Spontaneous Remission Research Articles

Clinical Manifestations and Management of Pediatric Granuloma Annulare: A Systematic Review

To determine the clinical manifestations, management, and outcomes of pediatric granuloma annulare. We searched MEDLINE via PubMed, Latin American and Caribbean Health Sciences (LILACS), and EMBASE from inception to January 2021. We included all original reports of patients < 18 years of age with a diagnosis of GA and all original reports describing any intervention, including topical or systemic agents, in these patients. Two authors independently extracted socio-demographics and clinical data of the study patients, and treatment(s) used. Of 2,440 reports screened, 202 were included (836 patients). The mean age was 5.7 (SD 3.8) years and F:M ratio 1.3:1. Localized GA (n=384, 46.8%) and subcutaneous GA (n=353, 43.0%) were the most prevalent subtypes. The most affected site was lower limbs (n=272/568, 47.9%). Suspected triggering factors were mainly local trauma. Diabetes was associated in 22 (2.6%) patients. The rate of spontaneous remission was high (n=140/155, 90.3%), with a median time of 12 months. The most frequent therapies were surgery and topical corticosteroids. Recurrence was observed in 38.3% (n=168/439) of patients, regardless of treatment. Pediatric GA frequently resolves spontaneously yet displays a high recurrence rate. Hence, in asymptomatic forms, invasive therapies are not recommended as first-line treatment.

Sustained spontaneous remission in KMT2A-MLLT3 mutated myeloid sarcoma

Sustained spontaneous remission in KMT2A-MLLT3 mutated myeloid sarcoma

Genetic stratification reveals COL4A variants and spontaneous remission in Egyptian children with proteinuria in the first 2 years of life.

The earlier the onset of proteinuria, the higher the incidence of genetic forms. Therefore, we aimed to analyse the spectrum of monogenic proteinuria in Egyptian children presenting at age <2 years. The results of 27-gene panel or whole-exome sequencing were correlated with phenotype and treatment outcomes in 54 patients from 45 families. Disease-causing variants were identified in 29/45 (64.4%) families. Mutations often occurred in three podocytopathy genes: NPHS1, NPHS2 and PLCE1 (19 families). Some showed extrarenal manifestations. Additionally, mutations were detected in 10 other genes, including novel variants of OSGEP, SGPL1 and SYNPO2. COL4A variants phenocopied isolated steroid-resistant nephrotic syndrome (2/29 families, 6.9%). NPHS2 M1L was the single most common genetic finding beyond the age of 3 months (4/18 families, 22.2%). Biopsy results did not correlate with genotypes (n=30). On renin-angiotensin-aldosterone system antagonists alone, partial and complete remission occurred in 3/24 (12.5%) patients with monogenic proteinuria each, whereas 6.3% (1/16) achieved complete remission on immunosuppression. Genotyping is mandatory to avoid biopsies and immunosuppression when proteinuria presents at age <2 years. Even with such a presentation, COL4A genes should be included. NPHS2 M1L was prevalent in Egyptian children (4 months-2 years) with proteinuria, demonstrating precision diagnostic utility.

A mechanistic analysis of spontaneous cancer remission phenomenon: identification of genomic basis and effector biomolecules for therapeutic applicability.

The online version contains supplementary material available at 10.1007/s13205-023-03515-0.

Smoking and Carcinoma Trends in Recurrent Respiratory Papillomatosis Patients.

