Complete remission after glucocorticoid therapy in a patient with primary hypophysitis.

Summary: Primary hypophysitis is a rare disease that may have variable clinical presentations. The main treatment options are clinical observation, immunosuppressive drugs, and surgery. Glucocorticoids are used as first-line medical therapy; however, non-responsiveness and recurrences are the major problems. We present a 30-year-old male patient who had an excellent radiologic response to a single course of glucocorticoids. The patient presented with malaise and severe headaches of acute onset. Cranial MRI revealed a pituitary mass compressing the optic chiasm. Hormonal evaluation studies were consistent with anterior pituitary hormone dysfunction except for the growth hormone axis. There was a mild compression on the optic chiasm in the pituitary MRI. The patient was started on methylprednisolone therapy at a dose of 80 mg/day. The pituitary MRI revealed complete regression of the mass after 2 months, and there was a complete recovery of pituitary functions after 6 months. There is no consensus on the optimal dose and duration of glucocorticoid therapy for primary hypophysitis in the literature. We report that steroid therapy, even in lower doses, might be effective in mild-to-moderate cases. Learning points: Primary hypophysitis is a rare disease with a varied clinical course, and hence the treatment strategies should be individualized. There is no consensus on the optimal dose and duration of glucocorticoid therapy. Glucocorticoid therapy may induce complete remission in some patients, especially with a mild-to-moderate disease course and during the acute phase of the disease.

Clinico-Epidemiologic Profile and Response to Levocetirizine in Chronic Spontaneous Urticaria: A Retrospective Cohort Study from a Tertiary Care Center in North India.

Background: Comprehensive long-term follow-up data regarding chronic spontaneous urticaria (CSU) among general populations, especially from the Indian subcontinent is scanty. Aim and Objectives: The aim of the study were to analyze the clinico-epidemiological profile, comorbidities of CSU patients, and factors affecting patient response to various doses of levocetirizine. Materials and Methods: In this retrospective cohort study, complete history regarding demographic profile, clinical examination, investigations, treatment given, and follow-up details of all CSU patients attending urticaria clinic between 2010 and 2019 were analyzed. These were considered variables to determine the factors playing a role in response to various doses of levocetirizine. Results: Totally, 1104 files of CSU were analyzed. The male-to-female ratio was 1:1.5 with a mean age of 33.03 ± 14.33 years. Thyroid dysfunction and atopy were seen in 142 (12.8%) and 184 (16.7%) patients, respectively. Vitamin D deficiency and high serum immunoglobulin E (IgE) levels were seen in 461 (41.7%) and 340 (30.7%) patients, respectively. Immunosuppressives were required at some point in 196 (17.7%) patients. Patients with higher levels of serum IgE and D-dimer (P < 0.05) were found to require frequent updosing of levocetirizine, while age, sex, duration of illness, presence of angioedema, co-morbidities, identifiable precipitating factors, presence of diurnal variation, family history, and vitamin D deficiency were found to not have an effect on levocetirizine dosing. Conclusion: Ours is a large single-center study exemplifying the biomarkers including baseline serum IgE and D-dimer levels, which could identify a CSU patient who could warrant a higher dose of antihistamine/antihistamine refractory urticaria.

Presumed Fungal Retinitis Following Adalimumab Therapy for Juvenile Idiopathic Arthritis: A Rare Case Report.

PURPOSE: To report a case of presumed fungal infection in a patient with JIA following prolong immunosuppression, and after initiation of adalimumab therapy. Method: Retrospective Chart Review. RESULT: A 20-year-old female, previously diagnosed with JIA, presented with a three-week history of blurred vision in her left eye. She had a long history of treatment with oral corticosteroids, sulfasalazine, and methotrexate, followed by tocilizumab injections and later etanercept. Recently, she was started on adalimumab injections. Fundus examination of the left eye demonstrated multifocal retinitis scattered throughout the fundus. Optical coherence tomography of the lesions showed hyperreflectivity in the inner retina with posterior shadowing and vitreous aggregates extending into the vitreous cavity. After her second adalimumab dose, she experienced blurred vision. Examination of the fundus revealed multifocal retinitis in the left eye, sparing the macula. After stopping immunomodulators and starting empirical antifungal therapy with oral fluconazole, her retinal lesions began to improve. A vitreous biopsy was performed, and intravitreal voriconazole was administered, but microbiological tests were negative. Nevertheless, her retinal lesions resolved almost completely with continued antifungal treatment. By the 6-week follow-up, her retinitis had fully resolved, maintaining excellent visual acuity. CONCLUSION: This case underscores the need for a high index of suspicion for infection in patients with long-term immunosuppression, highlighting the importance of early therapeutic intervention.

