The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), also known as coronavirus disease (COVID-19), is a major pandemic challenging health care systems around the world. The optimal ...management of patients infected with COVID-19 is still unclear, although the consensus is moving toward the need of a biphasic approach. During the first phase of the disease (from onset of the symptoms up to 7–10 days) viral-induced effects are prominent, with the opportunity to institute antiviral therapy. In the second inflammatory phase of the disease, immunosuppressive strategies (for example with glucocorticoids or anticytokine drugs) may be considered. This latter stage is characterized by the development of progressive lung involvement with increasing oxygen requirements and occasionally signs of the hemophagocytic syndrome. The management of the disease in patients with kidney disease is even more challenging, especially in those who are immunosuppressed or with severe comorbidities. Here we present the therapeutic approach used in Brescia (Italy) for managing patients infected with COVID-19 who underwent kidney transplantation and are receiving hemodialysis. Furthermore, we provide some clinical and physiopathological background, as well as preliminary outcome data of our cohort, to better clarify the pathogenesis of the disease and clinical management.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Introduction
Patients with immune-mediated glomerular diseases are considered at high risk for severe COVID-19 outcomes. However, conclusive evidence for this patient population is scarce.
Methods
We ...created a global registry and retrospectively collected clinical data of patients with COVID-19 and a previously diagnosed immune-mediated glomerular disease to characterize specific risk factors for severe COVID-19 outcomes.
Results
Fifty-nine patients with a history of immune-mediated glomerular diseases were diagnosed with COVID-19 between 01.03.2020 and 31.08.2021. Over a mean follow-up period of 24.79 ± 18.89 days, ten patients (16.9%) developed acute kidney injury. Overall, 44.1% of patients were managed in an outpatient setting and therefore considered as having “non-severe” COVID-19, while 55.9% of patients had severe COVID-19 requiring hospitalization including worse outcomes. Comparing both groups, patients with severe COVID-19 were significantly older (53.55 ± 17.91 versus 39.77 ± 14.95 years, p = .003), had lower serum albumin levels at presentation (3.00 ± 0.80 g/dL versus 3.99 ± 0.68 g/dL, p = .016) and had a higher risk of developing acute kidney injury (27% versus 4%, p = .018). Male sex (p <.001) and ongoing intake of corticosteroids at presentation (p = .047) were also significantly associated with severe COVID-19 outcomes, while the overall use of ongoing immunosuppressive agents and glomerular disease remission status showed no significant association with the severity of COVID-19 (p = .430 and p = .326, respectively).
Conclusion
Older age, male sex, ongoing intake of corticosteroids and lower serum albumin levels at presentation were identified as risk factors for severe COVID-19 outcomes in patients with a history of various immune-mediated glomerular diseases.
ObjectiveApart from the distinct microvascular lesions characterizing antiphospholipid antibodies nephropathy (aPL-N), various glomerular conditions, especially membranous nephropathy (MN) and focal ...segmental glomerulosclerosis (FSGS), have been reported in primary APS (PAPS) patients, even without evidence of aPL-related vascular lesions. Our aims are i) to evaluate factors associated to intra-renal involvement in PAPS patients ii) to clinically and histologically characterize PAPS patients with non-aPL-N intra-renal involvement.MethodsAn observational retrospective multicentric study was conducted including all PAPS patients regularly and prospectively followed in the period 1984–2023 in the Centers. Case-control study: PAPS patients with intra-renal involvement histologically confirmed vs PAPS patients without signs of renal involvement (figure 1).ResultsAmong the 258 PAPS patients evaluated (78% females, median age at onset: 32 years, 67% thrombotic phenotype, 54% obstetric phenotype, 41% triple aPL+), 7% had intra-renal involvement manifesting with isolated urinary abnormalities in 53% of the cases. At renal biopsy 35% had classic aPL-N injuries while 65% had non-aPL-N intra-renal lesions. i) As compared to patients without intra-renal involvement, the 17 patients with intra-renal involvement had less macrovascular thrombosis, more thrombocytopenia, epilepsy, and LA+ (table 1). ii) PAPS patients with non-aPL-N intra-renal lesions had MN in 55% and FSGS in 45% of the cases, with some degree of non-specific vascular injury (glomerular basement