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European Heart Journal. Case Reports May 2024Hypereosinophilic syndrome (HES) is characterized by moderate to severe eosinophilia, exclusion of neoplastic or secondary origins of eosinophilia, and systemic...
BACKGROUND
Hypereosinophilic syndrome (HES) is characterized by moderate to severe eosinophilia, exclusion of neoplastic or secondary origins of eosinophilia, and systemic involvement with end-organ damage. Coronary arteries can be affected to cause vasospastic angina (VSA); however, the association of the two diseases is not well recognized.
CASE SUMMARY
A 55-year-old woman who had a history of multiple allergic disease such as bronchial asthma and chronic sinusitis with nasal polyps was hospitalized due to attacks of chest pain at rest. During a spontaneous attack of chest pain, ECG revealed ST-segment elevation in the inferior leads and emergency coronary angiography showed focal spasms of the right and left anterior descending coronary arteries, both of which were relieved after intracoronary administration of nitroglycerine. She was diagnosed with VSA according to the Japanese Circulation Society guidelines. Despite conventional vasodilator therapies, her resting angina remained refractory. Laboratory findings were notable for moderate eosinophilia. A comprehensive evaluation to uncover the underlying cause of refractory VSA led to the diagnosis of HES, concomitant with eosinophilic pneumonia and eosinophilic chronic rhinosinusitis. Pericoronary inflammation by fat attenuation index (FAI) was increased in the proximal segment of the right coronary artery. Treatment was initiated with oral prednisolone at a starting dose of 30 mg/day. The response to treatment was rapid, with her symptoms disappearing and a regression of eosinophilia observed the following day.
DISCUSSION
Hypereosinophilic syndrome manifests with refractory VSA, and eosinophil-suppressing corticosteroid therapy proves effective in improving both conditions along with reduction of the pericoronary inflammation by FAI.
PubMed: 38807943
DOI: 10.1093/ehjcr/ytae247 -
Caspian Journal of Internal Medicine 2024Intracranial inflammatory pseudotumours (IPT) are rare entities that frequently lead to misdiagnosis with malignant lesions. The identification of these lesions is...
BACKGROUND
Intracranial inflammatory pseudotumours (IPT) are rare entities that frequently lead to misdiagnosis with malignant lesions. The identification of these lesions is difficult, but important to avoid inadvertent iatrogenicity and to adjust therapeutic protocols.
CASE PRESENTATION
We report the case of a 30-year-old man who presented a single tonic-clonic seizure. Brain imaging showed a right frontal lesion with intra and extra axial components. Facing the radiologic presentation, a brain tumor was suspected, thus the patient underwent surgery. Pathological exam concluded to a plasma cell granuloma. A whole-body CT-scan showed only a thoracic aortitis. Complete blood work studies came back negative. The patient was also tested for an array of antibodies among which antinuclear antibodies were positive (blood level superior to 1/100). CSF evaluation revealed clear fluid with normal glucose concentration, normal protein levels and lymphocytic pleocytosis. Finally, IgG-4 plasma levels were elevated which led to the diagnosis of an IgG4-RD. The patient was put under prednisolone with a favorable outcome.
CONCLUSION
IPT have several etiologies, among which IgG4 related disease may be one of the less known as only 2 cases have previously been reported. Herein, we report a new case of a young man who presented for seizures related to an intracranial lesion of an IgG4 related disease. The challenge is to suspect such conditions to avoid unnecessary surgeries.
PubMed: 38807725
DOI: 10.22088/cjim.15.2.354 -
BMC Health Services Research May 2024Disease registries are comprehensive databases that record detailed information on patients diagnosed with specific conditions, providing valuable insights into their...
BACKGROUND
Disease registries are comprehensive databases that record detailed information on patients diagnosed with specific conditions, providing valuable insights into their diagnosis, treatment, and outcomes. This study aims to describe the pilot phase of the national pediatric Immune Thrombocytopenia(ITP) registry (NPITP) in Iran, serving as the inaugural interpretive report.
METHODS
This patient-centered software system was implemented as a national program across multiple pediatric centers in Iran. Several focus groups were conducted to establish a minimum data set (MDS) comprising six main classes, 14 sub-classes, and 187 data elements. Following expert consensus on the final data set, a web-based software tool was developed by the dedicated IT team, accessible online and offline via https://disreg.sbmu.ac.ir/q/ITP.html . The registry included children aged between two months and 18 years with a platelet count below 100 × 10/L, based on predefined inclusion criteria.
