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.

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.

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.

AGA Technical Review on the Medical Management of Moderate to Severe Luminal and Perianal Fistulizing Crohn's Disease.

The incidence and prevalence of Crohn's disease (CD) is rising globally. Patients with moderate to severe CD are at high risk for needing surgery and hospitalization and for developing disease-related complications, corticosteroid dependence, and serious infections. Optimal management of outpatients with moderate to severe luminal and/or fistulizing (including perianal) CD often requires the use of immunomodulator (thiopurines, methotrexate) and/or biologic therapies, including tumor necrosis factor-α antagonists, vedolizumab, or ustekinumab, either as monotherapy or in combination (with immunomodulators) to mitigate these risks. Decisions about optimal drug therapy in moderate to severe CD are complex, with limited guidance on comparative efficacy and safety of different treatments, leading to considerable practice variability. Since the last iteration of these guidelines published in 2013, significant advances have been made in the field, including the regulatory approval of 2 new biologic agents, vedolizumab and ustekinumab. Therefore, the American Gastroenterological Association prioritized updating clinical guidelines on this topic. To inform the clinical guidelines, this technical review was completed in accordance with the GRADE (Grading of Recommendations Assessment, Development and Evaluation) framework. The review addressed the following focused questions (in adult outpatients with moderate to severe luminal CD): overall and comparative efficacy of different medications for induction and maintenance of remission in patients with or without prior exposure to tumor necrosis factor-α antagonists, comparative efficacy and safety of biologic monotherapy vs combination therapy with immunomodulators, comparative efficacy of a top-down (upfront use of biologics and/or immunomodulator therapy) vs step-up treatment strategy (acceleration to biologic and/or immunomodulator therapy only after failure of mesalamine), and the role of corticosteroids and mesalamine for induction and/or maintenance of remission. Finally, in adult outpatients with moderate to severe fistulizing CD, this review addressed the efficacy of pharmacologic interventions for achieving fistula and the role of adjunctive antibiotics without clear evidence of active infection.

Pemphigus vulgaris relapse during the coronavirus disease pandemic.

Pemphigus vulgaris (PV) is the most common type of pemphigus group of autoimmune skin diseases. The treatment of PV relapse is challenging especially during the coronavirus disease (COVID-19) pandemic. In this prospective study, we aimed to evaluate the treatment of patients with relapsing PV during the pandemic. Twelve patients with PV who experienced relapse from March 2020 to January 2022 were included. The patients were asked whether they experienced COVID-19 symptoms and the pemphigus disease area index (PDAI) was measured. PCR for COVID-19, chest computed tomography, routine investigations, and electrocardiography were performed for the admitted patients. The mean PDAI of the patients during relapse was 23.6 ± 14.8 (range 5-60). Seven patients received azathioprine; one patient received mycophenolate mofetil; and six patients received 1000 mg of rituximab (RTX) twice at an interval of 14 days. None of the 12 patients had COVID-19-suggestive symptoms. Only 1 patient relapsed after receiving the COVID-19 vaccine. The six admitted patients who received RTX were negative for COVID-19 based on the PCR testing results. Out of the 12 patients, eight achieved complete remission, while four achieved partial remission. No major adverse effects were observed. In conclusion, the treatments with systemic steroids, immunosuppressive drugs, and rituximab were well tolerated by the patients with relapsing PV, provided that there was no contact with individuals with COVID-19. These treatments can then be provided to patients with PV during the pandemic with careful follow-up.

Risk of Developing Melanoma With Systemic Agents Used to Treat Psoriasis: A Review of the Literature.

