Clinico-Epidemiological Profile and Outcome of Children with IgA Vasculitis in Aseer Region, Southwestern Saudi Arabia

Introduction: Immunoglobulin A (IgA) vasculitis is one of the most common forms of primary vasculitis in children; it typically has a benign course but can be aggressive and require intervention. Aim of the work: The aim of this retrospective study was to evaluate the epidemiological and clinical profile and treatment modalities used for children with IgA vasculitis in the southwestern region of Saudi Arabia. Material and Methods: We reviewed the medical records of 89 children admitted to Abha Maternity and Children Hospital in the southwestern region of Saudi Arabia from January 2016 to December 2020 with a confirmed diagnosis of IgA vasculitis according to the European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Pediatric Rheumatology European Society criteria. Results: Eighty-nine children had a confirmed diagnosis of IgA vasculitis, with 50 boys (56.2%) and 39 girls (43.8%; male-to-female ratio of 1.28:1) and a mean age at diagnosis of 5.87 ± 2.81 years. The mean hospital stay duration was 5.66 ± 4.72 days. Infections preceded 29.2% of the cases, with upper respiratory tract infections comprising 24.7%. Approximately 31.5% of the cases were diagnosed in summer, followed by autumn in 28% of the cases. Rash was present in 100%, arthritis in 72.2%, gastrointestinal tract involvement in 60.7%, and renal involvement in 23.5% of cases. Thrombocytosis and leukocytosis were found in 35% and 46% of all cases, and 52.3% and 47.6.25% of cases with renal involvement, respectively (OR = 2.035, 95% CI: 0.75–5.52 and OR = 1.393, 95% CI: 0.522–1.716, respectively). Approximately 26% of cases experienced relapses. Treatment was conservative in 23.6%, oral prednisolone in 23.6%, and pulse steroid in 45% of cases. Abdominal pain with lower gastrointestinal tract bleeding was the primary indication for initiating pulse steroid treatment. Conclusions: There were similarities and differences in the epidemiology and frequency of clinical manifestations of patients with IgA vasculitis compared to previous studies. Children presenting with such epidemiological and clinical profile need to be closely monitored and long-term follow-up is recommended to improve the outcomes.

A case of Hemophagocytic lymphohistiocytosis induced by COVID-19, and review of all cases reported in the literature

Novel coronavirus infections 2019 (COVID-19) associated hyperinflammatory syndromes are well-defined clinical conditions and have a potential risk for severe infection. Hemophagocytic lymphohistiocytosis (HLH), a rare type of acute progressive hyperinflammatory syndrome, has been reported in a limited number of COVID-19 cases. In this article, we aimed to present a patient with HLH secondary to COVID-19 diagnosed by bone marrow biopsy, and to summarize and review HLH cases associated with COVID-19 in the literature. A 47-year-old male patient presented with complaints of fever, cough, abdominal discomfort, and nausea-vomiting. He had recovered from COVID-19 a month ago and was readmitted to the hospital due to the re-appearance of clinical symptoms after a two-week interval. The patient was diagnosed with HLH secondary to COVID-19 on sixth day of admission and fully recovered with systemic pulse steroid, intravenous immunoglobulin, and plasma exchange therapy. Analysis of literature searches revealed that 22 cases were definitely diagnosed with COVID-19-associated HLH, 16 of them were male. They had been treated with different anti-cytokine drugs, of which nine had died. The increasing number of HLH cases, which have high mortality rates, shows the importance of hyperinflammatory syndromes in COVID-19 patients. Some patients may experience hemophagocytosis in the late period of COVID-19, even while in recovery. Increased awareness and early treatment for HLH triggered by COVID-19 can be a life-saving effort for reducing mortality in severe COVID-19 cases.

Oral mini-pulse steroid therapy in severe chronic urticaria

Background: Treatment of chronic urticaria can be difficult at times. The present study aimed to evaluate the efficacy of oral mini-pulse (OMP) therapy with methylprednisolone in management of severe chronic urticaria (CU). Methods and material: 100 patients with severe chronic urticaria, not controlled with maximum dose of a second generation antihistamine, were enrolled in the study after an informed written consent. All patients were treated with methylprednisolone 16 mg tablet on two consecutive days of a week for 2 months along with levocetirizine 5 mg tablet once daily. All patients were reviewed at 0, 2, 4, and 8 weeks with urticaria activity score (UAS). Results: The study comprised of 100 patients (33 males and 67 females) with severe chronic urticaria. 29 patients (29%) had raised TSH levels while Autologous Serum Skin Test was positive in 37 patients. Mean UAS in patients treated with OMP was 5.76 at baseline which reduced to 0.6 at the end of treatment period. Conclusion: Mean UAS showed a significant decline following OMP therapy with methylprednisolone. Most of the patients maintained the benefits of therapy at the end of follow up period of 4 months.

