Immunoglobulins in systemic sclerosis management. A large multicenter experience.

OBJECTIVE: To analyze the effectiveness and safety of intravenous immunoglobulin (IVIG) given in routine care to patients with systemic sclerosis (SSc). METHODS: A retrospective multicenter observational study was conducted in SSc patients treated with IVIG. We collected data on epidemiological parameters and clinical outcomes. Firstly, we assessed changes in organ manifestations during IVIG treatment. Secondly, we analyzed the frequency of adverse effects. The following parameters were collected from baseline to the last follow-up: the patient's weight, modified Rodnan Skin Score (mRSS), modified manual muscle strength scale (MRC), laboratory test(creatine kinase(CK), hemoglobin and protein levels), The University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0) questionnaire, pulmonary function tests, and echocardiography. RESULTS: Data were collected on 78 patients (82% females; 59% with diffuse SSc). Inflammatory idiopathic myopathy was the most frequent concomitant overlap disease (41%). The time since Raynaud's phenomenon and SSc onset were 8.8 ± 18 and 6.2 ± 6.7 years respectively. The most frequent IVIG indication was myositis (38/78), followed by gastrointestinal (27/78) and cutaneous (17/78) involvement. The median number of cycles given were 5. 54, 53 and 9 patients have been treated previously with glucocorticoids, synthetic disease-modifying antirheumatic drugs and biologic therapies respectively. After IVIG use we found significant improvements in muscular involvement (MRC ≥ 3/5 92% IVIG, p = 0.001 and CK levels from 1149 ± 2026 UI to 217 ± 224 UI, p = 0.02), mRSS (15 ± 12.4 to 13 ± 12.5, p = 0.015) and improvement in total score of UCLA GIT 2.0 (p = 0.05). None Anti-RNA polymerase III patients showed an adequate response in gastrointestinal involvement (0/7) in comparison with other antibodies (0 vs. 25, p = 0,039). Cardiorespiratory involvement remained stable. A total of 12 adverse events were reported with only one withdrawn due to serious adverse effect. CONCLUSIONS: this study suggest that IVIG may improve myositis, gastrointestinal and skin involvement in SSc patients treated in routine care and seems to have a good safety profile.

Immunoglobulin utilization in Canada: a comparative analysis of provincial guidelines and a scoping review of the literature.

BACKGROUND: Canada has high immunoglobulin (IG) product utilization, raising concerns about appropriate utilization, cost and risk of shortages. Currently, there is no national set of standardized IG guidelines, and considerable variations exist among the existing provincial guidelines. The aims of this study were: (1) to compare the existing Canadian provincial guidelines on the use of IG products to identify their consistencies and differences and (2) to examine the existing research in Canada on IG supply and utilization following the establishment of IG guidelines to understand the scope of research and pinpoint the gaps. METHODS: A comparative analysis accounted for the differences across provincial IG guidelines. We highlighted similarities and differences in recommendations for medical conditions. A scoping review of citations from MEDLINE, PubMed, Scopus and Embase databases was conducted for studies published from January 01, 2014, to April 12, 2023. RESULTS: While provincial guidelines represented a considerable overlap in the medical conditions delineated and relatively uniform dose calculations, numerous differences were observed, including in recommendation categories, provision of pediatric dosing, and divergent recommendations for identical conditions based on patient demographics. The scoping review identified 29 studies that focused on the use of IG in Canada. The themes of the studies included: IVIG utilization and audits, the switch from IVIG to SCIG, patient satisfaction with IVIG and/or SCIG, the economic impact of self-administered SCIG versus clinically administered IVIG therapy, and the efficacy and cost-effectiveness of alternative medications to IG treatment. CONCLUSION: The differences in guidelines across provinces and the factors influencing IVIG/SCIG use, patient satisfaction, and cost savings are highlighted. Future research may focus on clarifying costs and comparative effectiveness, exploring factors influencing guideline adherence, and evaluating the impact of updated guidelines on IG use and patient outcomes.