membrane wrinkling, mild hyaline plaques) in 64%. As compared to patients with aPL-N, they had more frequently normal serum creatinine and higher 24h-proteinuria levels (0.980.6–1.2 vs 1.91.2–2.0 mg/dL; p:0.095 and 3.51.9–3.9 vs 0.50.4–0.5 g/24h; p:0.003, respectively), but no differences in systemic autoantibodies or complement levels. All the patients belonging to this subgroup experienced aPL-related events (73% had thrombosis, 83% had obstetric events, 36% had ‘extra-criteria’ manifestations), that in 45% of the cases were preceded by the renal disease.ConclusionsThe present study highlights the importance of conducting appropriate renal assessment and, when necessary, renal biopsy in PAPS patients, even in presence of mild urinary alterations. On the other hand, from the nephrologists’ point of view, it may be relevant to consider the routinary research of aPL at time of glomerulopathies evaluation, in particular because of the aPL prognostic role in the development of subsequent related events.Abstract P1 Figure 1Flow chart: identification of PAPS patients with (cases) and without (controls) intra-renal involvementAbstract P1 Table 1Continuous variables are presented as median (1st-3rd quartile) and compared with Mann-Whitney test; categorical variables are presented as number/number available data (%) and compared with Chi-square test/Fisher’s exact test. *= ‘extra-criteria’ according to Syndey APS criteria 2006 Patients’ characteristics All patients n=258 Intra-renal involvement n=17 No intra-renal involvement n=241 p value Male sex 56/258 (21.7) 6/17 (35.3) 50/241 (20.7) 0.218 Caucasians 244/258 (94.6) 15/17 (88.2) 229/241 (95.0) 0.233 Age at disease onset, years 32.0 (25.0–44.0) 29.0 (24.0–28.0) 33.0 (25.0–44.0) 0.377 Thrombotic APS 173/258 (67.1) 6/17 (35.3) 167/241 (69.3) 0.004 Obstetric APS 109/202 (53.9) 5/11 (45.5) 104/191 (54.5) 0.561 Catastrophic APS 4/258 (1.6) 3/17 (17.6) 1/241 (0.4) 0.001 ‘Extra-criteria’ APS* 19/258 (7.4) 8/17 (47.1) 11/241 (4.6) <0.001 Thrombotic + obstetric APS 36/202 (17.8) 2/11 (18.2) 34/191 (17.8) 1.000 Thrombocytopenia 29/258 (11.2) 6/17 (35.3) 23/241 (9.5) 0.006 Heart valve lesions 21/258 (8.1) 2/17 (11.8) 19/241 (7.9) 0.637 Livedo reticularis 22/258 (8.5) 1/17 (5.9) 21/241 (8.7) 1.000 Epilepsy 17/258 (6.6) 4/17 (23.5) 13/241 (5.4) 0.018 Single aPL profile 87/250 (34.8) 2/15 (13.3) 85/235 (36.2) 0.072 Double aPL profile 59/250 (23.6) 4/15 (26.7) 56/235 (23.8) 0.761 Triple aPL profile 103/250 (41.2) 9/15 (60.0) 94/235 (40.0) 0.127 LA+ 165/254 (64.9) 17/17 (100.0) 148/237 (62.4) 0.002 aCL+ 171/257 (66.5) 11/16 (68.8) 160/241 (66.4) 0.846 aβ2GPI+ 190/251 (75.7) 11/16 (68.8) 179/235 (76.2) 0.548 ANA+ 125/255 (49.0) 6/17 (35.3) 119/238 (50.0) 0.241 anti-ENA+ 17/240 (7.1) 2/16 (12.5) 15/224 (6.7) 0.316 anti-DNA+ 0/241 (0.0) 0/17 (0.0) 0/224 (0.0) 1.000 C3, g/dL 101.1 (88.0–118.0) 108.5 (101.3–122.5) 101.0 (87.5–118.0) 0.477 C4, g/dL 19.0 (13.0 -24.0) 18.0 (12.5–23.5) 19.0 (13.0–24.0) 0.790
The SARS-CoV-2 epidemic is pressuring healthcare systems worldwide. Disease outcomes in certain subgroups of patients are still scarce, and data are needed. Therefore, we describe here the experience ...of four dialysis centers of the Brescia Renal COVID Task Force. During March 2020, within an overall population of 643 hemodialysis patients, SARS-CoV-2 RNA positivity was detected in 94 (15%). At disease diagnosis, 37 of the 94 (39%) patients (group 1) were managed on an outpatient basis, whereas the remaining 57 (61%) (group 2) required hospitalization. Choices regarding management strategy were made based on disease severity. In group 1, 41% received antivirals and 76% hydroxychloroquine. Eight percent died and 5% developed acute respiratory distress syndrome (ARDS). In group 2, 79% received antivirals and 77% hydroxychloroquine. Forty two percent died and 79% developed ARDS. Overall mortality rate for the entire cohort was 29%. History of ischemic cardiac disease, fever, older age (over age 70), and dyspnea at presentation were associated with the risk of developing ARDS, whereas fever, cough and a C-reactive protein higher than 50 mg/l at disease presentation were associated with the risk of death. Thus, in our population of hemodialysis patients with SARS-CoV-2 infection, we documented a wide range of disease severity. The risk of ARDS and death is significant for patients requiring hospital admission at disease diagnosis.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Abstract
Background and Aims
The recent decades have witnessed significant changes in the epidemiology and clinical course of infection-related glomerulonephritis (IRGN). We analyzed the ...clinicopathological features and long-term outcome of adult patients with biopsy-proven IRGN followed in a large Italian referral centre.