RESULTS
Within a four-month period, a total of 60 ITP patients were registered, including 41 (68.3%) newly diagnosed cases, 68 (13.6%) persistent cases, and 14 (23.3%) with chronic ITP. The mean age of the registered patients was 55.93 ± 9.72 months. The most frequently observed bleeding symptoms were petechiae (68.3%), purpura (51.6%), and ecchymosis (13.3%). Among the newly diagnosed patients, 20 (33.3%) received intravenous immunoglobulin (IVIG), 17 (28.3%) were treated with prednisolone, and 17 (28.3%) received combined IVIG and steroid therapy. Of all patients, 40 (66.7%) demonstrated a complete response to treatment, while 16 (26.7%) exhibited a partial response. Four patients (6.7%) remained unresponsive to therapy. Treatment-related complications, such as Cushing's syndrome, edema, weight gain, hirsutism, and mood disorders, were reported in 10 patients (16.6%). However, the majority of patients (81.7%) did not experience therapy-related complications.
CONCLUSION
The pilot phase of the NPITP registry successfully implemented a web-based software tool for data collection, aiming to enhance the quality of care, facilitate clinical research, and support health service planning in the future.
Topics: Humans; Registries; Child; Iran; Purpura, Thrombocytopenic, Idiopathic; Child, Preschool; Adolescent; Male; Female; Infant; Pilot Projects
PubMed: 38807135
DOI: 10.1186/s12913-024-11102-z -
Pharmacy (Basel, Switzerland) May 2024Glucocorticoid-induced osteoporosis (GIOP) is a side effect of glucocorticoid (GC) treatment; however, despite established prevention guidelines in various countries, a...
Glucocorticoid-induced osteoporosis (GIOP) is a side effect of glucocorticoid (GC) treatment; however, despite established prevention guidelines in various countries, a gap persists between these guidelines and clinical practice. To address this gap, we implemented a collaborative intervention between hospitals and community pharmacists, aiming to assess its effectiveness. Pharmacists recommended to the prescribing doctor osteoporosis treatment for patients who did not undergo osteoporosis treatment with a fracture risk score of ≥3 via tracing reports (TRs), between 15 December 2021, and 21 January 2022. Data were extracted from electronic medical records, including prescriptions, concomitant medications, reasons for not pursuing osteoporosis treatment, and TR contents. Of 391 evaluated patients, 45 were eligible for TRs, with 34 (75.6%) being males. Prednisolone was the most common GCs administered, and urology was the predominant treatment department. Among the 45 patients who received TRs, prescription suggestions were accepted for 19 (42.2%). After undertaking the intervention, guideline adherence significantly increased from 87% to 92.5%. This improvement indicates that TRs effectively bridged the evidence-practice gap in GIOP prevention among GC patients, suggesting their potential utility. Expansion of this initiative is warranted to further prevent GIOP.
PubMed: 38804472
DOI: 10.3390/pharmacy12030080 -
Renal Failure Dec 2024Acute kidney injury (AKI) is one of the most common and severe clinical syndromes of diffuse proliferative lupus nephritis (DPLN), of which poor prognosis is indicated...
BACKGROUND AND OBJECTIVES
Acute kidney injury (AKI) is one of the most common and severe clinical syndromes of diffuse proliferative lupus nephritis (DPLN), of which poor prognosis is indicated by aggravated renal function deterioration. However, the specific therapy and mechanisms of AKI in DPLN remain to be explored.
METHODS
The correlation between AKI and clinical pathological changes in DPLN patients was analyzed. Expression of STAT3 signaling was detected in MRL/lpr mice with DPLN using immunohistochemical staining and immunoblotting. Inhibition of STAT3 activation by combination therapy was assessed in MRL/lpr mice.
RESULTS
Correlation analysis revealed only the interstitial leukocytes were significantly related to AKI in endocapillary DPLN patients. MRL/lpr mice treated with vehicle, which can recapitulate renal damages of DPLN patients, showed upregulation of STAT3, pSTAT3 and caspase-1 in renal cortex. FLLL32 combined with methylprednisolone therapy significantly inhibited the STAT3 activation, improved acute kidney damage, reduced the interstitial infiltration of inflammatory cells and decreased the AKI incidence in MRL/lpr mice.
CONCLUSION
STAT3 activation may play an important role in the pathogenesis of DPLN and the development of AKI. Hence, STAT3 inhibition based on the combination of FLLL32 with methylprednisolone may represent a new strategy for treatment of DPLN with AKI.
