scholarly journals P194 Non-bacterial osteitis syndromes: characterising an adult population

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Tiara Gill ◽  
Judith Bubbear ◽  
Richard Keen
Keyword(s):  
Retrospective Analysis ◽  
Symptom Onset ◽  
Adult Population ◽  
Interleukin 1 ◽  
Treatment Options ◽  
Diagnostic Delay ◽  
Joint Involvement ◽  
Metabolic Bone ◽  
Disease Registries ◽  
Mean Time

Abstract Background Inflammatory bone syndromes, such as SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) and CRMO (chronic recurrent multifical osteomyelitis) are rare and have not been extensively described in the existing literature. Although there have been proposed classification and diagnostic criteria, there is still limited consensus and uniformity of approach in identifying and treating these patients. This may be due to a lack of data and understanding about the nature and prognosis of these conditions. Attempts to characterise, especially in adult populations, has been limited thus far. As a tertiary metabolic bone service with an allied regional sarcoma service, we present further data on these conditions in an adult population. Methods Retrospective analysis of notes of patients, with a diagnosis of SAPHO and CRMO, that were registered on a departmental database was performed. Data on demographics, symptom onset and character, serum and radiological tests and treatment were analysed. Results 77 adults were identified with a diagnosis of SAPHO and CRMO, with a 60:40 % split respectively. Female preponderance was noted with 74% of this cohort represented. SAPHO patients tended to present slightly later with a mean age of symptom onset of 37 years as compared to 24 years in the CRMO group. Diagnostic delay was evident with mean time to diagnosis averaging 34 months. Pain and bony swelling were the most common symptoms (99% and 39% respectively), with the clavicle, sternum and spine being the most common sites involved. Osteitis was a universal feature in both groups, whereas skin and inflammatory joint involvement was more of a feature of SAPHO patients (59% vs 39%). There is limited consensus on appropriate diagnostic tests. This was reflected in this cohort with variable testing of inflammatory markers, serological testing of HLA B27 and extent and modality of imaging. Non-steroidal anti-inflammatories (NSAIDS), intravenous bisphosphonates, steroids and anti-microbials were all employed as treatment options, although efficacy was difficult to establish in this retrospective analysis, a significant source of referrals were from the local Sarcoma Unit; this may have introduced a degree of bias in our results. Conclusion As rare conditions, promoting awareness, having an index of suspicion and early referral to specialist centres would help address current issues faced by this patient population. Several national and international disease registries are in development and will undoubtly help to standardise data collection in order to guide further work in this area. Future studies examining treatment efficacy in current as well as emerging biological therapies, such as anti-tumour necrosis factor, anti- interleukin 1 and 17 agents, would be of significant value. Disclosures T. Gill None. J. Bubbear None. R. Keen None.

scholarly journals Delayed Diagnosis and Diagnostic Pathway of ALS Patients in Portugal: Where Can We Improve?

2021 ◽  
Vol 12 ◽  
Author(s):  
Catarina Falcão de Campos ◽  
Marta Gromicho ◽  
Hilmi Uysal ◽  
Julian Grosskreutz ◽  
Magdalena Kuzma-Kozakiewicz ◽  
...  
Keyword(s):  
Symptom Onset ◽  
Treatment Options ◽  
Diagnostic Delay ◽  
Delayed Diagnosis ◽  
Educational Interventions ◽  
Sociocultural Factors ◽  
Diagnostic Pathway ◽  
Tertiary Center ◽  
Modifiable Factor ◽  
Als Patients

Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with unsatisfactory treatment options. Best management and recruitment into clinical trials requires early diagnosis. However, diagnosis is often delayed. Analysis of the diagnostic pathway and identification of the causes of diagnostic delay are imperative.Methods: We studied a cohort of 580 ALS patients followed up in our ALS clinic in Lisbon. Demographic, disease, and sociocultural factors were collected. Time from first symptom onset to diagnosis, the specialist's assessment, and investigations requested were analyzed. Predictors of diagnostic delay were evaluated by multivariate linear regression, adjusting for potential confounders.Results: The median diagnostic delay from first symptom onset was 10 months. Spinal-onset, slower disease progression, cognitive symptoms at onset, and lower income were associated with increased diagnostic delay. Most patients were first assessed by general practitioners. Patients who were first evaluated by a neurologist were more likely to be correctly diagnosed, decreasing time to diagnosis. Electromyography was decisive in establishing the diagnosis.Conclusions: Late referral from non-neurologists to a neurologist is a potentially modifiable factor contributing to significant diagnostic delay. Educational interventions targeted to non-neurologists physicians, in order to increase awareness of ALS and, consequently, promote early referral to a neurologist at a tertiary center, will be important in reducing diagnostic delay.

