Cardiac involvement in systemic sclerosis: differences between clinical subsets and influence on survival.

Cardiac involvement (CI) is a known complication of SSc associated with increased mortality. Our objective was to describe a cohort of patients with SSc and CI and to assess the differences between cutaneous subsets regarding their presentation and survival. Three hundred and ninety-three Spanish patients from a single center, diagnosed with SSc, were retrospectively studied for evidence of CI using noninvasive and invasive tests from 1976 to 2011. Clinical, epidemiological, immunological and therapeutic features of patients with CI were compared to those without it and within the different cutaneous subsets of SSc. CI was present in 173 (44 %) patients. Mitral regurgitation (67 %), conduction alterations (45 %) and left ventricle diastolic dysfunction (40 %) were the most common findings. Pericardial involvement and heart failure were more frequent in diffuse SSc (dcSSc) than in limited or sine scleroderma SSc. CI accounted for 20 % of deaths, and it was an independent mortality risk factor (HR 2.1, P = 0.02), but once CI was established, classical dcSSc mortality risk factors determined mortality. Patients with dcSSc developed CI faster than limited (HR 1.9, P = 0.003) or sine SSc patients (HR 2.5, P = 0.002), specially during the first year after SSc onset. We found statistically significant differences between the 3 SSc subsets in the presentation of pericardial involvement and heart failure. CI increased the mortality and appeared at a higher rate, especially during the first year after SSc onset. Screening for heart involvement should be performed at diagnosis and during follow-up.

IgG4-related disease: Evidence from six recent cohorts.

IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosing disorder. In this review we aim to describe and compare the characteristics of the six largest IgG4-RD cohorts, since the new 2012 consensus diagnostic criteria were released. These observational studies were published between 2012 and 2015. Patients were included using the comprehensive diagnostic criteria or the 2012 consensus criteria. Results were reviewed and summarized. Most patients were middle aged men. Fibro-inflammatory masses developed in virtually all organs except the brain, with an unexplained preference for salivary glands, lymph nodes and pancreas. Corticosteroids were the treatment of choice but up to 40% of patients relapsed within the first year. Standardized response assessment tools, biomarkers and the validation of new treatments are still in development. In conclusion, the features of IgG4-RD are similar across the globe. At the moment, corticosteroids are the only validated treatment but rituximab seems to be promising.

Fast 1.5 T chest MRI for the assessment of interstitial lung disease extent secondary to systemic sclerosis.

This study aims to evaluate the utility of magnetic resonance imaging (MRI) to assess interstitial lung disease (ILD) extent in patients with systemic sclerosis (SSc). Patients with SSc and varying degrees of ILD with a high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), and a chest MRI containing an ultrafast SE sequence performed less than 1 year apart were included in the study. Wells global disease extent and Goh's staging algorithm were used to measure and categorize ILD both for MRI and HRCT. Correlation and diagnostic performance of MRI compared with HRCT and PFTs were calculated. Eighteen SSc patients were studied. MRI showed a good performance to detect ILD (AUC = 0.96) and was correlated with forced vital capacity (r = -0.60, p = 0.01), diffusing capacity of the lung for carbon monoxide (r = -0.79, p = 0.04), and also with HRCT (r = 0.85, p < 0.001), but MRI extent values were consistently lower than HRCT and, thus, not directly comparable. Goh's algorithm using HRCT and transformed to be used with MRI showed a good agreement (kappa = 0.73, p < 0.001) and MRI-measured ILD extent presented good intra-observer (ICC = 0.86) and inter-observer (ICC = 0.90) reliability. In SSc patients, MRI proved to be a good technique to detect and categorize ILD extent compared with HRCT, suggesting that it may be a valuable x-ray sparing technique for selected cases.

Pleural irregularity, a new ultrasound sign for the study of interstitial lung disease in systemic sclerosis and antisynthetase syndrome.