Topics: Animals; Lupus Nephritis; STAT3 Transcription Factor; Mice, Inbred MRL lpr; Mice; Female; Disease Models, Animal; Acute Kidney Injury; Humans; Methylprednisolone; Kidney; Signal Transduction; Adult; Male
PubMed: 38803234
DOI: 10.1080/0886022X.2024.2358187 -
Medicine International 2024In certain cases, it is difficult to distinguish hepatic sarcoidosis from malignant lymphoma or drug-induced liver injury and to select the proper treatment for this...
In certain cases, it is difficult to distinguish hepatic sarcoidosis from malignant lymphoma or drug-induced liver injury and to select the proper treatment for this condition. The present study describes the case of a female patient in her 30s who was referred to the hospital due to fever, arthralgia, myalgia and abnormal liver function test results for 4 months. A laboratory examination revealed elevated levels of serum angiotensin-converting enzyme (ACE) and soluble interleukin-2 receptor (sIL-2R), as well as an increase in serum hepatic and biliary tract enzymes. Gallium scintigraphy revealed a marked uptake in the liver, as well as an uptake in the mediastinal, inguinal and external iliac lymph nodes. Magnetic resonance imaging revealed extensive hepatosplenomegaly with multiple non-enhancing splenic nodules. Hepatic sarcoidosis was diagnosed by a liver biopsy as non-caseating hepatic granulomas, and multinucleated giant cells were observed. The patient responded to treatment with 20 mg prednisolone daily, and exhibited an improvement in her symptoms. An improvement was also observed in her serum levels of ACE, sIL-2R, and serum hepatic and biliary tract enzymes; decreased gallium uptake in the liver was also observed. On the whole, the present case report reconfirms that liver biopsy is a useful diagnostic tool for hepatic sarcoidosis.
PubMed: 38799006
DOI: 10.3892/mi.2024.162 -
Journal of Clinical Medicine May 2024This study aimed to compare the incidence rates (IRs) of infections, including herpes zoster (HZ), in rheumatoid arthritis (RA) patients treated with Janus kinase...
Incidence Rates of Infections in Rheumatoid Arthritis Patients Treated with Janus Kinase or Interleukin-6 Inhibitors: Results of a Retrospective, Multicenter Cohort Study.
This study aimed to compare the incidence rates (IRs) of infections, including herpes zoster (HZ), in rheumatoid arthritis (RA) patients treated with Janus kinase inhibitors (JAKis) or interleukin-6 inhibitors (IL-6is). We retrospectively analyzed 444 RA patients treated using IL-6is ( = 283) or JAKis ( = 161). After adjusting for clinical characteristic imbalances by propensity score matching (PSM), we compared the IRs of infections including HZ between the JAKi and IL-6i groups. Observational period: 1423.93 patient years (PY); median observational period: 2.51 years. After PSM, incidence rate ratios comparing JAKi with IL-6i were 3.45 (95% confidence interval [CI]: 1.48-9.04) for serious infections other than HZ indicating that the JAKi-treated group was more likely to develop serious infection than the IL-6i-treated group. Multivariate Cox regression analyses revealed that the use of prednisolone > 5.0 mg/day, coexisting interstitial lung disease (ILD), and diabetes mellitus (DM) were independent risk factors for serious infections. The crude IR for HZ was significantly higher in the JAKi group, but the difference between groups was not significant (IRR: 2.83, 95% CI: 0.87-10.96) in PSM analysis. Unadjusted and PSM analyses performed in our study showed increased IRs of serious infections in patients with RA treated with JAKis compared with those treated with IL-6is. The presence of ILD or DM and the use of prednisolone were found to be independent risk factors for serious infection in RA patients treated using JAKis. Whereas the IRs for HZ after PSM were not significantly different between the JAKi and IL-6i groups.
PubMed: 38792541
DOI: 10.3390/jcm13103000 -
Children (Basel, Switzerland) May 2024Juvenile myasthenia gravis (MG) is a rare autoimmune neuromuscular disease, often treated with anticholinesterases, corticosteroids, and immunosuppressants. However,...
Juvenile myasthenia gravis (MG) is a rare autoimmune neuromuscular disease, often treated with anticholinesterases, corticosteroids, and immunosuppressants. However, optimal treatment durations remain unclear. This study investigated the clinical characteristics and treatment of juvenile MG, including medication duration. The administration period for all drugs, immunosuppressants, and prednisolone at doses greater than 0.35 mg/kg daily was extracted retrospectively from medical records. Nineteen participants (8 boys, 11 girls) aged 8 months to 14 years (median, 2.5 years) at onset were identified. Fourteen patients (73.7%) had ocular MG and five (26.3%) had generalized MG. Drug treatment was conducted in 18 cases; however, 7 patients did not complete the treatment. Among the patients who completed drug treatment, the duration of treatment ranged from 11 to 100 months (median, 47 months). In the six patients treated with continuous administration of prednisolone or immunosuppressants, the treatment duration ranged from 33 to 99 months (median, 56 months). No severe adverse effects requiring hospitalization were reported. The patients treated with prednisolone or immunosuppressants required at least 33 months of treatment. These results will help develop protocols for juvenile MG treatment.