Recurrent respiratory papillomatosis (RRP) is a chronic disease of the upper respiratory tract caused by human papillomavirus types 6 and 11. The disease course is characteristically unpredictable, ranging from spontaneous remission to aggressive, recurrent disease. Thus, management is often challenging and requires unique approaches tailored to each individual patient. While recent literature has described risk factors for more aggressive disease, few sources have investigated the impact of smoking on RRP disease course and risk for malignant transformation. A retrospective chart review was conducted for adult RRP patients evaluated at an academic tertiary care center between 2005 and 2020. A total of 188 patients were identified. Demographic and clinical data were collected, including smoking and alcohol history, HPV subtype, history of dysplasia and/or carcinoma, voice handicap index scores, Derkay scores, debulkings (in office and operating room), and days to papilloma recurrence. Malignant degeneration in RRP occurred in 16.3% of smokers and 3.6% of nonsmokers. Smokers who developed carcinoma had less debulkings per years of evaluation than those not developing carcinoma (0.21 vs 0.92, P = .004). Additionally, patients that either presented with or developed carcinoma during their course had a higher pack-year smoking history (18.0 vs 12.21, P = .0002). No difference in days to recurrence or inter-surgical interval was demonstrated between smokers and nonsmokers. The report demonstrates that smoking can increase the risk of malignant transformation in RRP patients.

How sexuality is affected and managed in patients under antipsychotic drugs

IntroductionSexual dysfunction (SD) is a prevalent side effect of antipsychotic drugs (AP), and it impairs patients’ quality of life. Because of the distress caused by it, it should be borne in mind when prescribed since it is responsible for treatment nonadherence or discontinuation. SD affects about 45- 80% of males and 30-80% of females that take it. In SD, all phases of the sexual response cycle may be compromised.ObjectivesThis non-systematic review of the literature aims to better understand the antipsychotic-induced SD and its management to better compliance of AP-treated patients without compromising their quality of life.MethodsA semi-structured review on PubMed linking SD as a side effect of AP drugs.ResultsAll AP drugs can cause SD. It seems related to their mechanism of activity at receptors D2, 5-HT2, α1, H1, and M, which are also involved in sexual function. They do it by diminishing arousal, decreasing libido by blocking motivation and reward system and orgasm indirectly, provoking erectile dysfunction by vasodilatation, and decreasing woman lubrification. Hyperprolactinemia is a significant cause of sexual dysfunctions. Haloperidol, Risperidone, and Amisulpride (prolactin elevating AP) are more likely to cause SD than Olanzapine, Clozapine, Quetiapine, and Aripiprazole (prolactin sparing AP). Although psychotic disorders (Schizophrenia and other psychotic disorders) can impact sexual functioning, according to evidence, there is no denying the role of AP in this issue. Aripiprazole, a D2 partial agonist, has been associated with lower rates of SD and seems to reduce the rates of SD in patients previously treated with other AP. Other AP with the same potential dopamine agonist activity, such as Cariprazine and Brexpiprazole, can probably have the same effect. The management of SD induced by AP drugs should include measuring serum prolactin and modifying risk factors like hypertension, smoking, hyperglycemia, and hypercholesterolemia. In that regard, waiting for spontaneous remission, reducing the dose of the AP prescribed, or switching to Aripiprazole are all viable strategies, if possible. Although the evidence supporting the addition of symptomatic therapies is weak, adding dopaminergic drugs (amantadine, bromocriptine, cabergoline) or drugs with specific effects on sexual functioning (such as phosphodiesterase inhibitors or yohimbine) may be helpful in selected cases.ConclusionsAlthough all AP drugs can cause sexual dysfunction, it is difficult to determine its true prevalence accurately. AP-induced sexual dysfunction can adversely affect compliance and is one of the factors that must be considered when selecting treatment. In summarizing, Aripiprazole has shown to be the AP with the most favorable profile concerning SD. Cariprazine and Brexpiprazole, being also D2 partial agonists, may cause less SD.Disclosure of InterestNone Declared

Integrated Mental Healthcare and Vocational Rehabilitation for People on Sick Leave with Anxiety or Depression: 24-Month Follow-up of the Randomized IBBIS Trial

