillary sinuses.Corticosteroids were the mainstay of treatment (46 patients, 83.6%). Eighteen patients (32.7%) required additional immunosuppressive agents. Twenty-four (43.6%) patients achieved a complete response and 26 (43.7%) presented a partial response (<50% of regression) after 22 months of follow-up. No deaths were attributed directly to IgG4-RD and malignancy was infrequent.This is the largest IgG4-RD series reported in Europe. Patients were middle-aged males, with histologically probable IgG4-RD. The systemic form of the disease was frequent, involving mainly sites of the head and abdomen. Corticosteroids were an effective first line treatment, sometimes combined with immunosuppressive agents. Neither fatalities nor malignancies were attributed to IgG4-RD.

Idiopathic retroperitoneal fibrosis: a clinicopathological study in 24 Spanish cases.

Idiopathic retroperitoneal fibrosis (IRPF) is a rare condition of unknown aetiology characterized by chronic non-specific inflammation of the retroperitoneum. The aim of this study is to describe, for the first time, the features of Spanish patients with IRPF. In this retrospective study, a clinical description was performed to examine the histopathological features, radiologic findings, laboratory data and treatment of a cohort of 24 IRPF Spanish patients who were admitted to Vall d'Hebron Hospital in Barcelona (Spain) between 1982 and 2009. Patients with secondary retroperitoneal fibrosis were excluded. Nineteen patients (79.1 %) were male, whereas 5 were female. The mean age at diagnosis was 51.8 ± 16.4 years. Pain (79.1 %) and hydronephrosis (70.8 %) were the most common clinical manifestations. Abdominal computed tomography and ultrasonography were performed on all cases. Acute-phase reactants were elevated in most patients. Thirteen surgical biopsies were performed, and all were consistent with retroperitoneal fibrosis. Steroid therapy was given in 19 cases, and eight patients (42.1 %) required urethral catheterisation. Chronic renal failure (CRF) rate after 2 years of follow-up was 42.8 %, more frequently in patients with higher serum creatinine at diagnosis. IRPF in Spain is a rare condition affecting mainly middle-aged males, with pain and hydronephrosis as the most frequent manifestations. Steroids are the mainstay treatment, and CRF is the main sequel. An earlier diagnosis and uniform treatment protocols could prompt lower CRF rates.

Multilocular cystic renal cell carcinoma : a report of 45 cases of a kidney tumor of low malignant potential.

The 2004 World Health Organization (WHO) classification of kidney tumors recognizes multilocular cystic renal cell carcinoma (MCRCC) as a rare variant of clear cell renal cell carcinoma with a good prognosis. Available information on its clinical significance is limited. The study cohort included 45 MCRCC cases classified according to 2004 WHO criteria obtained through a multi-institutional international search. Most patients had unilateral MCRCC with no side predominance that was found incidentally; 62% were men, but women had tumors at an earlier age (P = .385). MCRCC occurred slightly more often in men than in women (1.7:1). At diagnosis, 82% of patients had stage T1 and 16%, stage T2; 1 patient had stage T3. The Fuhrman grade was 1 (62%) or 2 (38%), with smaller tumors (