scholarly journals Duodenal injuries in blunt trauma abdomen: a retrospective analysis

2019 ◽  
Vol 6 (9) ◽  
pp. 3238
Author(s):  
Sreejayan Meethale Purayil ◽  
Arun Suthan
Keyword(s):  
Retrospective Analysis ◽  
Blunt Trauma ◽  
Treatment Options ◽  
Diagnostic Delay ◽  
Morbidity And Mortality ◽  
Time Interval ◽  
High Morbidity ◽  
Surgical Department ◽  
Upper Abdominal ◽  
Duodenal Injuries

Background: Blunt trauma to abdomen very rarely produces isolated duodenal injuries and are to the tune of 3-5%. They are associated with high morbidity and mortality because of the diagnostic delay and missed diagnosis. The aim of this study is to discuss the mode of presentation, investigations and treatment options of isolated duodenal injuries in blunt trauma abdomen.Methods: Retrospective analysis of patients with blunt trauma abdomen who presented to surgical department at government medical college, Kozhikode, Kerala was done. Those who sustained isolated duodenal injuries from January 2010 to 2016 January were included in this study. Complex biliopancreatic, bowel and vascular injuries were excluded.Results: For the single D2 injury, primary closure in 2 layers with 2-0 vicryl and 3-0 braided black silk was done with an omental wrap. The D3 and D4 injuries, limited resection of the unhealthy perforated segment including D3 and D4 were done followed by an end to end duodeno jejunostomy and duodenal exclusion by anterior gastrojejunostomy. Leak test was performed, feeding jejunostomy was done.Conclusions: Isolated duodenal injuries due to blunt trauma abdomen are very rare and should be suspected in patients presenting with upper abdominal pain and vomiting after trauma. CECT abdomen helps to detect it. The time interval between the onset and definitive intervention greatly influences the morbidity and mortality in these injuries. 

Paraneoplastic ataxic syndrome with anti-Yo antibodies in breast and ovarian cancer: A retrospective analysis.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e18089-e18089
Author(s):  
Akshjot Puri ◽  
Usman Khan ◽  
Polly Ann Niravath
Keyword(s):  
Breast Cancer ◽  
Plasma Exchange ◽  
Retrospective Analysis ◽  
Cancer Diagnosis ◽  
Symptom Onset ◽  
Performance Status ◽  
Treatment Options ◽  
Breast Cancer Patients ◽  
Her2 Breast Cancer ◽  
Work Up

e18089 Background: Paraneoplastic cerebellar syndrome is a rare immunological reaction caused most commonly by Anti-Yo or Purkinje cell cytoplasmic antibody type 1(PCA1) to cerebellar degeneration protein 2(CDR2), found in the cerebellum and ectopically produced by tumor. Most cases are reported in women with HER2+ breast and ovarian cancers with underlying breast cancer patients having relatively better prognosis. Treatment initiation within one month of symptom onset has shown better outcomes. Methods: A retrospective analysis was conducted using a clinical database at a single institution and included 3 patients with a cancer diagnosis and ataxic symptoms diagnosed between 2016 and 2020. Data collection included age, gender, performance status, site of disease, pathology, diagnostic work up, treatment and overall survival (OS). Results: The study included 3 patients who presented with new onset ataxic symptoms with the median time of cancer diagnosis from onset of symptoms being 5 months (range 4-7). Median age was 62 years (range 59-83), all 3 patients were females and functionally independent before symptom onset. Out of 3 patients 2 had HER2+ breast cancer (one with IHC 3+ and one with equivocal IHC but 20 HER2 signals/nucleus) and the third had ovarian carcinoma. All 3 patients had stage IIIA disease as per AJCC 8th edition and presented with bilateral extremity weakness, ataxia, visual disturbances, vertigo and nausea/vomiting. Extensive neurological work up including MRI brain, CSF analysis, EEG were inconclusive except for high titres of anti- Yo antibodies in all patients. All patients were treated with cancer directed therapy and immunomodulation with plasma exchange, IVIG and steroids. The median OS being 22 months (range 12-28) with 2 patients with underlying breast cancer alive at the time of the analysis albeit there has been no neurological recovery, despite IVIG, steroids, and plasma exchange. Conclusions: The anti-Yo associated paraneoplastic ataxic syndrome most commonly associated with breast and pelvic tumors in women has a very poor prognosis despite anti-tumor and immunomodulating therapies, leaving most patients bed-ridden. The neurologic damage is irreparably incurred once the antibodies attack cerebellar cells. Thus, it is critical for clinicians to recognize the potential signs and symptoms as early as possible, so that treatment can be started, and long term symptoms are minimized. Further studies are needed to know the exact mechanism of this syndrome and provide evidence based treatment options.