Integration of vocational rehabilitation and mental healthcare has shown some effect on work participation at 1-year follow-up after sick leave with depression and anxiety. We aimed to study the effect on work and health outcomes at 2-year follow-up, why we performed a randomized trial was conducted to study the effectiveness of integrated intervention (INT) compared to service as usual (SAU) and best practice mental healthcare (MHC). We included 631 participants, and at 24-month follow-up, we detected no differences in effect between INT and SAU. Compared to MHC, INT showed faster return-to-work (RTW) rates (p = 0.044) and a higher number of weeks in work (p = 0.024). No symptom differences were observed between the groups at 24 months. In conclusion, compared to SAU, INT was associated with a slightly higher work rate reaching borderline statistical significance at 12-month follow-up and lower stress levels at 6-month follow-up. The disappearance of relative effect between 12 and 24 months may be explained by the fact that the intervention lasted less than 12 months or by delayed spontaneous remission in the SAU group after 12 months. Despite the lack of effect at long-term follow-up, INT still performed slightly better than SAU overall. Moderate implementation difficulties, may partly explain the absence of the hypothesized effect. Integrated intervention, as implemented in this trial, showed some positive effects on mid-term vocational status and short-term stress symptom levels. However, these effects were not sustained beyond the duration of the intervention.

Cyclic Cushing's syndrome: difficulties of diagnostic search and choice of treatment tactics. A clinical case

Cyclic Cushing's syndrome is a pathological condition characterized by alternating periods of excessive cortisol secretion with corresponding clinical manifestations and periods of spontaneous remission of the disease.To diagnose Cyclic Cushing's syndrome it is necessary to record at least three episodes of excessive cortisol secretion alternating with periods of normalization of its production.In most cases, this pathology is diagnosed in patients with ACTH-secreting pituitary tumor, however, there are rare cases of cyclic hypercorticism with ectopic ACTH secretion by tumors of different localization and without verification of pathological hormonal secretion focus. In addition, cyclic hyperproduction of cortisol can be also observed in ACTH-independent Cushing's syndrome associated with the presence of corticosteroma or adrenal hyperplasia. The exact causes and mechanisms of the cyclic hypercorticism are currently insufficiently studied.Due to the atypical course of the disease, the unpredictability of the occurrence of a new «cycle», the variability of its duration and manifestations (not only in different patients, but also in the same patient), verification of the diagnosis and determination of treatment tactics may be difficult in the daily practice of specialists, and the prevalence of this condition can be undervalued.

Pituitary Apoplexy in Patients with Pituitary Neuroendocrine Tumors (PitNET)

Various complications of pituitary neuroendocrine tumors (PitNET) are reported, and an intratumor hemorrhage or infarct underlying pituitary apoplexy (PA) represents an uncommon, yet potentially life-threatening, feature, and thus early recognition and prompt intervention are important. Our purpose is to overview PA from clinical presentation to management and outcome. This is a narrative review of the English-language, PubMed-based original articles from 2012 to 2022 concerning PA, with the exception of pregnancy- and COVID-19-associated PA, and non-spontaneous PA (prior specific therapy for PitNET). We identified 194 original papers including 1452 patients with PA (926 males, 525 females, and one transgender male; a male-to-female ratio of 1.76; mean age at PA diagnostic of 50.52 years, the youngest being 9, the oldest being 85). Clinical presentation included severe headache in the majority of cases (but some exceptions are registered, as well); neuro-ophthalmic panel with nausea and vomiting, meningism, and cerebral ischemia; respectively, decreased visual acuity to complete blindness in two cases; visual field defects: hemianopia, cranial nerve palsies manifesting as diplopia in the majority, followed by ptosis and ophthalmoplegia (most frequent cranial nerve affected was the oculomotor nerve, and, rarely, abducens and trochlear); proptosis (N = 2 cases). Risk factors are high blood pressure followed by diabetes mellitus as the main elements. Qualitative analysis also pointed out infections, trauma, hematologic conditions (thrombocytopenia, polycythemia), Takotsubo cardiomyopathy, and T3 thyrotoxicosis. Iatrogenic elements may be classified into three main categories: medication, diagnostic tests and techniques, and surgical procedures. The first group is dominated by anticoagulant and antiplatelet drugs; additionally, at a low level of statistical evidence, we mention androgen deprivation therapy for prostate cancer, chemotherapy, thyroxine therapy, oral contraceptives, and phosphodiesterase 5 inhibitors. The second category includes a dexamethasone suppression test, clomiphene use, combined endocrine stimulation tests, and a regadenoson myocardial perfusion scan. The third category involves major surgery, laparoscopic surgery, coronary artery bypass surgery, mitral valvuloplasty, endonasal surgery, and lumbar fusion surgery in a prone position. PA in PitNETs still represents a challenging condition requiring a multidisciplinary team from first presentation to short- and long-term management. Controversies involve the specific panel of risk factors and adequate protocols with concern to neurosurgical decisions and their timing versus conservative approach. The present decade-based analysis, to our knowledge the largest so far on published cases, confirms a lack of unanimous approach and criteria of intervention, a large panel of circumstantial events, and potential triggers with different levels of statistical significance, in addition to a heterogeneous clinical picture (if any, as seen in subacute PA) and a spectrum of evolution that varies from spontaneous remission and control of PitNET-associated hormonal excess to exitus. Awareness is mandatory. A total of 25 cohorts have been published so far with more than 10 PA cases/studies, whereas the largest cohorts enrolled around 100 patients. Further studies are necessary.