Effects of peripartum treatment on delivery outcomes in women with primary immune thrombocytopenia: a prospective cohort study / 中华围产医学杂志

Objective:To investigate the effects of peripartum administration of low-dose corticosteroids or intravenous immunoglobulin (IVIG) on delivery outcomes in pregnant patients with primary immune thrombocytopenia (ITP).Methods:This prospective cohort study involved pregnant women (≥34 gestational weeks) who were diagnosed with ITP in Peking University People's Hospital from January 2017 to December 2021. Their platelet counts were between 20×10 9/L to 50×10 9/L without bleeding and none of them had been treated with any medications. All patients were divided into medication group (prednisone or IVIG) and platelet transfusion group based on their preference. Differences in vaginal delivery rate, postpartum hemorrhage rate and platelet transfusion volume between the two groups were compared using t-test, Wilcoxon rank sum test and Chi-square test. Binary logistic regression was used to investigate the factors influencing the rates of vaginal delivery and postpartum hemorrhage. Multiple linear regression was used to analyze the factors influencing the platelet transfusion volume. Results:A total of 96 patients with ITP were recruited with 70 in the medication group and 26 in the platelet transfusion group. The vaginal delivery rate in the medication group was higher than that in the platelet transfusion group [60.0% (42/70) vs 30.8% (8/26), χ 2=6.49, P=0.013]. After adjusted by the proportion of multiparae and the gestational age at delivery, binary logistic regression showed that the increased vaginal delivery rate in patients undergoing the peripartum treatment ( OR=4.937, 95% CI: 1.511-16.136, P=0.008). The incidence of postpartum hemorrhage in the two groups was 22.9% (16/70) and 26.9% (7/26), respectively, but no significant difference was shown ( χ 2=0.17, P=0.789). The median platelet transfusion volume was lower in the medication group than in the platelet transfusion group [1 U(0-4 U) vs 1 U(1-3 U), Z=－2.18, P=0.029]. After adjustment of related factors including the platelet count at enrollment, obstetrical complications and anemia, multiple linear regression showed that the platelet transfusion volume was also lower in the medication group (95% CI:0.053-0.911, P=0.028). Ninety-six newborns were delivered without intracranial hemorrhage. The overall incidence of neonatal thrombocytopenia was 26.0% (25/96). There was no significant difference in birth weight, and incidence of neonatal asphyxia or thrombocytopenia between the two groups. Conclusion:Peripartum therapy in ITP patients may increase vaginal delivery rate and reduce platelet transfusion volume without causing more postpartum hemorrhage.

Fatal Autonomic Dysfunction Due to Guillain-Barré Syndrome After Cardiac Surgery.

Guillain-Barré syndrome, a rare peripheral neuropathy, appears to occur more often in patients who have recently undergone surgery than in the general population. However, the pathophysiologic relationship between surgery and Guillain-Barré syndrome is elusive. Few cases of Guillain-Barré syndrome after cardiac surgery have been reported. Autonomic dysfunction, a serious complication of Guillain-Barré syndrome, has not been previously reported after cardiac surgery. We describe the case of a 71-year-old woman in whom the acute motor axonal neuropathic subtype of Guillain-Barré syndrome developed after mitral valve replacement. Despite plasmapheresis and intravenous immunoglobulin therapy, she died of complications from severe autonomic dysfunction 25 days postoperatively. Recognizing the potential cardiovascular involvement of Guillain-Barré syndrome is important, because patients who undergo cardiac surgery can be vulnerable to autonomic dysfunction in the early postoperative period.

Limb ischemia due to spontaneous heparin-induced thrombocytopenia as the primary presentation of acute COVID-19 infection.