OBJECTIVES: To evaluate a new ultrasound sign, pleural irregularity (PI), for the study of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) and antisynthetase syndrome (ASS). METHODS: The study included patients from our SSc and ASS cohorts with varying degrees of ILD, enrolled from 2011 to 2014. Chest high-resolution computed tomography (HRCT), pulmonary function tests (FVC and DLCO) and chest sonography were performed in each patient. Ultrasound PI and B-lines were quantified using a 72-sonographic point score and HRCT lung abnormalities were quantified using Warrick and Wells scores and categorised through Goh's algorithm. PI was correlated with HRCT and pulmonary function test parameters and its diagnostic performance to detect and classify the extent of ILD was evaluated and compared with B-lines. RESULTS: Thirty-seven patients were studied, 21 with ASS and 16 with SSc (8 without ILD). PI correlated with the Warrick score both in SSc (r=0.6, p=0.01) and ASS patients (r=0.6, p=0.005), showing a higher performance to detect ILD than using B-lines (p=0.01). In SSc patients PI also correlated with Wells score (r=0.7, p<0.001) and with DLCO (r=-0.5, p=0.05), showing a high diagnostic value for detecting ILD (AUC=0.85, 95% CI 0.64-1) and classifying it into limited or extensive (AUC=0.81, 95% CI 0.57-1). A modification of the Goh algorithm including PI was developed as a screening tool to avoid the use of HRCT in SSc patients without ultrasound evidence of extensive ILD. CONCLUSIONS: PI is useful for evaluation of ILD in SSc and ASS patients, and can be incorporated into a diagnostic algorithm in SSc patients to reducing the need for exposure to ionising radiation.

Delayed diagnosis of late-onset Pompe disease in patients with myopathies of unknown origin and/or hyperCKemia.

Pompe disease is a rare metabolic myopathy whose diagnosis is sometimes delayed despite being essential for improving clinical outcomes. We aimed to investigate the prevalence of late-onset Pompe disease among patients with a myopathy of unknown etiology, including polymyositis, or with idiopathic rise of creatine kinase (CK) levels, in a department of internal medicine. A cohort study was conducted in 241 subjects: 140 patients with myopathies of unknown origin or increased CK levels, 30 with polymyositis and 71 who constituted the control group of other myopathies. Acid α-glucosidase (GAA) activity was tested in dried blood spots. If a positive result was obtained, GAA activity in isolated lymphocytes and/or genetic testing was performed as a confirmatory diagnosis. Out of the 140 investigated patients, 2 patients with myopathies of unknown origin were confirmed to be positive for Pompe disease. Thus, late-onset Pompe disease should be considered among adult patients with myopathy of unknown origin.

Etanercept in refractory lupus arthritis: An observational study.

OBJECTIVE: To investigate the long-term safety and preliminary efficacy of etanercept in patients with refractory lupus arthritis. METHODS: We evaluated 43 patients in this observational cohort study. All received etanercept (50mg/week) in addition to concomitant immunosuppressive agents. Patient and disease characteristics were collected. Incidence of adverse events and the effect on autoantibody levels were evaluated. Clinical efficacy was measured by the 28-joint count and the SLEDAI-2K scores. Remission of lupus arthritis was defined by a 28-joint score = 0. Clinically inactive systemic disease was defined by a SLEDAI-2K score <4. RESULTS: The total follow-up time was 93 patient-years (median: 2.3 years per patient; range: 0.4-6.8 years). Most side effects were minor and related to local reactions. Only 2 significant adverse events occurred (8%), both were of infectious nature. The rate of autoantibody production was low (18%). A mild increase in titres of ANA (2), IgG anti-dsDNA (3) and IgM anticardiolipin (aCL) (2) antibodies was observed. All anti-dsDNA antibody increments were transient and coincided with systemic flares. No vascular events occurred. In general, disease activity declined during therapy. Most patients (83%) with lupus arthritis achieved clinical remission by week 12. All patients with simultaneous serositis experienced clinical and radiological resolution of this condition. Relapses were frequent (23%), mostly mild and related to etanercept reduction. A total of 24 patients discontinued treatment, 12 of them due to clinical remission. CONCLUSIONS: Long-term therapy with etanercept was relatively safe and had remarkable long-term efficacy for refractory lupus arthritis. In view of these results, further controlled trials are warranted.

Identification of a novel myositis-associated antibody directed against cortactin.