Sudden cardiac arrest after late permanent pacemaker implantation in a heart transplant patient

Bradycardia during the early period following heart transplantation frequently occurs with an incidence of 14 to 44% and it is usually self-limited. The incidence of late bradycardia (from 30 days to more than 5 or 6 months after transplantation) has been reported to be 1.5%. A 33-year-old male patient with a history of orthotopic heart transplantation in 2013 presented with complaints of dizziness and near syncope. A DDDR permanent pacemaker was implanted for sinus pauses exceeding 3 seconds recorded on Holter examination. Shortly after the procedure, he developed sudden cardiovascular collapse. Cardiopulmonary resuscitation was performed and a pulse steroid treatment (2 grams of methylprednisolone) was given. After 2 days, the patient was extubated. While making preparations for re-transplantation, cardiopulmonary arrest developed again and he died. Sinus pause may be a clue for rejection and is an important finding in predicting clinical course.

Efficacy of plasma exchange for antineutrophil cytoplasmic antibody-associated systemic vasculitis: a systematic review and meta-analysis

Objective To assess through systematic review and meta-analysis whether plasma exchange (PE) is associated with prognosis in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. Methods A systematic search of PubMed, MEDLINE, Embase, and CENTRAL databases from inception to 17 June 2020 was conducted. Ongoing or unpublished trials were also searched in ClinicalTrials.gov and the World Health Organization trials portal. Randomised controlled trials (RCTs) comparing PE vs. non-PE in AAV patients (microscopic polyangiitis [MPA], granulomatosis with polyangiitis [GPA], or eosinophilic granulomatosis with polyangiitis [EGPA]) were included. The combined risk ratio (RR) was calculated by the random-effects model using the Mantel-Haenszel method. Heterogeneity was measured using the I2 statistic. Primary outcomes were mortality, clinical remission (CR), and adverse events (AEs). Results Four RCTs comparing PE vs. no PE (N = 827) and 1 RCT comparing PE vs. pulse steroid treatment (N = 137) were included. All participants were MPA or GPA patients (no EGPA patients). PE was not associated with main primary outcomes compared with no PE (mortality RR 0.93 [95% confidence interval {CI} 0.70–1.24], I2 = 0%; CR RR 1.02 [95% CI 0.91–1.15], I2 = 0%; and AE RR 1.10 [95% CI 0.73–1.68], I2 = 37%) or pulse steroid (mortality RR 0.99 [95% CI 0.71–1.37]; CR [the Birmingham Vasculitis Activity score] mean difference − 0.53 [95% CI − 1.40–0.34]; and AE RR 1.05 [95% CI 0.74–1.48]). Focusing on the early treatment phases, PE was associated with a reduction in end-stage renal disease incidence compared with both no PE (PE 1/43 vs. no PE 10/41; RR 0.14 [0.03–0.77] at 3 months) and pulse steroid (PE 11/70 vs. pulse steroid 23/67; RR 0.46 [0.24–0.86] at 3 months). Conclusion We carried out a systematic review and meta-analysis targeting all AAV patients, including MPA, GPA, and EGPA. In AAV patients, performing PE was not associated with the risk of mortality, CR, and AE. No RCT exists evaluating the efficacy of PE for EGPA; hence, this is required in the future. The results may affect the development of guidelines for AAV and may indicate the direction of future clinical research on AAV. Trial registration UMIN R000045239, PROSPERO CRD42020182566.

Severe Acute Hemolytic Transfusion Reaction Treated with Ruxolitinib and Plasma Exchange

<b><i>Introduction:</i></b> Acute hemolytic transfusion reaction is a rare but extremely mortal condition. Even small quantities of ABO-incompatible erythrocytes, as much as 50 mL, can lead to fatality. Since there is no successful standard therapy, preventive measures are very important. In this case report, we presented a 29-year-old woman who was transfused with 2 units of AB Rh-positive instead of 0 Rh-positive red blood cells following a cesarean section. As far as we know, this is the first patient in the literature for whom ruxolitinib was used as a part of therapy. <b><i>Case Report:</i></b> The patient was referred to our center 22 h after the ABO-mismatched transfusion. On admission, she had severe hemolysis, acute renal failure, and disseminated intravascular coagulation. Massive plasma exchange, hemodialysis, and pulse steroid therapy were commenced. The patient was refractory to first-line therapies. She was intubated on day 2 due to hypoxia, respiratory failure and changes in consciousness. Ruxolitinib, 2 × 10 mg/day, was started on day 3. The patient’s clinical status improved on day 6. Ruxolitinib was withdrawn on day 15, and the patient was discharged without any complications or sequels on day 26. <b><i>Conclusion:</i></b> Ruxolitinib may be life-saving in patients with ABO-incompatible transfusion reaction which follows a severe and catastrophic course.