Heparin-induced thrombocytopenia (HIT) occurs with the development of IgG antibodies that bind complexes of heparin and platelet factor 4 (PF4), which activate platelets and result in a profoundly prothrombotic condition. In rare instances, this syndrome develops in the absence of proximate heparin administration, referred to as spontaneous HIT, for which less than three dozen cases have been reported. Spontaneous HIT is considered a subtype of "autoimmune HIT" (aHIT), characterized by platelet activation in the serotonin release assay (SRA) without the addition of exogenous heparin. Here, we report spontaneous HIT as the presenting feature in a patient with 2019 coronavirus disease infection (COVID-19).A 66-year-old male presented with progressive leg pain and was found to have a platelet count of 39 × 109/L and multiple lower extremity arterial thromboses requiring fasciotomy and thrombectomy. He had no recent hospitalization, heparin exposure, vaccinations, or known thrombophilia. He had a strongly positive IgG-specific enzyme-linked immunosorbent assay for heparin-PF4 antibodies, and the SRA was strongly positive both with and without the addition of heparin. He was treated successfully with bivalirudin, intravenous immunoglobulin, and apixaban.

Intravenous Immunoglobulin to Suppress Progression in a Patient With Advanced Breast Cancer.

Intravenous immunoglobulin (IVIG) is used to treat various diseases and has anticancer effects that suppress metastases in animal models of sarcoma and melanoma. However, these effects have been observed in a limited number of clinical cases. We report the case of a patient with metastatic breast cancer in which long-term IVIG treatment stopped disease progression in the absence of salvage chemotherapy. The patient was treated with IVIG for the treatment of immune thrombocytopenia. Surprisingly, the lung and brain metastases were stabilized, and the patient achieved a progression-free interval of 29 months. More cases are needed to investigate and confirm the efficacy of IVIG in solid tumors in the future.

Application of high-dose intravenous immunoglobulin in the treatment of severe dermatoses / 中华皮肤科杂志

High-dose intravenous immunoglobulin (IVIG) has emerged as an effective treatment option for some refractory and severe dermatoses with few adverse reactions. The Fab and Fc fragments of IgG can exert anti-inflammatory effect by mediating specific downstream reactions via binding to a variety of autoantigens, autoantibodies and complements. This review summarizes action mechanisms of IVIG, focuses on the progress towards its application in severe dermatoses (such as severe drug eruption, refractory dermatomyositis and autoimmune bullous diseases) and special populations, as well as its adverse reactions.

Long-term outcomes of emergency ABO-incompatible living donor liver transplantation using a modified desensitization protocol for highly sensitized patients with acute liver failure: A case report.

Although there is no established desensitization protocol for liver transplantation (LT), desensitization usually entails treatment with rituximab, plasmapheresis, splenectomy, and intravenous immunoglobulin (IVIG) infusion together with a local graft. The desensitization protocol is usually initiated 2 to 3 weeks before transplantation. Therefore, patients with acute liver failure warranting urgent LT are usually ineligible for ABO-incompatible (ABOi) LT. For these reasons, several attempts have been made to abridge the desensitization protocol and extend the indication for ABOi living donor LT (LDLT). Here we report a 40-year-old female diagnosed with chronic hepatitis B and acute-on-chronic liver failure (model for end-stage liver disease score, 31). In the absence of a suitable compatible liver donor, emergency ABOi LT was planned using a modified desensitization protocol. The preoperative isoagglutinin (IA) titer was 1 : 1,024 and the preoperative T- and B-cell cross-matches were positive. The patient received a single dose of rituximab (375 mg/m2) and IVIG (0.8 g/kg) was administered from the anhepatic phase until three days after transplantation. Although the patient developed acute cellular rejection in the early stages after LT, she has maintained a stable graft function, even after 5 years. In summary, a modified desensitization protocol consisting of rituximab and IVIG is a feasible strategy for highly sensitized patients with elevated IA titers indicated for urgent LDLT.

Recombinant Human C1 Esterase Inhibitor for the Management of Adverse Events Related to Intravenous Immunoglobulin Infusion in Patients With Common Variable Immunodeficiency or Polyneuropathy: A Pilot Open-Label Study.