OBJECTIVE: The aim of this study is to describe a novel myositis-associated autoantibody (anti-cortactin antibody) and assess related clinical and immunological manifestations and its clinical significance. METHODS: Adult patients with myositis (dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, and inclusion body myositis), as well as patients with other autoimmune diseases and non-inflammatory myopathies were analyzed for the presence of anti-cortactin antibody using in-house developed ELISA and immunoblotting techniques with a commercial source of purified cortactin. The cut-off for positive status was determined in a group of healthy volunteers. RESULTS: Antibody against cortactin was positive in 7/34 (20%) polymyositis patients, 9/117 (7.6%) dermatomyositis, 2/7 (26%) immune-mediated necrotizing myopathy, and none of the 4 patients with inclusion body myositis. The antibody also tested positive in 3/101 patients with other autoimmune diseases (2 systemic sclerosis and 1 systemic lupus erythematosus), and in 1/29 patients with non-inflammatory myopathy. No relevant association with specific clinical features was found in patients with these antibodies. Anti-cortactin antibody was more frequently positive in patients with polymyositis and immune-mediated necrotizing myopathy than in the remaining myositis patients, and was the only myositis autoantibody found in sera of 3 patients from these groups. CONCLUSIONS: Our data indicate that cortactin is a novel target antigen in patients with autoimmune diseases, especially patients with polymyositis or immune-mediated necrotizing myopathy. Anti-cortactin can be considered a new myositis-associated antibody.

Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody.

OBJECTIVE: The objective of this article was to establish the clinical course of interstitial lung disease (ILD) in scleroderma related to the presence of anti-PM/Scl antibody compared with anti-Scl-70 in a Spanish cohort. Furthermore, no study has thoroughly investigated the outcome of pulmonary function test in the first group of patients. METHODS: A total of 63 Spanish patients with scleroderma and ILD were selected in a retrospective observational study. Among them, 14 were positive for anti-PM/Scl antibodies and 49 for anti-Scl-70. Clinical assessments, including pulmonary function test, were collected. Variations equal or greater than 10% in forced vital capacity (FVC) were considered significant. Progression-free survival of disease was defined as the period of stable illness since pulmonary fibrosis diagnosis. RESULTS: Anti-Scl-70 patients had a higher frequency of diffuse SSc subset, peripheral vasculopathy, and gastrointestinal involvement. Inflammatory myopathy was associated to anti-PM/Scl antibody. Anti-PM/Scl patients presented more improvement in FVC during follow-up, 30.8% compared to a 7.1% in Scl-70 group (P = 0.04), with less worsening of this parameter (15.4% vs 52.4% in Scl-70 patients, P = 0.01), and secondary less frequency of severe restrictive pattern (FVC < 50%) (7.7% compared to 42.9% in the other group, P = 0.02). Regarding treatment, more anticalcineurinics were used in anti-PM/Scl patients, while cyclophosphamide and mycophenolate were mainly used in anti-Scl-70 patients. The progression-free survival of disease was higher in anti-PM/Scl patients, with 76% at 10 years from diagnosis of ILD against a 29% in the Scl-70 group. CONCLUSIONS: Several features and prognosis of ILD in SSc may be modified depending on the identified immunological profile.

"Pregnancy in adult-onset idiopathic inflammatory myopathy": report from a cohort of myositis patients from a single center.

BACKGROUND AND PURPOSE: Idiopathic inflammatory myopathies (IIM) are systemic diseases, characterized by the presence of an inflammatory muscle infiltrate. Although more frequent in women, its relationship with pregnancy has not been extensively studied. Our goal was to analyze the interaction between pregnancy and myositis in a cohort of IIM women from a single center. METHODS: A total of 51 patients from a historical cohort of IIM diagnosed between 1983 and 2013 were interviewed with a specific questionnaire. Comparisons between pregnancies occurring before and after the onset of the disease were performed using generalized mixed-effect models with normal and binomial distributions adjusted for confounding factors and clustering. RESULTS: A total of 102 pregnancies from 51 patients (41 with dermatomyositis and 10 with polymyositis) were analyzed. A total of 14 pregnancies from 8 patients occurred after disease onset; statistically significant (p = 0.02) clinical improvement during gestation was evident in 7 pregnancies (4 patients), 5 of them (from 2 patients) experienced a relapse of IIM symptoms afterwards, while in the rest, there was no influence of pregnancy on the disease. No disease flare associated with pregnancy was observed. Two patients were diagnosed within the first 6 months after delivery and none during pregnancy. No evidence was found to support pregnancy as a trigger for myopathy (p = 0.71). CONCLUSIONS: Pregnancy does not seem to carry a worse prognosis for the mother nor for the fetus in patients with IIM; on the contrary, nearly half of the patients in our series improved clinically when they became pregnant, a relapse of IIM symptoms being common afterwards. Pregnancy does not appear to be a trigger for IIM.