Method
We included patients with biopsy-proven IRGN diagnosed from 2000 to 2018. Clinical and laboratory findings, histological features, possible risk factors and therapy were assessed for both renal and patient outcome.
Results
Forty-one patients met the inclusion criteria (male:female ratio 3:1, mean age 61±16 years). Smoke habit (47.2%), alcoholism (30.6%), and diabetes (27.5%) were the most common risk factors. The most frequently identified sites of infection were skin, lung and heart (Table 1). Staphylococci spp. accounted for 76.5% of positive cultures (Figure 1). Hypocomplementaemia emerged in 48.5% of cases. The most frequent histologic patterns were diffuse proliferative (56.0%) and membranoproliferative (29.3%) glomerulonephritis. Haemodialysis was required by 22.5% of patients at inception. Two thirds of patients developed chronic kidney disease; half of them reached end-stage renal disease (ESRD). By multivariate analysis ESRD was associated with diabetes (HR 13.7; 95% CI, 1.6-121.0; p=0.018), crescents (HR 25.2; 95% CI, 2.7-235.7; p=0.005), and interstitial fibrosis (HR 31.0; 95% CI, 3.3-287.3; p=0.003). Male gender (HR 12.7; 95% CI, 10.8-14.6; p=0.008) hypertension (HR 40.8; 95% CI, 38.6-43.1; p=0.001), gross haematuria (HR 11.8; 95% CI, 9.4-14.2; p=0.047), need for haemodialysis at onset (HR 16.3; 95% CI, 14.7-17.8; p<0.0001), and interstitial infiltrate (HR 13.3; 95% CI, 11.6-15.0; p=0.003) significantly affected survival. Corticosteroids did not affect prognosis.
Conclusion
While traditionally considered a “benign” disease with a favorable course in children, IRGN is a potentially severe disease in adults, particularly when a background of major comorbidities and older age are present. A significant proportion of patients does not recover renal function, with a remarkable risk of ESRD.
Table 1.
Sites of infection. Three patients (11.5%) presented multiple infections
N°/ TOT
%
Pneumonia
9/26
34.6
Skin infection
7/26
26.9
Endocarditis
6/26
23.1
Bone infection
3/26
11.5
Pharyngitis
1/26
3.8
Cholangitis
1/26
3.8
Prosthetic aortic graft infection
1/26
3.8
Urinary tract infection
1/26
3.8
Granulomatous rhinitis overinfection
1/26
3.8
Figure 1.
Results from microbiological tests.
Individuals with asthma spend less time engaging in physical activity compared to the general population. Increasing physical activity has become a patient-centered goal for the treatment of ...treatable traits of individuals with asthma. There are data showing the possible effects of a pulmonary rehabilitation program on physical activity in obese individuals with asthma but not in normal-weight asthmatics. The objective of this feasibility study is to estimate the number of daily steps and time spent on activity in normal-weight individuals with asthma, measured before and after a pulmonary rehabilitation program.
Normal-weight individuals with moderate to severe asthma were evaluated. The individuals measured their daily steps with an accelerometer for 5 days before and after a pulmonary rehabilitation program. The study was registered on ClinicalTrials.gov: NCT05486689.