BACKGROUND: Psoriasis is a chronic inflammatory skin disease induced by autoimmune-like dysregulation of the immune system. Treatment options have drastically evolved in recent years, and treatment advances that target specific cytokines and other molecules involved in dysregulation have had a profound effect in controlling the disease. OBJECTIVE: We reviewed the literature to assess the risk of developing melanoma with conventional therapies and newer agents used to treat psoriasis. METHODS: A comprehensive literature search using Medline (via Ovid) and Embase was conducted. RESULTS: The majority of studies reviewed reported insignificant results. Potential risk for melanoma was identified for only 3 out of 15 anti-psoriatic treatments analyzed: adalimumab (relative risk 1.8, 95% CI 1.06-3.00), etanercept (relative risk 2.35, 95% CI 1.46-3.77) and infliximab (Empirical Bayes Geometric Mean 7.90, 95% CI 7.13-8.60). The confidence intervals provided are from prior studies. There are not enough collective data on newer agents to make any conclusions on risk. CONCLUSIONS: We were unable to identify any substantial risk for developing melanoma due to the use of anti-psoriatic treatments. Until additional long-term registry data become available, it would be prudent to continue screening patients with psoriasis at baseline and periodically for melanoma when these agents are used.

Advances in the Treatment of Behcet's Disease.

PURPOSE OF REVIEW: To assess current management of Behcet's disease (BD). Controversies on therapeutic approaches to different manifestations, whether conventional immunosuppressives (IS) or biologic agents, should be chosen, and options for refractory disease are discussed. RECENT FINDINGS: Glucocorticoids are still the main agents for remission-induction and azathioprine the first-line conventional IS in maintenance phase to prevent relapses of major organ involvement. Apremilast is shown to be a safe and effective option approved by the FDA for oral ulcers. Large case series confirmed the efficacy and safety of TNFα inhibitors and Interferon-α. Promising results are observed with IL-1 inhibitors, ustekinumab, secukinumab, and tocilizumab for refractory BD. Although both conventional IS and biologic agents are effectively used to suppress inflammation in BD, there is still an unmet need for clear therapeutic strategies in the management for different manifestations. Further controlled studies with new biologic agents, anticoagulants and the benefit of concomitant IS usage with biologics are needed to optimize the management of BD.

[Medications when travelling with underlying inflammatory rheumatic disease]. / Medikamente auf Reisen mit entzündlich-rheumatischer Grunderkrankung.

Travelling poses particular challenges for patients with rheumatic diseases. This article provides specific guidance on how best to manage medication while away from home. Besides outlining advice on basic logistic issues, such as the transportation, importation and storage of drugs, the article concentrates on travelling while receiving immunosuppressive therapy and carrying narcotics. Especially when transporting narcotics, travel requires careful planning in advance in close collaboration with physicians on account of the strict and internationally diverse import restrictions on controlled substances. While travelling, all drugs should be kept in the original packaging, including the package insert and stored in carry-on luggage. A specific medical passport may be needed. Immunosuppressive and narcotic drugs require medical certificates issued by the prescribing physician, which may need to be certified by the responsible national agencies. Patients receiving glucocorticoid treatment who travel in or across multiple time zones should also be aware of how the medication impacts and interacts with circadian rhythms so as to optimize the anti-inflammatory effects of the drugs and to avoid unnecessary complications. Given the significant discrepancies in medical care and availability of medication worldwide, the article further stresses the importance of a comprehensive medical kit tailored to the patient's individual medicinal needs. Finally, as immunocompromised travellers are at increased risk of infections, advice is given on the use of anti-infective drugs and chemoprophylaxis for patients travelling to areas in which malaria is endemic as well as on their possible interactions with immunosuppressive treatment.

Treating systemic lupus erythematosus in the 21st century: new drugs and new perspectives on old drugs.

Besides treating acute flares, the management of SLE should aim at preventing organ damage accrual and drug-associated harms, improving health-related quality of life and prolonging survival. At present, therapy is based on combinations of antimalarials (mainly HCQ), considered the backbone of SLE treatment, glucocorticoids and immunosuppressive drugs. However, these regimens are not universally effective and a substantial degree of damage can be caused by exposure to glucocorticoids. In this review we provide a critical appraisal of the efficacy and safety of available treatments as well as a brief discussion of potentially novel compounds in patients with SLE. We emphasize the use of methylprednisolone pulses for moderate-severe flares, followed by low-moderate doses of oral prednisone with quick tapering to maintenance doses of ≤5 mg/day, as well as the prompt institution of immunosuppressive drugs in the setting of severe disease but also as steroid-sparing agents. Indications for the use of biologic agents, namely belimumab and rituximab, in refractory or organ-threatening disease are also presented. We conclude by proposing evidence- and experience-based treatment strategies tailored to the clinical scenario and prevailing organ involvement that can aid clinicians in managing this complex disease.