scholarly journals Efficacy and safety of gout flare prophylaxis and therapy use in people with chronic kidney disease: a Gout, Hyperuricemia and Crystal-Associated Disease Network (G-CAN)-initiated literature review

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Huai Leng Pisaniello ◽  
Mark C. Fisher ◽  
Hamish Farquhar ◽  
Ana Beatriz Vargas-Santos ◽  
Catherine L. Hill ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Renal Function ◽  
Kidney Disease ◽  
Literature Review ◽  
Interleukin 1 ◽  
Treatment Options ◽  
Cochrane Library ◽  
Efficacy And Safety ◽  
Clinical Trial Results ◽  
Gout Flare

AbstractGout flare prophylaxis and therapy use in people with underlying chronic kidney disease (CKD) is challenging, given limited treatment options and risk of worsening renal function with inappropriate treatment dosing. This literature review aimed to describe the current literature on the efficacy and safety of gout flare prophylaxis and therapy use in people with CKD stages 3–5. A literature search via PubMed, the Cochrane Library, and EMBASE was performed from 1 January 1959 to 31 January 2018. Inclusion criteria were studies with people with gout and renal impairment (i.e. estimated glomerular filtration rate (eGFR) or creatinine clearance (CrCl) < 60 ml/min/1.73 m2), and with exposure to colchicine, interleukin-1 inhibitors, non-steroidal anti-inflammatory drugs (NSAIDs), and glucocorticoids. All study designs were included. A total of 33 studies with efficacy and/or safety analysis stratified by renal function were reviewed—colchicine (n = 20), anakinra (n = 7), canakinumab (n = 1), NSAIDs (n = 3), and glucocorticoids (n = 2). A total of 58 studies reported these primary outcomes without renal function stratification—colchicine (n = 29), anakinra (n = 10), canakinumab (n = 6), rilonacept (n = 2), NSAIDs (n = 1), and glucocorticoids (n = 10). Most clinical trials excluded study participants with severe CKD (i.e. eGFR or CrCl of < 30 mL/min/1.73 m2). Information on the efficacy and safety outcomes of gout flare prophylaxis and therapy use stratified by renal function is lacking. Clinical trial results cannot be extrapolated for those with advanced CKD. Where possible, current and future gout flare studies should include patients with CKD and with study outcomes reported based on renal function and using standardised gout flare definition.

scholarly journals Diagnosis and treatment delay of head and neck cancers during COVID-19 era in a tertiary care academic hospital: what should we expect?

2021 ◽  
Author(s):  
Pietro De Luca ◽  
Antonella Bisogno ◽  
Vito Colacurcio ◽  
Pasquale Marra ◽  
Claudia Cassandro ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Head And Neck ◽  
Retrospective Analysis ◽  
Tertiary Care ◽  
Diagnostic Delay ◽  
Treatment Delay ◽  
Head And Neck Cancers ◽  
Control Measures ◽  
Academic Hospital ◽  
The Impact

Abstract Background Since the spreading of SARS-CoV-2 from China, all deferrable medical activities have been suspended, to redirect resources for the management of COVID patients. The goal of this retrospective study was to investigate the impact of COVID-19 on head and neck cancers’ diagnosis in our Academic Hospital. Methods A retrospective analysis of patients treated for head and neck cancers between March 12 and November 1, 2020 was carried out, and we compared these data with the diagnoses of the same periods of the 5 previous years. Results 47 patients were included in this study. We observed a significative reduction in comparison with the same period of the previous 5 years. Conclusions Our findings suggest that the COVID-19 pandemic is associated with a decrease in the number of new H&N cancers diagnoses, and a substantial diagnostic delay can be attributable to COVID-19 control measures.

scholarly journals FRI0480 SCHNITZLER’S SYNDROME: DESCRIPTION OF AN ITALIAN MULTICENTER COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 837.1-837
Author(s):  
F. Crisafulli ◽  
P. Airò ◽  
F. Franceschini ◽  
A. Tincani ◽  
M. Frassi
Keyword(s):  
Monoclonal Gammopathy ◽  
Interleukin 1 ◽  
Diagnostic Delay ◽  
Injection Site Reaction ◽  
Lymphoproliferative Disease ◽  
Severe Allergic Reaction ◽  
Intermittent Fever ◽  
Multicenter Cohort ◽  
Laboratory Features ◽  
Schnitzler’S Syndrome