Retinal Vasoproliferative Tumours in Uveitis.

PURPOSE: To describe a cohort of patients with retinal vasoproliferative tumours (RVPT) seen in the uveitis clinic of a single tertiary eye care institute in India. METHODS: Retrospective Chart Review. RESULTS: In our study of 17 patients with 18 eyes affected by RVPTs, 94.1% showed unilateral involvement, one patient had bilateral RVPTs, and another had two RVPTs in the same eye. The mean age was 36.3 ± 15.1 years (range: 12-63 years), with a male majority (76.4%). The most common location was inferotemporal quadrant (44.4%). The most common associated uveitic condition was intermediate uveitis (50%). Patients were treated with oral corticosteroid (in 94.1%) and immunosuppressive (35.2%) in addition to transconjunctival cryotherapy (in 52.9%) or laser photocoagulation (in 41.1%). Regression of the RVPT was achieved in all cases except one. Visual acuity at time of detection of RVPT ranged from 6/6 to HM. No significant change was noted in the mean best corrected visual acuity pre and post treatment in the study group. CONCLUSION: RVPTs, rare peripheral retinal lesions often associated with intermediate uveitis, require early diagnosis with targeted therapy to prevent vision loss and achieve favorable long-term outcomes with rare recurrences.

Abdominal aortic aneurysm healed with calcification in a female patient with Behçet's syndrome: A case report with a 2-decade follow-up.

Abdominal aortic aneurysms (AAA) are rare in Behçet syndrome (BS) but may result in life-threatening complications. AAA are generally treated with surgical intervention following aggressive immunosuppressive therapy. Healing with wall calcification and mural thrombus for AAA is scarcely reported in the literature. Herein, we present a female patient who developed a large saccular aneurysm in the infra-renal abdominal aorta 6 months after the diagnosis. Endovascular intervention was not found to be possible because of the dimensions of the aneurysm and the patient did not consent for open surgery. Therefore, she received only immunosuppressive treatment. By the second year of follow-up, we detected reduction in the size of AAA, along with circumferential wall calcification and mural thrombus. The patient was followed-up for 20 years without rupture and currently doing well. This case demonstrates that calcified large saccular AAA might not necessitate surgical intervention and be followed-up for many years without any complication.

Withdrawal of Immunomodulators or TNF Antagonists in Patients With Inflammatory Bowel Diseases in Remission on Combination Therapy: A Systematic Review and Meta-analysis.

BACKGROUND & AIMS: Withdrawal of immunomodulators (IMMs) or tumor necrosis factor (TNF) antagonists in patients with inflammatory bowel diseases (IBDs) in remission on combination therapy is attractive. We evaluated the efficacy and safety of (1) IMM, or (2) TNF antagonist withdrawal in patients with IBD in sustained remission on combination therapy. METHODS: Through a systematic review till March 31, 2023, we identified randomized controlled trials (RCTs) that compared the efficacy and safety of IMM or TNF antagonist withdrawal vs continued combination therapy, in patients with IBD in sustained corticosteroid-free clinical remission for >6 months on combination therapy. Primary outcome was risk of relapse and serious adverse events at 12 months. We conducted meta-analysis to calculate relative risk (RR) and 95% confidence interval (CI) and used Grading of Recommendations Assessment, Development and Evaluation (GRADE) to appraise certainty of evidence. RESULTS: We identified 8 RCTs with 733 patients (77% with Crohn's disease, 91% on infliximab-based combination therapy). On meta-analysis of 5 RCTs, there was no difference in the risk of relapse between patients with IMM withdrawal (continued TNF antagonist monotherapy) vs continued combination therapy (16.8% vs 14.9%; RR, 1.15; 95% CI, 0.75-1.76) without heterogeneity (low certainty of evidence). TNF antagonist withdrawal (continued IMM monotherapy) was associated with 2.4-times higher risk of relapse compared with continuing combination therapy (31.5% vs 11.2%; RR, 2.35; 95% CI, 1.38-4.01), with minimal heterogeneity (low certainty of evidence). There was no difference in the risk of serious adverse events with IMM or TNF antagonist withdrawal vs continued combination therapy. CONCLUSIONS: In patients with IBD in sustained corticosteroid-free clinical remission for >6 months on combination therapy, de-escalation with TNF antagonist withdrawal, but not IMM withdrawal, was associated with an increased risk of relapse.