Long-Term Follow-up of Parenteral Bevacizumab In Patients with Recurrent Respiratory Papillomatosis.

The clinical course of recurrent respiratory papillomatosis (RRP) varies from spontaneous remission to severe airway obstruction with wide variability in recurrence. Standard treatment involves debulking to improve voice and/or breathing. Non-surgical therapies are emerging in hopes of non-operative disease control. This retrospective review analyzes long-term safety, efficacy, and durability of clinical control in the largest reported series of parenteral bevacizumab in adults with RRP. Twenty-three patients with known RRP who have been receiving off-label systemic bevacizumab were included. Dosage, infusion interval, number of cycles, debulking requirements, subjective outcomes, adverse events, and reasons for treatment termination were investigated. Patients have been followed for an average of 791.43 (21-1468) days. The most common starting dosing regimen was 15 mg/kg at 3 weeks in 11 followed by 10 mg/kg at 6 weeks intervals in 6 individuals. Long-term maintenance dosage varied with the least intensive regimen being 10 mg/kg at 14-week intervals. Subjective improvement of voice and/or breathing was reported in 18/23 subjects. The median time for patients that needed a procedure after treatment was 634 days. Procedures after infusions decreased from 3.08 ± 2.48 procedures in the year prior to 0.52 ± 1.12 during systemic Bevacizumab, and to 0.86 ± 2.05 after stopping bevacizumab. Therapy termination occurred in 8 subjects where only 3 were due to adverse events. Parenteral bevacizumab remains a well-tolerated treatment for patients with recalcitrant RRP. There appears to be a durable reduction in the frequency of debulking surgery requirements although on a maintenance regimen. Laryngoscope, 133:2725-2733, 2023.

Stepping Down Treatment in Chronic Spontaneous Urticaria: What We Know and What We Don't Know.

In chronic spontaneous urticaria (CSU), wheals, angioedema, or both appear spontaneously for >6 weeks. Current recommended treatment options for urticaria target mast cell mediators such as histamine, or activators, such as autoantibodies. The goal of CSU treatment is to treat the disease until it is gone as effectively and safely as possible. As no cure is available for CSU as of now, the treatment is aimed at continuously suppressing disease activity, with complete control of the disease and a normalization of quality of life. To achieve this, pharmacological treatment should be continued until no longer needed. Treatment of CSU should follow the basic principles of treating as much as needed and as little as possible taking into consideration that the activity of the disease may vary. Since CSU is a disease with spontaneous remission, it is hard to tell, in patients with complete control and no signs or symptoms, when medication is no longer needed. The current international guideline for urticaria suggests that the treatment can be stepped down once a patient is free of signs and symptoms. Other reasons for stepping down the treatment of CSU patients include safety concerns or issues, pregnancy or wanting to become pregnant, and economic factors. As of now, it is unclear over which period, with what intervals and with which dosages CSU treatment should be stepped down. Guidance on this is needed for all recommended therapies: (i) standard-dosed second-generation H1-antihistamine (sgAH), (ii) higher than standard-dosed sgAH, (iii) standard-dosed omalizumab, (iv) higher than standard-dosed omalizumab, and (v) cyclosporine. However, there is a lack of controlled trials on the step down and discontinuation of these treatments. Here, we aim to provide a summary of what is known and what needs to be investigated in further studies, based on our own experience and real-world evidence.

