Despite the recent advances in the field of autoinflammatory diseases, most patients with recurrent fever episodes do not have any defined diagnosis. The present study aims at describing a cohort of ...patients suffering from apparently unexplained recurrent fever, in whom non-radiographic axial spondylarthritis (SpA) represented the unique diagnosis identified after a complete clinical and radiologic assessment.
Patients' data were obtained from the international registry on Undifferentiated Systemic AutoInflammatory Diseases (USAIDs) developed by the AutoInflammatory Disease Alliance (AIDA) network.
A total of 54 patients with recurrent fever episodes were also affected by non-radiographic axial SpA according to the international classification criteria. SpA was diagnosed after the start of fever episodes in all cases; the mean age at the diagnosis of axial SpA was 39.9 ± 14.8 years with a diagnostic delay of 9.3 years. The highest body temperature reached during flares was 42°C, with a mean temperature of 38.8 ± 1.1°C. The most frequent manifestations associated to fever were: arthralgia in 33 (61.1%) cases, myalgia in 24 (44.4%) cases, arthritis in 22 (40.7%) cases, headache in 15 (27.8%) cases, diarrhea in 14 (25.9%) cases, abdominal pain in 13 (24.1%) cases, and skin rash in 12 (22.1%) cases. Twenty-four (44.4%) patients have taken daily or on-demand non-steroidal anti-inflammatory drugs (NSAIDs) and 31 (57.4%) patients have been treated with daily or on demand oral glucocorticoids. Colchicine was used in 28 (51.8%) patients, while other conventional disease modifying anti-rheumatic drugs (cDMARDs) were employed in 28 (51.8%) patients. Forty (74.1%) patients underwent anti-tumor necrosis factor (TNF) agents and 11 (20.4%) were treated with interleukin (IL)-1 inhibitors. The response to TNF inhibitors on recurrent fever episodes appeared more effective than that observed with anti-IL-1 agents; colchicine and other cDMARDs were more useful when combined with biotechnological agents.
Signs and symptoms referring to axial SpA should be inquired in patients with apparently unexplained recurrent fever episodes. The specific treatment for axial SpA may lead to a remarkable improvement in the severity and/or frequency of fever episodes in patients with unexplained fevers and concomitant axial SpA.
Behçet’s disease (BD) is a heterogeneous multifactorial autoinflammatory disease characterized by a plethora of clinical manifestations. Cutaneous lesions are considered hallmarks of the disease. ...However, their evolution over time and a thorough description are scarcely reported in non-endemic regions. The aim of this study was to detail BD skin manifestations and their evolution over time in Italy, as well as the dermatological prognostic impact of specific cutaneous features in long-standing disease. Data were collected in a double fashion, both retrospectively and prospectively, from the AutoInflammatory Disease Alliance (AIDA) international registry dedicated to BD, between January 2022 and December 2022. A total of 458 Italian patients were included. When assessing skin manifestations course, the constant or sporadic presence or absence of cutaneous involvement between onset and follow-up was considered. Oral ulcers (OU) (88.4%) and genital ulcers (GU) (52.6%), followed by skin involvement (53.7%) represented the most common presenting mucocutaneous manifestations at disease onset. Up to the time of enrolment into the AIDA registry, 411 (93.8%) patients had suffered from OU and 252 (57.9%) from GU; pseudofolliculitis (PF) accounted for the most common skin manifestation (170 patients, 37.1%), followed by erythema nodosum (EN) (102 patients, 22.3%), skin ulcers (9 patients, 2%) and pyoderma gangrenosum (4 patients, 0.9%). A prospective follow-up visit was reported in 261/458 patients; 24/148 (16.2%) subjects with skin involvement as early as BD onset maintained cutaneous lesions for the entire period of observation, while 120 (44.1%) patients suffered from sporadic skin involvement. Conversely, 94/113 (83.2%) with no skin involvement at disease onset did not develop skin lesions thereafter. At follow-up visits, cutaneous involvement was observed in 52 (20%) patients, with a statistically significant association between PF and constant skin involvement (
p
= 0.031). BD in Italy is characterized by a wide spectrum of clinical presentations and skin manifestations in line with what is described in endemic countries. Patients with skin disease at the onset are likely to present persistent cutaneous involvement thereafter; mucocutaneous lesions observed at the onset, especially PF, could represent a warning sign for future persistent skin involvement requiring closer dermatological care.
Background
Cardiac allograft vasculopathy (CAV) is an important cause of late mortality after heart transplantation, which may be influenced by preexisting coronary disease (CAD) in the donor heart.
...Methods
The aim of this study was to verify whether CAD in the donor heart had any influence on survival, cardiac‐related adverse events (CRAEs), and coronary disease progression after transplantation. Donor coronary angiography performed in 289 hearts showed absence of CAD in 232 (no‐CAD group) and moderate (≤50%) stenoses (CAD group) in 57. The 2 groups were compared for survival, freedom from CRAEs, and development of grade ≥ 2 CAV after transplantation.
Results
Of 30‐day mortality and postoperative complication rate was similar as mean follow‐up (76 ± 56 and 75 ± 55 months) for no‐CAD and CAD (P = 0.8). Ten‐year actuarial survival was 58 ± 4% and 62 ± 7% for no‐CAD and CAD (P = 0.4). Ten‐year freedom from grade ≥ 2 CAV and from CRAEs was 81 ± 4% and 66 ± 5% vs 75 ± 8% and 67 ± 9% in no‐CAD and CAD (P = 0.9 and 0.9, respectively).
Conclusions
Donor hearts with moderate CAD did not affect survival, freedom from CRAEs and did not accelerate development of high‐grade CAV after transplantation supporting the use of such grafts to expand the donor pool. Routine use of coronary angiography in donor selection appears justified.
The present study provides novel data regarding levels of polychlorinated biphenyls (PCBs) and organochlorine pesticides (DDTs) in muscle tissue of European conger eel (
Conger conger
) from Ionian ...Sea (Mediterranean Sea). The mean concentration of PCBs (891 ng g
−1
lipid weight) was higher than those of DDTs (543 ng g
−1
lipid weight). PCB pattern was dominated by higher chlorinated congeners (hexa-CBs: 69.3 %, penta-CBs: 17.2 %, hepta-CBs: 13.3 %). Regarding DDT pattern,
p,p
′-DDE was prevalent in all samples (85.5 %), suggesting no recent DDT input. The total 2,3,7,8-TCDD toxic equivalent (TEQs) of coplanar PCBs, including mono-and non-
ortho
congeners was 0.41 pg g
−1
wet weight (29.92 pg g
−1
lipid weight). The PCB and DDT levels, as well as TEQ concentrations were lower than most of the corresponding published data for fish from Mediterranean and non-Mediterranean regions, probably reflecting a moderately contaminated area.