It has been hypothesized that low levels of C1 esterase inhibitor (C1-INH), a key inhibitor of the complement pathway, may play a role in the occurrence of adverse events (AEs) associated with intravenous immunoglobulin (IVIG) therapy. This open-label pilot study evaluated C1-INH replacement, with recombinant human C1-INH (rhC1-INH), as a potential therapy for adults requiring IVIG and experiencing AEs. Patients received two rounds of IVIG infusion [pre-treatment phase (no rhC1-INH), 4-8 weeks] and then three rounds of one dose of intravenous rhC1-INH 50 U/kg (maximum, 4,200 U) with subsequent IVIG infusion (treatment phase, 6-12 weeks). Nineteen adults completed the study; all had an autoimmune condition linked to common variable immunodeficiency (CVID) or polyneuropathy, and 57.9% had low baseline C1-INH levels. Mean ± SD total scores improved significantly with the Headache Impact Test (from 62.8 ± 6.2 at pre-treatment to 57.7 ± 9.1 after treatment; mean Δ, -5.0; p = 0.02) and Modified Fatigue Impact Scale (from 59.3 ± 13.1 to 51.2 ± 15.4; mean Δ, -8.1; p = 0.006). Significant improvements in the Migraine Disability Assessment were observed for three of five items (p ≤ 0.002). Mean ± SD C1-INH level increased from 26.8 ± 5.9 mg/dl after the second round of IVIG (pre-treatment) to 32.1 ± 7.8 mg/dl after the third rhC1-INH treatment; functional C1-INH levels increased from 115.8 ± 34.7% to 158.3 ± 46.8%. Future research is warranted to explore the benefit of C1-INH therapy for reduction of IVIG-related AEs, as well as the role of C1-INH in patients with CVID and autoimmune disease. Clinical Trial Registration: ClinicalTrials.gov, identifier NCT03576469.

[Treatment-refractory anaemia in a 35-year-old heart transplant recipient on chronic hemodialysis]. / Therapierefraktäre Anämie bei einem 35-jährigen Dialysepatienten nach Herztransplantation.

This article presents a case of pure red cell aplasia in a 35-year-old heart transplant recipient on chronic hemodialysis. Elevated parvovirus B19 immunoglobulin M blood levels were detected along with a high viral load of 80 billion IU/ml quantified by polymerase chain reaction. Bone marrow examination revealed giant proerythroblasts confirming parvovirus B19 infection. High-dose intravenous immunoglobulin was used for treatment. Anaemia had significantly improved 4 weeks later. Parvovirus B19 infection should be excluded in organ transplant recipients with anaemia due to ineffective erythropoiesis.

[Persistent anemia after kidney transplantation in a 36-year-old male patient-an unusual cause]. / Persistierende Anämie nach Nierentransplantation bei einem 36-jährigen Patienten ­ eine ungewöhnliche Ursache.

An allogeneic kidney transplantation (match 1­1­0, cytomegalovirus, CMV, donor, D, +/recipient, R, - high risk) was performed in a 36-year-old patient. The patient was on dialysis due to a tubulointerstitial nephritis confirmed by biopsy 11 years previously. Posttransplantation there was a gradual decrease in the hemoglobin (Hb) level from 11.4 g/dl to 7.3 g/dl during the initial hospitalization period. Initially this was explained by the kidney transplantation and chronic fibrosing antral gastritis with erosions. Despite repeated transfusion of red cell concentrates, a refractory anemia persisted, which is why the patient presented several times at our clinic for further diagnosis and treatment. The presence of giant erythroblasts in the bone marrow and quantitative detection of parvovirus B19 (>900 million IU/ml DNA replications) was consistent with a virus-associated red cell aplasia. Intravenous immunoglobulin administration was established and showed long-term therapeutic success.

[Co-existence of Guillain-Barré syndrome and Behcet syndrome: A case report].