Acute pancreatitis caused by Systemic Lupus Erythematosus activity: A case report and literature review.

A 32-year-old woman with systemic lupus erythematosus came to the rheumatology outpatient clinic reporting abdominal pain for a week, along with fever, arthralgias, myalgias, alopecia, asthenia and dyspnea on exertion over the last two months. She was hypotensive and tachycardic, requiring admission to the intensive care unit. She was diagnosed with lupus-related acute pancreatitis, an unusual complication occurring in less than 1% of cases. Most cases are mild and self-limited; however, severe and life-threatening events with multiple organ failure are possible. This article is a case report of lupus-related critical acute pancreatitis, and a literature review.

Mujer de 32 años con lupus eritematoso sistémico acude a consulta externa de reumatología por dolor abdominal de una semana de evolución, además de fiebre, artralgias, mialgias, alopecia, astenia y disnea de esfuerzo de 2 meses de evolución. También presentó hipotensión y taquicardia, por lo que requirió ingreso en la unidad de cuidados intensivos. Le diagnosticaron pancreatitis aguda relacionada con el lupus, que es una complicación inusual que ocurre en menos del 1% de los pacientes. La mayoría de los casos son leves y autolimitados, sin embargo, es posible que se presenten eventos graves y potencialmente mortales, con disfunción multiorgánica. Este artículo es un reporte de caso de una pancreatitis aguda crítica relacionada con lupus y una revisión de la literatura.

Acute pancreatitis caused by Systemic Lupus Erythematosus activity: a case report and literature review / Pancreatitis aguda por actividad del lupus eritematoso sistémico: reporte de caso y revisión de la literatura

A 32-year-old woman with systemic lupus erythematosus came to the rheumatology outpatient clinic reporting abdominal pain for a week, along with fever, arthralgias, myalgias, alopecia, asthenia and dyspnea on exertion over the last two months. She was hypotensive and tachycardic, requiring admission to the intensive care unit. She was diagnosed with lupus-related acute pancreatitis, an unusual complication occurring in less than 1% of cases. Most cases are mild and self-limited; however, severe and life-threatening events with multiple organ failure are possible. This article is a case report of lupus-related critical acute pancreatitis, and a literature review.

Mujer de 32 años con lupus eritematoso sistémico acude a consulta externa de reumatología por dolor abdominal de una semana de evolución, además de fiebre, artralgias, mialgias, alopecia, astenia y disnea de esfuerzo de 2 meses de evolución. También presentó hipotensión y taquicardia, por lo que requirió ingreso en la unidad de cuidados intensivos. Le diagnosticaron pancreatitis aguda relacionada con el lupus, que es una complicación inusual que ocurre en menos del 1% de los pacientes. La mayoría de los casos son leves y autolimitados, sin embargo, es posible que se presenten eventos graves y potencialmente mortales, con disfunción multiorgánica. Este artículo es un reporte de caso de una pancreatitis aguda crítica relacionada con lupus y una revisión de la literatura.

Third dose of BNT162b2 improves immune response in liver transplant recipients to ancestral strain but not Omicron BA.1 and XBB.