Analysis of the efficacy and safety of coblation and pulsed radiofrequency in the treatment of cervicogenic headache

Objective: To explore the efficacy and safety of coblation and pulsed radiofrequency on cervicogenic headache (CEH). Methods: A total of 118 patients with CEH who underwent coblation or pulsed radiofrequency in the Department of Pain Management at Xuanwu Hospital, Capital Medical University from August 2018 to June 2020 was retrospectively collected. Patients were divided into the coblation group (n=64) and the pulsed radiofrequency group (n=54) according to different surgical methods. In the coblation group, there were 14 males and 50 females, aged 29-65 (49.8±10.2) years, while in the pulse radiofrequency group, there were 24 males and 30 females, aged 18-65 (41.7±14.8) years. Visual analogue scale (VAS) score, postoperative numbness in the affected areas and other complications were recorded and compared between the two groups at preoperative 3 d, and 1 month, 3 months and 6 months postoperatively. Results: The VAS score of the coblation group was (7.16±0.91), (3.67±1.13), (1.59±0.91), (1.66±0.84) and (1.56±0.90) before operation, and 3 days, 1 month, 3 months and 6 months after surgery. Likewise, the VAS score of the pulsed radiofrequency group was (7.01±0.78), (1.58±0.88), (1.57±0.94), (3.71±1.08) and (6.92±0.83) at the aforementioned time points. There were statistically significant differences of VAS scores between the coblation group and the pulsed radiofrequency group at 3 days, 3 months and 6 months postoperatively (all P<0.001). Intra-group comparison revealed that VAS scores in the coblation group were significantly lower than those before surgery at all time points after surgery (all P<0.001), while VAS scores in the pulsed radiofrequency group were significantly decreased at 3 days, 1 month and 3 months after surgery (P<0.001). The incidence of numbness was 72% (46/64), 61% (39/64), 6% (4/64) and 3% (2/62) in the coblation group and 7% (4/54), 7% (4/54), 2% (1/54) and 0 (0/54) in the pulsed radiofrequency group, respectively. At 3 days and 1 month after surgery, the incidence of numbness in the coblation group was higher than those in the pulsed radiofrequency group (both P<0.001). In the coblation group, one patient developed pharyngeal discomfort 3 days after surgery, which disappeared spontaneously 1 week after surgery without special treatment. One patient developed vertigo after getting up in the morning at 3 days postoperatively, and the possibility of transient cerebral ischemia was considered. In the pulsed radiofrequency group, one patient developed nausea and vomiting after operation, but spontaneous remission was observed after one hour without special treatment. Conclusions: Both coblation and pulsed radiofrequency are effective and safe in the treatment of CEH. But the VAS scores at 3 and 6 months after coblation is significantly lower than those of pulsed radiofrequency ablation group, and the efficacy is better in those undergoing coblation.

Rituximab as Initial Therapy in Adult Patients With Minimal Change Disease

Rituximab as Initial Therapy in Adult Patients With Minimal Change Disease

Transient profound hearing loss and severe facial nerve palsy in schwannomas within the internal acoustic canal: A case report.