A 40-year-old male patient was referred to our department with complains of recurrent oral ulcer for more than 20 years and vulvar ulcer for more than 10 years. He presented with a 3-month history of right external ophthalmoplegia. More than 10 days ago, the patient received ganglioside infusion. And one week ago, he developed numbness and pain of his lambs, and progressive myasthenia, accompanied by right blepharoptosis and dysuria. On exam, motor strength was graded 0/5 in the lower and the upper extremities. Deep tendon reflexes were diminished in extremities. His admission medical examination: hemoglobin (HGB), white cell and platelet counts were normal. C-reactive protein (CRP) was negative. Erythrocyte sedimentation rate (ESR) 53 mm/h. Antinuclear antibody (ANA), anti-dsDNA antibody, anti-Smith antibody, anti-cardiolipin antibody and human leucocyte antigen B51 were all within normal range. The etiological tests of influenza A pathogen, influenza B pathogen, parainfluenza virus, enterovirus and parvovirus were all negative. He tested positive for serum anti-GM1 IgG. Cerebrospinal fluid had a normal white cell count, an elevated protein content. Gram staining, culture and PCR detection for varicella-zoster virus, cytomegalovirus and herpes simplex virus were all negative. Antibodies associated with autoimmune encephalitis and paraneoplastic syndrome were negative in cerebrospinal fluid. Electromyography and nerve conduction studies showed a severe axonal damage affecting motor nerves. No obvious abnormalities were observed in his magnetic resonance imaging of brain and cavernous sinus. The patient was diagnosed with Behcet syndrome complicated with acute Guillain-Barré syndrome. He received intravenous methylprednisolone, intravenous immunoglobulin (IVIg) therapy, plasma exchange and rituximab treatment. After treatment, the patient's muscle strength of limbs was restored to grade 1, blepharoptosis and pain disappeared. The nervous system involvement of Behcet syndrome is relatively rare, especially combined with Guillain-Barré syndrome, which is easy to cause misdiagnosis. The treatment of Behcet syndrome complicated with acute Guillain-Barré syndrome includes the treatment of primary disease, plasma exchange and IVIg therapy. In addition, supportive treatment is very important for such patients. The focus of treatment is to avoid respiratory insufficiency, prevent deep vein thrombosis, monitor cardiac function and hemodynamics. Pain-relieving, physical exercise and psychological support are often under-recognized. The rehabilitation treatment is very important to improve the prognosis and quality of life of patients. What we need to learn is that when the symptoms and signs of the nervous system are difficult to be explained by neuro-Behcet syndrome alone, we should be alert to the possibility of other nervous system diseases.

Scleromyxedema with multiple systemic involvement: Successful treatment with intravenous immunoglobulin.

Scleromyxedema is a rare connective tissue disorder characterized by a generalized lichenoid eruption and sclerodermoid induration with histologic features of dermal mucin deposition. A 44-year-old man presented with a 3-year history of generalized progressive skin thickening and sclerosis. He had diffuse skin-colored to erythematous firm papules coalescing into indurated plaques over his whole body. He had been diagnosed with scleromyxedema from a skin biopsy with monoclonal gammopathy of undetermined significance (MGUS) at another tertiary hospital 3 years earlier. He had been treated with systemic corticosteroids and methotrexate, but his systemic symptoms (dyspnea, dysphagia, skin swelling, and induration) had worsened over the past year, so he visited our clinic seeking further evaluation and management. The patient received high-dose intravenous immunoglobulin (IVIG) therapy once a month in combination with systemic corticosteroids. After three courses of IVIG, his cutaneous symptoms and dyspnea had improved dramatically. Herein we report a case of scleromyxedema with systemic involvement with significant improvement following IVIG therapy.

Successful ABO-incompatible living donor liver transplantation using splenectomy and intravenous immunoglobulin in high isoagglutinin titer patients.