Autoimmune Enteropathy: An Updated Review with Special Focus on Stem Cell Transplant Therapy.

Autoimmune enteropathy (AIE) is a complex disease affecting both children and adults. Although associated with significant morbidity and mortality, the pathophysiology of the disease and its treatment have not been well characterized. This study aims to review the medical literature available on this rare but clinically significant ailment, to help establish a better understanding of its pathophysiology and enumerate the available diagnostic and treatment modalities. A literature search was conducted on PubMed using key terms related to autoimmune enteropathy and intractable diarrhea, with no restrictions on the date of publication or language. We found a total of 98 reports of AIE published in the form of case reports and case series. The evidence reviewed suggests that AIE is a multifaceted disorder that requires a high index of suspicion in the appropriate clinical setting to be able to make an early diagnosis. Current evidence supports the use of supportive care to correct nutritional and metabolic deficiencies, and immunosuppressives and immunomodulators as directed therapies. Hematopoietic stem cell transplant is an aggressive, but successful curative modality for patients with AIE as part of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. Cumulative clinical experience with management of AIE has allowed improved outcomes in transplanted and non-transplanted AIE patients even though morbidity and mortality with are still high in patients with this condition. More research is needed to further define the role of new therapies for AIE, and a central registry with participation of multiple institutions might help share and standardize care of patients with this rare but serious condition.

Lupus myocarditis: a single center experience and a comparative analysis of observational cohort studies.

Background Lupus myocarditis (LM) is reported in 3-9% of patients with systemic lupus erythematosus (SLE) but limited evidence exists regarding optimal treatment and prognosis. This study aims to describe LM in a defined lupus cohort as compared with the existing literature. Patients and methods Patients with LM were identified from the University of Toronto Lupus Clinic database. Diagnosis was based on clinical manifestations and electrocardiographic, imaging, and biochemical criteria. Demographic, clinical, diagnostic and therapeutic variables and outcomes were collected in a standardized data retrieval form. A literature review was performed to identify cohort studies reporting on LM treatment and outcome. A comparative analysis was conducted between our patients and the combined cohort of the existing studies. Results Thirty patients were diagnosed with LM (prevalence 1.6%) and compared with a cumulative cohort of 117 patients from five distinct studies. No significant differences were found regarding the age at diagnosis (32.6 ± 13.4 years) and SLE duration (2.5 years median). Concomitant lupus activity from other organ systems was observed in 97% of the patients. Chest pain was more frequently reported in our cohort whereas dyspnea was more prominent in the other studies. Diagnostic criteria were similar across studies. Therapeutic approach was comparable and consisted of glucocorticosteroids (96.6%) and immunosuppressives (70%). Mortality was approximately 20% whereas another 20% of the patients achieved partial and 60% complete recovery. Conclusions LM usually occurs early in the disease course and in the context of generalized lupus activity. Despite aggressive therapy, approximately 40% of the patients died or had residual heart damage.

[Update: polyarteritis nodosa]. / Update: Polyarteriitis nodosa.

Polyarteritis nodosa (PAN) is a necrotizing arteritis of medium-sized vessels, which is often fatal if untreated. It frequently affects the skin (nodules and ulcers), the peripheral nervous system (mononeuritis multiplex) and the visceral vessels (stenoses and microaneurysms). The complex diagnostic work-up requires discriminating PAN from infectious, malignant, drug-induced and other inflammatory conditions. It can be subclassified into further variants (idiopathic, associated with hepatitis B, associated with hereditary inflammatory diseases or isolated cutaneous disease). While idiopathic and hereditary inflammatory variants require immunosuppressive treatment, the hepatitis B-associated variant is treated with virustatic agents and plasmapheresis. The isolated cutaneous variant has a good prognosis and rarely requires highly potent immunosuppressives.