Background:Schnitzler’s syndrome is an autoinflammatory disease characterized by monoclonal gammopathy and recurrent episodes of urticaria accompanied by clinical and laboratory signs of acute inflammation. Although the exact pathogenic mechanisms have not been fully clarified, the role of Interleukin-1 seems to be central.Objectives:To describe clinical features and therapeutic approach in patients with Schnitzler’s Syndrome.Methods:Retrospective analysis of an Italian multicenter cohort. Data are expressed as the median (IQR).Results:The clinical data of 24 patients from 9 centers (median follow-up 6 years [2-10]; median age at diagnosis 56.5 years [51.25-64.25]) were collected. The median diagnostic delay was 2 years (0-10); the diagnosis was made consensually at the onset of symptoms in 4 cases. The main clinical and laboratory features are shown in Table 1. Therapeutic response was evaluable in 20 patients: all received corticosteroids (CS; 25mg/day [25-50]); in one case, a good clinical response was observed. Eight patients were initially treated with colchicine: in 3 cases it was effective in controlling symptoms and reducing the dose of CS; other 8 patients were treated with csDMARDs (n:1 [1-2]): only 1 patient had a good response to cyclosporin.Table 1.Clinical and laboratory featuresChronic Urticarial Rash, n (%)24/24 (100)Pruritus, n (%)17/24 (71)Intermittent fever, n (%)23/24 (96)Arthralgia/Arthritis, n (%)20/24 (83)Bone pain, n (%)8/24 (33)Weight loss, n (%)9/24 (38)Angioedema, n (%)4/24 (17)Lymphoadenopathy, n (%)7/24 (29)Hepatomegaly, n (%)3/24 (12)Splenomegaly, n (%)3/24 (12)Neuropathy, n (%)4/24 (17)Raised ESR or CRP, n (%)24/24 (100)Leukocytosis, n (%)17/24 (71)Anemia, n (%)9/24 (38)Monoclonal GammopathyIgG λ, n (%)5/22 (23)IgG κ, n (%)6/22 (27)IgM λ, n (%)1/22 (5)IgM κ, n (%)12/22 (55)Bence Jones Protein, n (%)6/23 (26)A bDMARD was initiated in 15 patients. In 7 of the 14 patients initially treated with anakinra this therapy was continued with benefit whereas in the other 7 patients the treatment was discontinued for primary inefficacy (1 patient), secondary inefficacy (3 patients) and adverse events (3 patients; 2 injection site reaction, 1 severe allergic reaction). After anakinra discontinuation, 5 patients were treated with canakinumab with a good response in 3 cases and a partial response in 1 case (persistent arthritis); 1 patient died during the treatment. No response was observed in 3 patients treated with TNF inhibitors as a 2ndor 3rdline bDMARDs, as well as in 1 case initially treated with tocilizumab (in which a good response was afterwards obtained with canakinumab). bDMARDs were associated with a csDMARD in 2 patients (methotrexate and methotrexate + cyclosporine).In one case monoclonal gammopathy evolved into Multiple Myeloma and the patient died 15 years after the onset of symptoms. Idiopathic myelofibrosis and myelodysplasia were found in one and in two patients, respectively.Conclusion:In most cases csDMARDs and bDMARDs like anti-IL6 and anti-TNFα were not able to control the disease. In contrast, in some cases, a good response to colchicine was observed; refractory patients may be successfully treated with anti-IL1 agents. Patients should be supervised for possible evolution towards lymphoproliferative disease.Disclosure of Interests:None declared

scholarly journals AB0490 IMPACT OF SPONDYLOARTHRITIS ON WORK PRODUCTIVITY: A REAL LIFE STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1272.1-1272
Author(s):  
M. Ben Majdouba ◽  
S. Boussaid ◽  
S. Rekik ◽  
S. Jemmali ◽  
H. Ajlani ◽  
...  
Keyword(s):  
Ankylosing Spondylitis ◽  
Age At Onset ◽  
Productivity Loss ◽  
Diagnostic Delay ◽  
Work Productivity ◽  
Symptomatic Treatment ◽  
Work Outcomes ◽  
Joint Involvement ◽  
The Mean