In total, 17 participants were enrolled; one dropout and data on the time in activity of two individuals are missing due to a software error during the download. Data from 16 patients were analyzed. The median number of steps/day at baseline was 5,578 (25th, 75th percentiles = 4,874, 9,685) while the median activity time was 214 min (25th, 75th percentiles = 165, 239). After the rehabilitation program, the number of daily steps increased by a median value of 472 (
-value = 0.561) and the time in activity reduced by 17 min (
-value = 0.357). We also found a significant difference in quality of life, muscle strength, and exercise capacity.
The results of this study make it possible to calculate the sample size of future studies whose main outcome is daily steps in normal-weight individuals with asthma. The difficulties encountered in downloading time in activity data do not allow the same for this outcome.
ClinicalTrials.gov, identifier NCT05486689.
Long peripheral catheters (LPCs) role in Difficult IntraVenous Access (DIVA) patients admitted to the emergency department has already been studied, resulting in a rapid, safe, and cost-effective ...procedure. Although their use in outpatient settings is established, there is a lack of studies assessing their benefits. In particular, rheumatologic outpatients affected by scleroderma, especially those affected by digital ulcers, are often treated with intravenous infusions of prostaglandin I
(PGI
) analog (IV-PGI
A).
From 1 October 2021 to 31 March 2024, we conducted a prospective study enrolling DIVA outpatients affected by systemic sclerosis or undifferentiated connective tissue disease who needed IV-PGI
A therapy at L. Sacco Hospital in Milan (Italy). Each treatment cycle consisted of four consecutive days of infusion of iloprost or alprostadil. The primary aim was to assess the efficacy and potential complications associated with LPCs for IV-PGI
A.
Twenty-six patients were enrolled 23 were females (88.5%), and the median age was 72 years (IQR 56-78.7). In total, 97 LPCs were inserted, with a mean number of insertions per patient/year of 2.3. An increase in LPCs insertion during the 30 months of the enrollment period was observed. Eighteen patients required more than one LPC placement, and in 61% of them, the second venipuncture was executed at a different site. No procedural complications were registered (accidental puncture of the brachial artery, accidental median nerve puncture, bleeding) nor late complications (Catheter-Related Thrombosis, Catheter-Related Bloodstream Infections, Accidental Removal).
Our experience shows that LPCs could be valuable and safe for rheumatologic outpatients. The increased number of insertions and new and total patients enrolled each year defines the satisfaction of patients and health care professionals.
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NUK, OILJ, SAZU, UKNU, UL, UM, UPUK
Nusinsersen is now available in Italy for all SMA types. We describe the experience with intrathecal treatment with nusinersen in 50 patients with SMA at the NEMO Center (NEuroMuscular Omniservice ...Clinical Center) in Milan, a neuromuscular patient-centered clinic hosted within Niguarda Hospital, a National Public General Hospital. Our results indicate that the pathway of care described outweighs the burden due to the repeated intrathecal injections. Irrespective of age and severity, the treatment is feasible, accessible, and replicable provided that there is a multidisciplinary team having experience and training in SMA.
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DOBA, EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, IZUM, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, SIK, UILJ, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
OBJECTIVETo report cross-sectional clinical findings in a large cohort of patients affected by type 1 spinal muscular atrophy.
METHODSWe included 122 patients, of age ranging between 3 months and 22 ...years, 1 month. More than 70% (85/122) were older than 2 years and 25% (31/122) older than 10 years. Patients were classified according to the severity of phenotype and to the number of SMN2 copies.
RESULTSPatients with the more common and the most severe phenotype older than 2 years were, with few exceptions, on noninvasive ventilation and, with increasing age, more often had tracheostomy or >16-hour ventilation and a gastrostomy inserted. In contrast, 25 of the 28 patients with the mildest phenotype older than 2 years had no need for tracheostomy or other ventilatory or nutritional support. In patients older than 2 years, the Childrenʼs Hospital of Philadelphia Infant Test of Neuromuscular Disorders scores were generally lower compared to those found in younger patients and showed distinct levels of functional abilities according to the severity of the phenotype. Similar findings were also observed on the Hammersmith Infant Neurological Examination.
CONCLUSIONSOur findings confirm that, after the age of 2 years, patients with type 1 spinal muscular atrophy generally survive only if they have gastrostomy and tracheostomy or noninvasive ventilation >16 hours and have low scores on the functional scales. More variability, however, can be expected in those with the mildest phenotype, who achieve head control. These data provide important baseline information at the time treatments are becoming available.
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