The role of the isoagglutinin (IA) titer in liver transplantation (LT) is still not well defined, but the general belief is that a higher titer may result in a higher risk of rejection in ABO-incompatible living donor LT. To reduce the IA titer by 1:16 or lower, plasmapheresis is usually performed before transplantation. However, there is no established protocol for patients for whom plasmapheresis has failed before reaching the target IA titers. Here, we report the cases of three patients who show high baseline IA titers and have failed plasmapheresis: no-response to plasmapheresis, allergic reaction associated with plasmapheresis, and anaphylactic reaction to platelet transfusion. For various reasons, after several plasmapheresis procedures, IA titers were not effectively reduced. In these patients, splenectomy and intravenous immunoglobulin (0.8 g/kg, from the anhepatic phase to 2 days after transplantation) were carried. The protocol biopsy on postoperative day 7 showed no histologic evidence of meaningful acute rejection. The main aim of this work is to demonstrate that we can apply this protocol to patients who have high baseline IA titers and have failed plasmapheresis. Furthermore, this report is enhanced to promote to the transplant community this approach with this type of recipient.

Outcomes Associated With Standardized Ideal Body Weight Dosing of Intravenous Immune Globulin in Hospitalized Patients: A Multicenter Study.

Background: There are limited outcomes data for ideal body weight (IBW)-based dosing of intravenous immune globulin (human, IVIG) in hospitalized patients. Objective: To investigate clinical outcomes associated with a standardized change from total body weight to IBW-based dosing of IVIG. Methods: This was a retrospective, multicenter, pre-post sequential period analysis. Data from pre-implementation and post-implementation of an IBW-based dosing strategy for IVIG were collected in 2-year periods (October 1, 2012, to August 31, 2014, and October 2, 2014, to October 1, 2016, respectively). The primary outcome was incidence of 30-day hospital readmission. Length of stay (LOS) was analyzed as a secondary outcome. Results: For the 2 study periods, 297 patients were included for analysis. Both groups had similar demographics, IVIG indications, and body weight measurements, but the post-implementation period had a lower median grams per dose as compared with the pre-implementation period (40 vs 30 g, P ≤ 0.01). 30-Day hospital readmission rates were not significantly different (4% vs 9%, P = 0.07). In-hospital all-cause mortality was also not statistically significant (7.7% vs 3.4%, P = 0.11). The 2 study groups had a similar median hospital LOS (8 vs 7.6 days, P = 0.27). Conclusion and Relevance: The implementation of a standardized IBW IVIG dosing strategy was not associated with a statistically significant increase in 30-day hospital readmission or LOS but was associated with significantly fewer grams per dose given. Application of these data may aid in decreasing institutional drug spend without affecting patient outcomes. However, the study was underpowered, and further investigation is necessary to validate these findings.

Co-existence of Guillain-Barré syndrome and Behcet syndrome: A case report / 北京大学学报(医学版)

A 40-year-old male patient was referred to our department with complains of recurrent oral ulcer for more than 20 years and vulvar ulcer for more than 10 years. He presented with a 3-month history of right external ophthalmoplegia. More than 10 days ago, the patient received ganglioside infusion. And one week ago, he developed numbness and pain of his lambs, and progressive myasthenia, accompanied by right blepharoptosis and dysuria. On exam, motor strength was graded 0/5 in the lower and the upper extremities. Deep tendon reflexes were diminished in extremities. His admission medical examination: hemoglobin (HGB), white cell and platelet counts were normal. C-reactive protein (CRP) was negative. Erythrocyte sedimentation rate (ESR) 53 mm/h. Antinuclear antibody (ANA), anti-dsDNA antibody, anti-Smith antibody, anti-cardiolipin antibody and human leucocyte antigen B51 were all within normal range. The etiological tests of influenza A pathogen, influenza B pathogen, parainfluenza virus, enterovirus and parvovirus were all negative. He tested positive for serum anti-GM1 IgG. Cerebrospinal fluid had a normal white cell count, an elevated protein content. Gram staining, culture and PCR detection for varicella-zoster virus, cytomegalovirus and herpes simplex virus were all negative. Antibodies associated with autoimmune encephalitis and paraneoplastic syndrome were negative in cerebrospinal fluid. Electromyography and nerve conduction studies showed a severe axonal damage affecting motor nerves. No obvious abnormalities were observed in his magnetic resonance imaging of brain and cavernous sinus. The patient was diagnosed with Behcet syndrome complicated with acute Guillain-Barré syndrome. He received intravenous methylprednisolone, intravenous immunoglobulin (IVIg) therapy, plasma exchange and rituximab treatment. After treatment, the patient's muscle strength of limbs was restored to grade 1, blepharoptosis and pain disappeared. The nervous system involvement of Behcet syndrome is relatively rare, especially combined with Guillain-Barré syndrome, which is easy to cause misdiagnosis. The treatment of Behcet syndrome complicated with acute Guillain-Barré syndrome includes the treatment of primary disease, plasma exchange and IVIg therapy. In addition, supportive treatment is very important for such patients. The focus of treatment is to avoid respiratory insufficiency, prevent deep vein thrombosis, monitor cardiac function and hemodynamics. Pain-relieving, physical exercise and psychological support are often under-recognized. The rehabilitation treatment is very important to improve the prognosis and quality of life of patients. What we need to learn is that when the symptoms and signs of the nervous system are difficult to be explained by neuro-Behcet syndrome alone, we should be alert to the possibility of other nervous system diseases.