Background:Work productivity of patients with spondyloarthritis is frequently affected by their disease.Objectives:We aim to identify disease-related factors associated with poor work productivity in patients with spondyloarthritis.Methods:A cross-sectional study was performed in patients with spondyloarthritis. Data on disease characteristics were collected as well as specific indices: Visual analogue scale (VAS) for fatigue and pain, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Ankylosing Spondylitis Disease Activity Score with CRP (ASDAS-CRP), Bath Ankylosing Spondylitis Functionnel Index (BASFI) and Bath Ankylosing Spondylitis Metrology Index (BASMI). EuroQol-5D (EQ5D) was used to assess health-related quality of life. Work productivity was assessed by the Work Productivity and Activity Impairment scale (WPAI:SpA). Factors associated with presenteeism, absenteeism and work productivity loss were evaluated.Results:One hundred patients were enrolled (73 men and 27 women); mean age was 43.68 ± 10.3 years. Fifty nine percent of patients were employed, 26% were off work and 15% were retired of which 8% were in early retirement. Sixty seven percent of patients had ankylosing spondylitis, 17% had rheumatism associated with inflammatory bowel disease and 16% had psoriatic rheumatism. The average disease duration was 12.24 ± 8.73 years. Mean age at onset was 33.2 ± 10 years [18-59]. The average diagnostic delay was 2.41 ± 3 years; it was more than five years in 17% of cases. Sacroiliac pain has been noted in 69 patients, lumbar or cervical stiffness in 78 patients and peripheral joint involvement in 18 cases. Thirty one percent of patients had hip joint involvement and 49% had extra-articular manifestation. Fifty percent had inflammatory biological syndrome, 63% were treated with anti-TNFα and 58% needed symptomatic treatment regularly. The mean fatigue and pain VAS was respectively 5.58 ± 2.5 and 5.56 ± 2.9. The mean BASDAI was 4.4 ± 2.4, the average BASFI was 4.6 ± 2.7 and the average ASDAS-CRP was 2.77 ± 1.18. The mean BASMI was 4.4 ± 2.8. The mean EQ5D score was 0.485 ± 0.378. Among employed patients, mean absenteeism, presenteeism and work productivity loss was 21.8 ± 33.13%, 42 ± 32% and 46.5 ± 35.31%, respectively. These work outcomes were correlated to diagnostic delay ≥ 2 years (p<0.03), peripheral joint involvement (p=0.006), psoriasis (p=0.02), inflammatory biological syndrome (p<0.001), need of symptomatic treatment (p=0.001), fatigue and pain VAS ≥ 4 (p<0.001), BASDAI ≥ 4 (p<0.001), ASDAS-CRP ≥ 2.1 (p<0.001), BASFI ≥ 4 (p<0.001), BASMI ≥ 4 (p=0.002) and low EQ5D score (p<0.001). Work productivity loss was in addition correlated to age at onset < 25 years (p=0.03).Conclusion:Active disease, reduced physical function and poorer quality of life are associated with reduced work productivity. Early diagnosis and good disease management especially fatigue and pain can potentially improve work outcomes in patients with spondyloarthritis.Disclosure of Interests:None declared.

Sex-based differences in outcomes for adult patients presenting to the emergency department with a concussion

2021 ◽  
pp. 1-10
Author(s):  
Brian H. Rowe ◽  
Esther H. Yang ◽  
Lindsay A. Gaudet ◽  
Leeor Eliyahu ◽  
Daniela R. Junqueira ◽  
...  
Keyword(s):  
Emergency Department ◽  
Logistic Regression ◽  
Motor Vehicle ◽  
Adult Population ◽  
Treatment Options ◽  
Multivariate Logistic Regression Model ◽  
Adult Women ◽  
Multivariate Logistic Regression ◽  
Persistent Symptoms ◽  
Postconcussion Symptoms

OBJECTIVE Patients with concussion frequently present to the emergency department (ED). Studies of athletes and children indicate that concussion symptoms are often more severe and prolonged in females compared with males. Given infrequent study of concussion symptoms in the general adult population, the authors conducted a sex-based comparison of patients with concussion. METHODS Adults (≥ 17 years of age) presenting with concussion to one of three urban Canadian EDs were recruited. Discharged patients were contacted by telephone 30 and 90 days later to capture the extent of persistent postconcussion symptoms using the Rivermead Post Concussion Symptoms Questionnaire (RPQ). A multivariate logistic regression model for persistent symptoms that included biological sex was developed. RESULTS Overall, 250 patients were included; 131 (52%) were women, and the median age of women was significantly higher than that of men (40 vs 32 years). Women had higher RPQ scores at baseline (p < 0.001) and the 30-day follow-up (p = 0.001); this difference resolved by 90 days. The multivariate logistic regression identified that women, patients having a history of sleep disorder, and those presenting to the ED with concussions after a motor vehicle collision were more likely to experience persistent symptoms. CONCLUSIONS In a community concussion sample, inconsequential demographic differences existed between adult women and men on ED presentation. Based on self-reported and objective outcomes, work and daily activities may be more affected by concussion and persistent postconcussion symptoms for women than men. Further analysis of these differences is required to identify different treatment options and ensure adequate care and management of injury.

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