Health-related quality of life and well-being in adults with idiopathic inflammatory myopathy.

Health-related quality of life (HRQoL) and well-being are concepts that attempt to objectively capture a person's subjective perceptions of vitality and energy. Our objectives were to determine HRQoL and well-being in adult patients diagnosed with inflammatory myopathy who attended at our outpatient clinic and to investigate clinical and biological correlations with these concepts. Sixty-two patients (52 women), with a mean age of 50.7 years, were evaluated in this cross-sectional study-47 with dermatomyositis and 15 with polymyositis. Disease damage and activity were assessed with the International Myositis Assessment and Clinical Studies-validated instruments. Manual muscle testing was used to evaluate muscle strength. Quality of life was evaluated with the WHO instrument (WHO Quality of Life Measure (WHOQOL-BREF)), adapted for use in the Spanish population, and well-being with the WHO-Five Well-Being Index (WHO-5). t tests were conducted to examine differences in HRQoL and well-being outcomes in relation to several disease- and patient-related variables. Correlation analyses were performed with the Pearson correlation coefficient. None of the clinical or biological variables analyzed was significantly associated with a poorer HRQoL or well-being. No differences in HRQoL or WHO-5 well-being score were found between the two myositis subgroups (dermatomyositis vs. polymyositis). Disease activity and muscle weakness were negatively associated with the physical and environmental domains of the HRQoL, respectively (p < 0.002), but not with well-being. Disease duration did not have a significant impact on HRQoL or well-being. In adult patients with myositis, disease activity and muscle weakness are associated with poorer HRQoL in the physical health and environmental domains, respectively.

Anti-MDA5 antibodies in a large Mediterranean population of adults with dermatomyositis.

A new myositis-specific autoantibody directed against melanoma differentiation-associated gene 5 (anti-MDA5) has been described in patients with dermatomyositis (DM). We report the clinical characteristics of patients with anti-MDA5 in a large Mediterranean cohort of DM patients from a single center, and analyze the feasibility of detecting this autoantibody in patient sera using new assays with commercially available recombinant MDA5. The study included 117 white adult patients with DM, 15 (13%) of them classified as clinically amyopathic dermatomyositis (CADM). Clinical manifestations were analyzed, with special focus on interstitial lung disease and its severity. Determination of anti-MDA5 antibodies was performed by a new ELISA and immunoblot technique. In sera, from 14 (12%) DM patients (8 CADM), MDA5 was recognized by ELISA, and confirmed by immunoblot. Eight of the 14 anti-MDA5-positive patients (57.14%) presented rapidly-progressive interstitial lung disease (RP-ILD) versus 3 of 103 anti-MDA5-negative patients (2.91%) (P < 0.05; OR: 44.4, 95% CI 9.3-212). The cumulative survival rate was significantly lower in anti-MDA5-positive patients than in the remainder of the series (P < 0.05). Patients with anti-MDA5-associated ILD presented significantly lower 70-month cumulative survival than antisynthetase-associated ILD patients. Among the cutaneous manifestations, only panniculitis was significantly associated with the presence of anti-MDA5 antibodies (P < 0.05; OR: 3.85, 95% CI 1.11-13.27). These findings support the reliability of using commercially available recombinant MDA5 for detecting anti-MDA5 antibodies and confirm the association of these antibodies with RP-ILD in a large series of Mediterranean patients with DM.