Cyclosporine causes no hearing defect in paediatric patients with nephrotic syndrome.

OBJECTIVE: We aimed to evaluate the ototoxicity of cyclosporine A (CsA) in children with nephrotic syndrome (NS). DESIGN: Data of paediatric patients with NS followed in paediatric nephrology department were evaluated retrospectively, and hearing functions were evaluated by pure tone audiometry (PTA) and transient evoked otoacoustic emissions (TEOAEs). Age, gender, type of NS, duration and cumulative doses of immunosuppressives were noted. STUDY SAMPLE: The patients who had received CsA (n: 16) and immunosuppressives other than CsA (n: 13) for at least 6 months formed two patient groups and healthy cases formed a control group (n: 20). Children with known previous hearing defect, inner ear trauma or surgery, recurrent otitis media and those using hearing aid were excluded. RESULTS: Gender, age at first clinical presentation, laboratory tests and number of relapses were similar between the groups. No hearing loss was defined in PTA at frequencies of 250, 500, 1000, 2000, 4000 and 8000 Hz. The results of TEOAEs were similar between the groups and compatible with normal hearing. CONCLUSIONS: CsA is not responsible for permanent sensorineural hearing loss in children with NS, and there is no sufficient evidence to consider routine hearing assessment in children with NS treated with CsA.

Challenges and open issues in the management of acquired hemophilia A (AHA).

Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by antibodies which neutralize the function of factor VIII (FVIII). The disease presents a complex clinical challenge to the treating Physicians and Hematologists. As the disease is associated with high mortality, prompt management is necessary. Early recognition, quick diagnosis and timely referral to a specialized center are important for better management of these patients. The different clinical manifestations, underlying pathology, inhibitor kinetics and the associated age related comorbidities do not allow extrapolation of the treatment protocols of congenital hemophilia to AHA. The basic strategies of the management of AHA patients involve maintaining hemostasis, suppression or eradication of antibodies, diagnosis and treatment of underlying pathology and avoid treatment related complications like thrombosis. The efficiency of hemostatic agents which are generally used to treat AHA is unpredictable. Due to the rarity of the disease, there are no randomized clinical trials on the management of this disorder and thus the expertise and experience of the treating Physicians' guide treatment strategies.

Glucocorticoid treatment and damage in the anti-neutrophil cytoplasm antibody-associated vasculitides: long-term data from the European Vasculitis Study Group trials.

OBJECTIVE: Granulomatosis with polyangiitis and microscopic polyangiitis are ANCA-associated vasculitides (AAVs). The Vasculitis Damage Index (VDI) quantifies damage. This study aims to determine the factors associated with long-term damage in the AAVs. METHODS: Data from 535 patients from four European Vasculitis Study Group trials were studied. A long-term follow-up (LTFU) questionnaire at 7 years post-diagnosis was completed. The associations between baseline (age, creatinine and BVAS score) and cumulative (number of relapses and duration of glucocorticoid use) factors and damage accrued (total VDI scores and individual treatment-related damage items) during follow-up were explored. Multiple regressions identified independent associations between baseline measures, cumulative factors and VDI scores at LTFU. RESULTS: Two hundred and ninety-six patients had glucocorticoid use and VDI data available at LTFU, with the mean length of glucocorticoid use being 40.4 months (S.D. 16.7). High levels of damage were independently associated with older age at baseline (P = 0.051), lower glomerular filtration rate (P = 0.041), higher BVAS scores (P = 0.046), increased cumulative glucocorticoid use (P = 0.016) and increasing number of relapses. Patients with longer duration of glucocorticoid treatment were more likely to have a total VDI score ≥5 [odds ratio 1.26 per 12 months of glucocorticoid use (95% CI 1.03, 1.53), P = 0.022]. The main limitation is that approximately half of the patients enrolled had no LTFU data available; these patients were older with more severe initial disease. CONCLUSION: Long-term damage in the AAVs may be associated with severity of initial disease, age, number of relapses and duration of glucocorticoid use.

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.

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.