Vaccine immunogenicity in transplant recipients can be impacted by the immunosuppressive (IS) regimens they receive. While BNT162b2 vaccination has been shown to induce an immune response in liver transplant recipients (LTRs), it remains unclear how different IS regimens may affect vaccine immunogenicity after a third BNT162b2 dose in LTRs, which is especially important given the emergence of the Omicron sublineages of SARS-CoV-2. A total of 95 LTRs receiving single and multiple IS regimens were recruited and offered three doses of BNT162b2 during the study period. Blood samples were collected on days 0, 90, and 180 after the first BNT162b2 dose. At each time point, levels of anti-spike antibodies, their neutralizing activity, and specific memory B and T cell responses were assessed. LTRs receiving single IS regimens showed an absence of poor immunogenicity, while LTRs receiving multiple IS regimens showed lower levels of spike-specific antibodies and immunological memory compared to vaccinated healthy controls after two doses of BNT162b2. With a third dose of BNT162b2, spike-specific humoral, memory B, and T cell responses in LTR significantly improved against the ancestral strain of SARS-CoV-2 and were comparable to those seen in healthy controls who received only two doses of BNT162b2. However, LTRs receiving multiple IS regimens still showed poor antibody responses against Omicron sublineages BA.1 and XBB. A third dose of BNT162b2 may be beneficial in boosting antibody, memory B, and T cell responses in LTRs receiving multiple IS regimens, especially against the ancestral Wuhan strain of SARS-CoV-2. However, due to the continued vulnerability of LTRs to presently circulating Omicron variants, antiviral treatments such as medications need to be considered to prevent severe COVID-19 in these individuals.

Necrotizing Scleritis: A Review.

Necrotizing scleritis is the most destructive and vision-threatening form of scleritis. Necrotizing scleritis can occur in systemic autoimmune disorders and systemic vasculitis, as well as following microbial infection. Rheumatoid arthritis and granulomatosis with polyangiitis remain the commonest identifiable systemic diseases associated with necrotising scleritis. Pseudomonas species is the most common organism causing infectious necrotizing scleritis, with surgery the most common risk factor. Necrotizing scleritis has the highest rates of complications and is more prone to secondary glaucoma and cataract than other phenotypes of scleritis. The differentiation between non-infectious and infectious necrotizing scleritis is not always easy but is critical in the management of necrotizing scleritis. Non-infectious necrotizing scleritis requires aggressive treatment with combination immunosuppressive therapy. Infectious scleritis is often recalcitrant and difficult to control, requiring long-term antimicrobial therapy and surgical debridement with drainage and patch grafting due to deep-seated infection and the avascularity of the sclera.

Earlier and more aggressive treatment with biologics may prevent relapses and further new organ involvement in Behçet's disease.

OBJECTIVE: Immunosuppressives (IS) are the choice of treatment for major organ involvement in Behçet's disease (BD). In this study, we aimed to investigate the relapse rate and new major organ development in BD under ISs during long-term follow-up. METHODS: The files of 1114 BD patients followed in Marmara University Behçet's Clinic were analyzed retrospectively. Patients with a follow-up less than 6 months were excluded. Conventional IS and biologic treatment courses were compared. 'Events under IS' were defined as a relapse of the same organ and/or new major organ development in patients receiving ISs. RESULTS: Among 806 patients included in the final analysis (male: 56%, age at diagnosis: 29 (23-35) years, median follow-up time: 68 (33-106) months). Major organ involvement was present in 232 (50.5%) patients at diagnosis, and 227 (49.5%) developed new major organ involvement during follow-up. Major organ involvement developed earlier in males (p = 0.012) and in patients with a first-degree relative history of BD (p = 0.066). ISs were given mostly for major organ involvement (86.8%, n = 440). Overall, 36% of the patients had a relapse or new major organ involvement under ISs (relapse: 30.9%, new major organ involvement: 11.6%.) 'Events under IS' (35.5% vs 20.8%, p = 0.004), and relapses (29.3% vs 13.9%, p = 0.001) were more common with conventional ISs compared to biologics. CONCLUSION: Any major event under ISs was less common with biologics compared to conventional ISs in patients with BD. These results suggest that earlier and more aggressive treatment may be an option in BD patients who had the highest risk for severe disease course.

Current pharmacological solutions for Behçet's syndrome.