Correlation of ultrasound B-lines with high-resolution computed tomography in antisynthetase syndrome.

OBJECTIVES: Interstitial lung disease is a common finding in patients with the antisynthetase syndrome. High-resolution computed tomography is the reference test for diagnosis and follow-up of this condition, but it involves considerable radiation exposure. Our aim was to describe chest ultrasound features and its correlation with high-resolution computed tomography findings in a series of patients with the antisynthetase syndrome. METHODS: The study included patients from our antisynthetase syndrome cohort with varying degrees of interstitial lung disease, consulting in our outpatient clinic over a 1-year period. Chest high-resolution computed tomography and chest sonography were prospectively performed within a 1-week period. High-resolution computed tomography Warrick score was calculated and chest sonography findings (B-lines) at several sonographic points along the anterior and posterior intercostal spaces were semi-quantitatively analyzed. Rho Spearman statistics were applied for possible correlations. RESULTS: Twenty-one consecutive patients were studied. A median of 59 thoracic points was studied per patient (IQR 6); 44.1% (95% CI 29.9-60.7) of them showed at least one B-line. A correlation coefficient of 0.135 (p=0.5) was found between the percentage of ultrasound points with B-lines and the Warrick's score. Only the number of bronchopulmonary segments showing ground glass findings was associated with the percentage of sonographic points with B-lines (Rho=0.5, p=0.02). CONCLUSIONS: A good correlation between the percentage of sonographic points with B-lines and high-resolution computed tomography ground glass opacities was observed in patients with the antisynthetase syndrome.

Neutrophil gelatinase-associated lipocalin as a biomarker for lupus nephritis.

BACKGROUND: One of the challenges of treating patients with lupus nephritis (LN) is to accurately assess disease activity and predict its outcome. Since renal-biopsy cannot be performed routinely, new surrogate biomarkers are needed. METHODS: We evaluated neutrophil gelatinase-associated lipocalin (NGAL), to predict renal outcome in LN. Serum and urinary NGAL levels, measured by the enzyme-linked immunosorbent assay, and the fractional excretion (FE) of NGAL relative to the FE of proteins (FE NGAL/FE protein ratio) were determined in a cross-sectional (n = 199) and longitudinal (n = 45) cohort of systemic lupus erythematosus (SLE) patients. Global and renal disease activity was assessed by the SLE disease activity indices, SLEDAI and rSLEDAI, respectively. Correlations between traditional biomarkers were established. Sensitivity, specificity and predictive values of NGAL for renal flare, response to therapy and progression to chronic kidney disease were calculated. RESULTS: The FE NGAL/FE protein ratio exhibited the best sensitivity and specificity to discriminate patients with active LN from those with non-renal flare and inactive SLE. In the prospective study, this biomarker was found to be the best candidate to predict proteinuric flares with an 87% sensitivity and 62% specificity for ratios >14.56 and complete response with a 61% sensitivity and 78% specificity for ratios >26.54 in the presence of a simultaneous worsening or improving rSLEDAIs, respectively. In both conditions, the FE NGAL/FE protein ratio outperformed the anti-dsDNA antibody titres and C3 predictive value. Progression to chronic kidney disease was best predicted by estimated glomerular filtration rate levels, but persistently high levels of serum NGAL (>444.4 ng/mL, P = 0.0001 by Kaplan-Meier) predicted a faster progression. CONCLUSIONS: The FE NGAL/FE protein ratio is a reliable marker of disease activity in patients with SLE and could be used as an indicator of response to therapy, although further studies are required to confirm these results.

Occupational exposure in patients with the antisynthetase syndrome.