Vestibular schwannoma is the most common intracranial schwannoma and constitutes ~8% of all intracranial tumors in adults with an estimated incidence rate of ~1.3/100.000. Facial nerve schwannomas and cochlear nerve schwannomas are rare, and information regarding incidence rates of these is still missing in the literature. All three variants of nerve origin present most frequently with unilateral hearing loss, unilateral tinnitus and disequilibrium. Facial nerve palsy is a common finding in facial nerve schwannomas but a rare finding with vestibular schwannomas. The symptoms are normally persistent and often progress over time, leading to therapeutic interventions that predispose to quality of life-reducing morbidities, e.g., deafness and/or imbalance. The case report describes a 17-year-old male who, during a 1-month period, presented with profound unilateral hearing loss and severe facial nerve palsy followed by complete remission. An MRI scan showed a 5×8-mm schwannoma within the internal acoustic canal. Profound hearing loss together with concomitant severe peripheral facial nerve palsy in small schwannomas within the internal acoustic canal may show spontaneous and total remission within weeks of symptom debut. This knowledge, as well as possible remission of objective findings, should be considered before interventions with potential severe morbidity are suggested.

A review of rheumatoid meningitis with case studies.

Rheumatoid arthritis (RA) is an autoimmune disease involving synovial joints, and it is known that extra-articular manifestations that may affect the central and peripheral nervous systems may develop during its course. Rheumatoid meningitis is very rare among all neurological involvements. In this study, cases diagnosed as rheumatoid meningitis with clinical, imaging, laboratory, and histopathological features are presented, and the aim of the study is to present current approaches in the diagnosis and treatment of rheumatoid meningitis in the light of case studies and current literature. The data of the patients who were followed up with the diagnosis of rheumatoid meningitis in neurology clinic between 2017 and 2021 were reviewed retrospectively. Three cases diagnosed with rheumatoid meningitis are presented in detail. In the first case, the diagnosis was reached by clinical, imaging, and laboratory findings as well as treatment response, while the diagnosis was made by histopathological verification in the second case. The third case shows that spontaneous remission can be observed in the course of rheumatoid meningitis. Rheumatoid meningitis, which is one of the rarest involvements in the course of RA, may present with headaches, focal neurological deficits, seizures, and altered consciousness. A meningeal biopsy is recommended when the differential diagnosis cannot be ruled out with imaging and laboratory findings. In the differential diagnosis Mucobacterium tuberculosis, syphilis, neuro-sarcoidosis, immunoglobulin G4-related disease, lymphoproliferative diseases, and systemic metastasis should be kept in mind. Aggressive RA management is recommended for treatment.

A case of type 2 autoimmune pancreatitis with spontaneous remission.

A 70-year-old man with epigastric pain was referred to our hospital. Computed tomography and magnetic resonance imaging showed the diffusely enlarged pancreas compared to his normal pancreas 6months prior to presentation. Serum levels of IgG4 and amylase were normal, while C-reactive protein was slightly elevated. Endoscopic ultrasound-guided fine-needle biopsy of the pancreas revealed acinar-ductal metaplasia with neutrophil infiltration and without infiltration of IgG4-positive plasma cells. After the clinical diagnosis of type 2 autoimmune pancreatitis (AIP), his symptoms spontaneously improved without steroid therapy. Three months later, radiological findings showed improved pancreas size and serological findings. The pathological diagnosis of type 2 AIP using endoscopic ultrasound-guided fine-needle biopsy is challenging, particularly for proving granulocyte epithelial lesions. This was a valuable type 2 AIP case in which the images before, at the time of onset, and at the time of spontaneous remission were evaluated.

Clinical characteristics of membranous nephropathy with spontaneous remission: An analysis of 24 patients

PurposeTo investigate the prognosis of patients with spontaneous remission (SR) of phospholipase A2 receptor (PLA2R)-associated membranous nephropathy (MN). Patients and methodsPatients diagnosed with MN were recruited after examining their renal biopsy in the Renal Department of China-Japan Friendship Hospital between January 2015 and September 2021. Among them, 24 patients with SR were included in this study and follow-up. ResultsTwenty-four patients diagnosed with SR of PLA2R-associated MN were recruited; 11 were male, and 13 were female, with a mean age of 49.5±14.5 years (range, 30–77 years). The initial 24-hour urinary total protein and serum albumin levels were 0.29±0.14g/d and 37.5±4.4g/L, respectively, and the initial serum creatinine was 65.0±15.8μmol/L. During the follow-up of 33.9±19.1 months (range, 6–73 months), 22 (91.7%) patients maintained remission; however, one patient had impaired renal function due to acute coronary syndrome and coronary angiography findings, and one patient experienced a repeated relapse caused by respiratory tract infection, at 50 and 70 months. A systematic review of the relevant literature was conducted, and records of patients with SR of PLA2R-associated MN were retrieved from 16 case reports or case series with a total of 97 cases. ConclusionsMost patients with SR of MN had a promising long-term prognosis, with only a few cases of relapse.