INTRODUCTION: Behçet's syndrome (BS) has a heterogeneous clinical phenotype, and its clinical manifestations may respond differently to drugs commonly used to treat BS. The type, dose, and duration of immunomodulatory, immunosuppressive, and biologic agents should be tailored individually. AREAS COVERED: We reviewed the literature for articles on BS management that were published until June 2022 and summarized the management options in BS for each type of organ involvement. We aimed to cover all currently available pharmacological agents used in BS, as well as surgical and interventional options, focusing on recent evidence. EXPERT OPINION: The management aims in BS are to preserve function and quality of life and to avoid damage. The choice of treatment modalities depends on the organs that are actively involved, the severity of that involvement, and prognostic factors. A treat-to-attack strategy would help improve long-term outcomes in BS.

Comparative Safety and Effectiveness of Biologic Therapy for Crohn's Disease: A CA-IBD Cohort Study.

BACKGROUND & AIMS: We compared the safety and effectiveness of tumor necrosis factor α (TNF-α) antagonists vs vedolizumab vs ustekinumab in patients with Crohn's disease (CD) in a multicenter cohort (CA-IBD). METHODS: We created an electronic health record-based cohort of adult patients with CD who were initiating a new biologic agent (TNF-α antagonists, ustekinumab, vedolizumab) from 5 health systems in California between 2010 and 2017. We compared the risk of serious infections (safety) and all-cause hospitalization and inflammatory bowel disease-related surgery (effectiveness) between different biologic classes using propensity score (PS) matching. RESULTS: As compared with TNF-α antagonists (n = 1030), 2:1 PS-matched, ustekinumab-treated patients with CD (n = 515) experienced a lower risk of serious infections (hazard ratio [HR], 0.36; 95% CI, 0.20-0.64), without any difference in the risk of hospitalization (HR, 0.99; 95% CI, 0.89-1.21) or surgery (HR, 1.08; 95% CI, 0.69-1.70). Compared with vedolizumab (n = 221), 1:1 PS-matched, ustekinumab-treated patients with CD (n = 221) experienced a lower risk of serious infections (HR, 0.20; 95% CI, 0.07-0.60), without significant differences in risk of hospitalization (HR, 0.76; 95% CI, 0.54-1.07) or surgery (HR, 1.42; 95% CI, 0.54-3.72). Compared with TNF-α antagonists (n = 442), 2:1 PS-matched, vedolizumab-treated patients with CD (n = 221) had a similar risk of serious infections (HR, 1.53; 95% CI, 0.84-2.78), hospitalization (HR, 1.32; 95% CI, 0.98-1.77), and surgery (HR, 0.63; 95% CI, 0.27-1.47). High comorbidity burden, concomitant opiate use, and prior hospitalization were associated with serious infections and hospitalization in biologic-treated patients with CD. CONCLUSION: In a multicenter cohort of biologic-treated patients with CD, ustekinumab was associated with a lower risk of serious infections compared with TNF-α antagonists and vedolizumab, without any differences in risk of hospitalization or surgery. The risk of serious infections was similar for TNF-α antagonists vs vedolizumab.

Effectiveness and Safety of Biologic Therapy in Hispanic Vs Non-Hispanic Patients With Inflammatory Bowel Diseases: A CA-IBD Cohort Study.

BACKGROUND & AIMS: There are limited data on outcomes of biologic therapy in Hispanic patients with inflammatory bowel diseases (IBDs). We compared risk of hospitalization, surgery, and serious infections in Hispanic vs non-Hispanic patients with IBD in a multicenter, electronic health record-based cohort of biologic-treated patients. METHODS: We identified adult patients with IBD who were new users of biologic agents (tumor necrosis factor α [TNF-α] antagonists, ustekinumab, vedolizumab) from 5 academic institutions in California between 2010 and 2017. We compared the risk of all-cause hospitalization, IBD-related surgery, and serious infections in Hispanic vs non-Hispanic patients using 1:4 propensity score matching and survival analysis. RESULTS: We compared 240 Hispanic patients (53% male; 45% with ulcerative colitis; 73% TNF-α antagonist-treated; 20% with prior biologic exposure) with 960 non-Hispanic patients (51% male; 44% with ulcerative colitis; 67% TNF-α antagonist-treated; 27% with prior biologic exposure). After propensity score matching, Hispanic patients were younger (37 ± 15 vs 40 ± 16 y; P = .02) and had a higher burden of comorbidities (Elixhauser index, >0; 37% vs 26%; P < .01), without any differences in patterns of medication use, burden of inflammation, and hospitalizations. Within 1 year of biologic initiation, Hispanic patients had higher rates of hospitalizations (31% vs 23%; adjusted hazard ratio [aHR], 1.32; 95% CI, 1.01-1.74) and IBD-related surgery (7.1% vs 4.6%; aHR, 2.00; 95% CI, 1.07-3.72), with a trend toward higher risk of serious infections (8.8% vs 4.9%; aHR, 1.74; 95% CI, 0.99-3.05). CONCLUSIONS: In a multicenter, propensity score-matched cohort of biologic-treated patients with IBD, Hispanic patients experienced higher rates of hospitalization, surgery, and serious infections. Future studies are needed to investigate the biological, social, and environmental drivers of these differences.