Interstitial lung disease (ILD) is common in patients with myositis and is related with the presence of antisynthetase autoantibodies (aSA). Together with other manifestations, the resulting condition is known as the antisynthetase syndrome (ASS). Contact with certain environmental and occupational agents is also associated with the development of ILD. The objective of this study was to analyze occupational exposure and associated clinical manifestations in a cohort of patients with ASS. aSA had been identified by line immunoassay and confirmed by immunoprecipitation. Serial pulmonary function tests had been carried out to assess lung function. Thirty-two ASS patients and a control group of 32 myositis patients without aSA underwent a specific questionnaire interview to evaluate their cumulative exposure to biological dust, mineral dust, and gases/fumes up to disease onset. Comparisons were done with the Fisher exact test and Mann-Whitney test. Out from 32 ASS patients (median age, 42.7 yeras; IQR 32.2-52.5), twenty-six patients had anti-Jo-1, three anti-PL-12, and three anti-PL-7. Nine had polymyositis, 15 dermatomyositis, one amyopathic dermatomyositis, and seven pure ILD without myositis. Sixteen ASS patients (50 %) and seven (22 %) myositis patients without aSA had ever been highly exposed to dust, gases, or fumes (p < 0.05). A more than 10 % improvement in forced vital capacity occurred in 61 % of highly exposed patients and 23 % of those with low/no exposure (p = 0.06) over the observation period. In conclusion, a high percentage of patients with ASS had been exposed to dusts, gases, or fumes.

Clinical and serological findings associated with the expression of ITGAL, PRF1, and CD70 in systemic lupus erythematosus.

We determined the expression of Integrin alpha L chain (ITGAL), Perforin 1 (PRF1), and CD70 and studied the associations with laboratory and clinical parameters. CD4+ T cells were isolated from 35 SLE patients and 30 healthy controls. The transcript levels of ITGAL, PRF1, and CD70 were quantified by real-time reverse-transcription polymerase chain reaction (RT-PCR). The SLE patients had significantly elevated transcript levels of ITGAL (18.61±22.17 vs. 7.33±9.17, p=0.042), PRF1 (21.67±26.34 vs. 10.67±11.65, p=0.039), and CD70 (1.45±1.63 vs. 0.67± 0.28, p=0.011). Patients with anti-microsomal and/or anti-thyroglobulin antibodies showed high levels of ITGAL (33.41±30.14 vs. 13.58±16.43, p=0.044; and 34.01±27.66 vs. 11.90±16.17, p=0.007, respectively). No association was seen either for the typical antibodies of SLE or for the disease activity. Although ITGAL, PRF1, and CD70 are overexpressed in SLE CD4+ T cells, their expression is not linked to the typical clinical and serological parameters associated with the disease. The role that ITGAL may play in autoimmune thyroiditis deserves further investigation.

Calcineurin inhibitors in a cohort of patients with antisynthetase-associated interstitial lung disease.

OBJECTIVES: The aim of this paper is to assess the effect of calcineurin inhibitors (tacrolimus or cyclosporine) for treating patients with interstitial lung disease (ILD) associated with antisynthetase autoantibodies. METHODS: Sixty patients with antisynthetase autoantibodies were identified in our myositis cohort of 179 patients. The medical records of 15 patients with antisynthetase autoantibody-associated ILD treated with tacrolimus/cyclosporine (11 for refractory disease and 4 as first-line therapy) between 1980 and 2011 were retrospectively reviewed. Serial pulmonary function tests were used to assess the clinical response. Qualitative data are presented as a number and percentage, and quantitative data as the median and interquartile range (IQR). RESULTS: Patients were classified as having probable or definite idiopathic inflammatory myopathy (8 dermatomyositis and 4 polymyositis), and pure interstitial lung disease (3 cases). The 15 patients had received tacrolimus/cyclosporine for an average of 19 (IQR 14-30) months. Median age at onset of ILD was 42.3 (IQR 32.4-56.8) years and median duration of lung disease before administration of calcineurin inhibitors was 11 (IQR: 5-49) months. Median duration of follow-up was 24 (IQR 12-32) months. Thirteen patients had anti-histidyl-transfer RNA synthetase autoantibody (anti-Jo-1) and two had anti-alanyl-transfer RNA synthetase autoantibody (anti-PL-12). A more than 10% increase in FVC or stabilisation was observed in 13 (87%; 95%CI 56-98) patients who received calcineurin inhibitors (9 [81%] refractory cases and 4 [100%] as first-line therapy). CONCLUSIONS: Calcineurin inhibitors seem to be a good therapeutic option for managing ILD associated with antisynthetase autoantibodies, not only in refractory cases, but also as first-line treatment.

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.