PubMed: 38790567
DOI: 10.3390/children11050572 -
CNS Neuroscience & Therapeutics May 2024Plasma exchange (PE) and immunoadsorption (IA) are recognized as effective ways to treat attacks in AQP4 antibody-positive NMOSD, but high-quality evidence was lacking.... (Observational Study)
Observational Study
OBJECTIVE
Plasma exchange (PE) and immunoadsorption (IA) are recognized as effective ways to treat attacks in AQP4 antibody-positive NMOSD, but high-quality evidence was lacking. To evaluate the efficacy and safety of PE/IA plus intravenous methylprednisolone (IVMP) in NMOSD attacks using propensity scores to match IVMP as control.
METHODS
Patients were from a prospective observational cohort study. Stratification and interval propensity score matching (PSM) were used to reduce selection bias by matching baseline characteristics (gender, age, time to IVMP, EDSS at attack) between PE/IA + IVMP and IVMP group (in a ratio of 1:2). The primary endpoint of efficacy was EDSS change at 6 months. Adverse events and changes in laboratory tests were recorded.
RESULTS
Four hundred and eleven attacks of 336 patients were screened for PSM, and 90 attacks (30 PE/IA + IVMP and 60 IVMP) were included in the analysis. There were significant differences in EDSS [6.25 vs. 6.75; IQR (4.50-8.38 vs. 5.00-8.00), p = 0.671] and visual acuity [median logMAR = 0.35 vs. 1.00; IQR (0.30-0.84 vs. 0.95-1.96), p = 0.002] change between two groups at 6 months. PE/IA + IVMP treatment demonstrated predictive capacity for good recovery as indicated by an area under the curve (AUC) of 0.726. Fibrinogen reduction was found during PE/IA + IVMP treatment [n = 15 (50.00%)], but no severe adverse events led to apheresis treatment discontinuation.
DISCUSSION
After PSM analysis, IVMP+PE/IA in acute attack of NMOSD achieved better and continuous improvement in neurological function within 6 months compared with IVMP alone. PE/IA treatment showed a good safety profile.
Topics: Humans; Female; Male; Neuromyelitis Optica; Middle Aged; Adult; Propensity Score; Aquaporin 4; Cohort Studies; Blood Component Removal; Treatment Outcome; Plasma Exchange; Methylprednisolone; Autoantibodies; Prospective Studies
PubMed: 38790106
DOI: 10.1111/cns.14780 -
Scientific Reports May 2024The treatment of immune thrombocytopenia (ITP) is challenging and treatment outcomes depend on numerous unknown and patient-specific factors. Corticosteroids are the...
The treatment of immune thrombocytopenia (ITP) is challenging and treatment outcomes depend on numerous unknown and patient-specific factors. Corticosteroids are the cornerstone of ITP treatment, but they are associated with many side effects. In this retrospective cohort study, treatment outcomes and treatment adherence in patients with ITP were investigated in 214 ITP patients from November 15, 2022 to March 15, 2023. Multinomial regression analysis models were used to identify predictive factors for treatment outcomes. A p value of less than 0.05 was considered statistically significant. Most study participants were female 161 (75.5%), and the majority 172 (80.4%) of them were taking prednisolone only. In terms of treatment adherence, 178 (83.2%) of the study participants adhered well to their ITP medications. The complete response rate at 3 months was 139 (65.0%). Predictive factors for partial response were increased negative impact of ITP on health-related quality of life (AOR = 1.221, 95% CI 1.096-1.360), being treated at Tikur Abessa Sepcialazed Hospital (AOR = 0.431, 95% CI 0.197-0.941) and the presence of heavy menstrual bleeding (AOR = 2.255, 95% CI 0.925-5.497) compared to patients with complete response. Hepatitis B virus-infected ITP patients (AOR = 0.052, 95% CI 0.004-0.621) were also a predictive factor for no response compared to complete response.
Topics: Humans; Female; Male; Purpura, Thrombocytopenic, Idiopathic; Retrospective Studies; Adult; Ethiopia; Middle Aged; Treatment Outcome; Hospitals, Teaching; Young Adult; Aged; Adolescent; Quality of Life; Medication Adherence; Prednisolone
PubMed: 38789461
DOI: 10.1038/s41598-024-62372-w