Post Renal Transplant Malignancies; A Basic Concept.

BACKGROUND: Kidney transplantation remains the best possible solution for patients with chronic kidney disease, providing better long-term outcomes and drastically improving quality of life. However, it comes with its own set of risks. The use of immunosuppressives following renal transplants has been shown to increase the development of malignancies and infections, and the occurrence of post-transplant malignancies is now the third most common cause of death in transplant patients. This involves multiple mechanisms, including the carcinogenic tendency of some immunosuppressive drugs, along with the induction and promotion of post-transplant malignancies by certain viruses. The quantification of Cancer risk must be made an integral part of the overall management of transplant patients, and appropriate follow-up screening needs to be adopted. Kaposi's sarcoma, lymphoma, and non-melanoma skin cancers have a greater incidence. If a malignancy develops immediately after transplantation, it may have been transmitted from the donor; donor-transmitted and donor-derived tumours may be differentiated based on a two-year time limit. Immunosuppressive medications with carcinogenic tendencies, reduced immunological control of oncogenic viruses, and poor immunosurveillance remain the most important risk factors. The gravity of this situation is further exacerbated by the fact that not only is there an increased risk of developing these malignancies in the post-transplant period, but the prognosis is also worsened when compared to non-transplant patients. All transplant centers should therefore adopt a multidisciplinary approach including early detection and prompt treatment, to improve outcomes in transplanted patients.

Evidence-Based Guidelines for SARS-COV-2 Vaccination of Patients of Skin Allergic Diseases and Patients on Immuno-Therapeutics.

There is a dearth of data regarding the safety and timing of the severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) vaccination of patients on immunosuppressive or immunomodulatory therapies. However, data from other vaccine trials may be extrapolated to get an idea regarding the recommendation of SARS-COV-2 vaccines. All the novel SARS-COV-2 vaccines are non-live, thus ensuring the safety of the vaccines. However, the vaccines may not be able to generate an equipotent immunogenic response in patients receiving immunotherapeutics, in comparison to those who are not. We have attempted to put forward certain statements, with respect to SARS-COV-2 vaccination of patients who are on treatment for different dermatological conditions. However, the risk-benefit ratio must be discussed between the patient and the physician, and the final call should be individualized.

Clinical profile, treatment, and visual outcome of scleritis: A single ophthalmologist experience.

PURPOSE: The purpose of this study was to analyze the clinical profile of patients with scleritis managed by a single ophthalmologist in a tertiary eye care center. METHODS: This was a retrospective analysis of 107 eyes of 96 patients with scleritis from January 2007 to December 2018. RESULTS: Female predominance (68%) with a relatively young-onset (46 ± 14 years) of scleritis was observed. Diffuse anterior scleritis (41%) was the most common subtype of scleritis, and the most common systemic association was rheumatoid arthritis (18%). Three-fourth of patients received immunosuppressive treatment (74%) along with corticosteroids. The mean follow-up period was 3 ± 2.5 (range: 0.6-10) years. Necrotizing scleritis was at a 3.5 times higher risk of developing ocular complications. Eighty percent of patients maintained the same vision. Recurrence of scleritis was noted in 25 eyes (23%). CONCLUSION: Diffuse scleritis is the most common scleritis in our population. Tuberculosis was commonly seen with diffuse scleritis. The likelihood of developing ocular complications (cataract and glaucoma) was higher in necrotizing scleritis, thus requiring periodic monitorization.

Extracorporeal photopheresis for the treatment of chronic graft versus host disease.