Reccurent painful ophthalmoplegic neuropathy

Reccurent painful ophthalmoplegic neuropathy (the Tolosa–Hunt syndrome) occurs due to a nonspecific granulomatous infl ammation of the walls of the cavernous sinus and the intracavernous segment of the internal carotid artery. It consists of one or more oculomotor nerves dysfunction associated with peri- and retroorbital pain; spontaneous remissions and relapses are typical. Oculomotor nerve disorders may coincide with the onset of pain or follow it within a period of up to 2 weeks. Demonstration of granulomatosis in the walls of the cavernous sinus and the intracavernous segment of the internal carotid artery, superior orbital fissure or orbit by MRI or biopsy confi rms the diagnosis of Tolosa–Hunt syndrome (THS). Corticosteroid therapy leads to rapid (within 72 hours) pain relief and recovery of oculomotor nerve function. Here, we report a case of reccurent THS in 47-year-old men. Neuroimaging schowed a nonspecific inflammation in the superior orbital fissure-cavernous sinus region on left side and contrast-enhanced thickening dura mater.

Advances in management of pediatric chronic immune thrombocytopenia: a narrative review.

Immune thrombocytopenia (ITP) is a disease in which thrombocytopenia occurs because of immune-mediated platelet destruction and decreased platelet production. Although many pediatric patients with ITP experience spontaneous remission or reach remission within 12 months of first-line therapy, approximately 20% progress to chronic ITP. Patients who do not respond to first-line treatment or experience frequent relapses are of great concern to physicians. This review summarizes recent treatments for second-line treatment of pediatric chronic ITP.

Phospholipase A2 Receptor Antibodies and Clinical Prognosis in Patients with Idiopathic Membranous Nephropathy: An Updated Systematic Review and Meta-Analysis

Background: Idiopathic membranous nephropathy (IMN) is the most common form of primary nephrotic syndrome in adults. Antibodies against the M-type phospholipase A2 receptor (PLA2R-ab) are considered as diagnostic biomarkers of IMN. Objective: Here, we performed an updated meta-analysis to assess the diagnostic value of PLA2R-ab for clinical remission in IMN patients. Method: PubMed, Embase, and Cochrane databases were searched for relevant studies published before September 2022. Odds ratios and corresponding 95% confidence intervals were determined using a fixed or random effects model. The heterogeneity among studies was explored by subgroup analysis. Results: Sixteen studies involving 1,761 IMN participants were included. There were significant differences between PLA2R-ab (+) and PLA2R-ab (−) patients in terms of complete remission (CR) and spontaneous remission. The rates of partial remission (PR) and relapse were similar between the two groups. Patients with PLA2R-ab (−) were at a higher CR rate when treated with a calcineurin inhibitor or a treatment course for 3 months and 6 months, while the spontaneous remission rate was higher in PLA2R-ab seronegative patients from Asia. However, the CR and spontaneous remission rate only significantly declined in IMN patients with the highest titer, but not a middle titer, when compared to those with the lowest titer. Conclusion: In contrast with previous meta-analyses, our results verified that PLA2R-ab can likely predict CR and spontaneous remission in IMN patients, instead of PR and relapse. Race, immunosuppressive agents, and duration of treatment may affect the prognostic value of PLA2R-ab. Considering that the remission rate of IMN patients with a middle level of PLA2R-ab was not different from that of patients with the lowest level, a proper cut-off value of PLA2R-ab for prognosis should be clarified.