OBJECTIVES: Chronic graft versus host disease (chronic GVHD) still remains the leading cause of late morbidity and mortality for allogeneic hematopoietic stem cell transplant (allo-HSCT) recipients. In this retrospective study, 53 consecutive allo-HSCT patients with chronic GVHD refractory to corticosteroids were treated with extracorporeal photopheresis (ECP). METHODS: This study was performed as a retrospective single-center study. Medical records of a total of 59 patients treated with ECP for chronic GVHD were reviewed. RESULTS: Best organ responses to ECP were observed in skin, mouth mucosa, eyes and liver. Overall response rate (ORR) to ECP was 81.2% (CR 17% and PR 64.2%). Overall survival (OS) was 84.9% and 36.7%, at 1 and 3 years, respectively. Female sex appears to have an advantage on ORR. Patients achieving ORR were able to maintain their responses with a prolonged continuation of treatments for +6 and +12 months indicating the benefits of longer ECP treatment. DISCUSSION: We found that patients with chronic GVHD who were treated with ECP for 12 months or longer had a higher response rate. Our findings in line with the data reported previously suggest that patients responding to ECP should continue longer therapy schedules to achieve a better and sustained response. In our cohort, long-term ECP therapy was safe and well-tolerated with no significant adverse effects. Best responses were observed in the patients with skin, eye, liver and oral involvement. The ECP procedure offers the advantage relative to the problems with typical immunosuppressive agents. The female sex appeared to have an advantage based on the cumulative probability of the OR after ECP for chronic GVHD.

The Effect of Hydroxychloroquine on Residual Proteinuria in Patients With Immunoglobulin A Nephropathy: A Retrospective Study Based on Propensity Score Matching.

Background: There is insufficient evidence to support the use of hydroxychloroquine (HCQ) in Immunoglobulin A nephropathy (IgAN) patients with high residual proteinuria in spite of 6-month supportive treatment combined with corticosteroids (P) and/or immunosuppressives (IM). This study aims to explore the effect of HCQ on residual proteinuria in IgAN. Materials and Methods: This is a retrospective study. IgAN patients who had residual proteinuria ≥0.3 g/24 h after 6-month treatment by renin-angiotensin system inhibitors (RASI) + P ± IM were included. Groups were divided based on the different regimens and then matched by the propensity score matching method. The primary outcome was defined as the cumulative frequency of residual proteinuria reduction ≥30%. Results: RASI (n = 183), HCQ + RASI (n = 59), RASI + P ± IM (n = 145), and HCQ + RASI + P ± IM (n = 38) groups were included. HCQ + RASI group had a higher level of residual proteinuria and a worse renal function than those in the RASI group. The renal function was worse in the HCQ + RASI + P ± IM group than that in the control group, but residual proteinuria levels were similar. After matching, there were 40 patients in the first two groups and 29 patients in the latter two groups, respectively. The cumulative frequency of residual proteinuria reduction ≥30% in HCQ + RASI + P ± IM group was higher than that in control group (86.2% vs. 62.1%, χ2 = 6.397, p = 0.011). HCQ combination treatment was one of independent factors. Conclusion: The addition of HCQ treatment can effectively reduce the residual proteinuria in IgAN patients previously treated with supportive treatment combined with P and IM treatment and the cumulative frequency of effective reduction of residual proteinuria can reach 86.2%.

Belimumab for systemic lupus erythematosus - Focus on lupus nephritis.

In recent years, advances in the treatment and management of patients with systemic lupus erythematosus (SLE) have improved their life expectancy and quality of life. However, lupus nephritis (LN) still represents a major life-threatening complication of the disease. Belimumab (BEL), a fully human monoclonal IgG1λ antibody neutralizing soluble B cell activating factor, was approved more than ten years ago as add-on therapy in adults and pediatric patients with a highly active, autoantibody-positive disease despite standard of care (SoC). Recently, the superiority of the addition of BEL to SoC was also demonstrated in LN. In this review, we provide a comprehensive overview of the study landscape, available therapeutic options for SLE (focusing on BEL in renal and non-renal SLE), and new perspectives in the treatment field of this disease. A personalized treatment approach will likely become available with the advent of novel therapeutic agents for SLE and LN.