Transfusion-related acute lung injury associated to intravenous immunoglobulin infusion in a pediatric patient / Lesão pulmonar aguda relacionada à transfusão associada com infusão de imunoglobulina intravenosa em paciente pediátrico

ABSTRACT Case report of a patient with an immunodeficiency who demands regular replacement of intravenous immunoglobulin. She presented an episode of transfusion-related acute lung injury shortly after using an immunoglobulin product different than the one she usually received. The patient evolved with respiratory changes (hypoxia, dyspnea, change in pulmonary auscultation) minutes after the end of the infusion, and received non-invasive respiratory support. She was discharged after 36 hours with good outcome. The patient achieved full recovery, showing no further reactions in subsequent immunoglobulin infusions (no longer receiving the product that was used when she had the episode of transfusion-related acute lung injury). Although rare, this reaction is potentially serious and has no specific treatment other than supportive therapy. The literature is scarce regarding the risk of recurrence. The decision on whether to proceed with immunoglobulin therapy after this adverse effect should be analyzed individually, assessing the possible risks and benefits for the patient.

RESUMO Relato de caso de paciente com imunodeficiência que necessitava de reposição regular de imunoglobulina endovenosa. Ela apresentou um episódio de lesão pulmonar aguda relacionada à transfusão após uso de produto de imunoglobulina diferente daquele que recebia habitualmente. Evoluiu com alterações respiratórias (hipóxia, dispneia e alteração de ausculta pulmonar) minutos após o fim da infusão, necessitando de suporte respiratório não invasivo. A paciente recebeu alta hospitalar após 36 horas, com boa evolução. Obteve recuperação total dos sintomas, sem mais reações nas infusões subsequentes de imunoglobulina (sendo optado por não mais prescrever o produto que foi usado quando ocorreu o episódio de lesão pulmonar aguda relacionada à transfusão). Apesar de rara, essa reação é potencialmente grave, não possui tratamento específico além de terapia de suporte, e há pouca informação na literatura sobre o risco de recorrência. A decisão sobre o seguimento da terapia com imunoglobulina após esse efeito adverso deve ser analisada individualmente, avaliando os possíveis riscos e benefícios para o paciente.

Passive Transfer of Hepatitis B Antibodies through Intravenous Immunoglobulin in a Neonate.

Passive transfer of antibodies secondary to intravenous immunoglobulin infusion is a rare but important side effect that can lead to the wrong diagnosis and therapeutic decisions. It has never been reported in a newborn. A male newborn, vaccinated against hepatitis B and diagnosed with dilated cardiomyopathy, presented positive hepatitis B core antibodies at 12 days of life. Exclusion of hepatitis B infection was mandatory as it would be a contraindication to heart transplant. Passive transfer of antibodies was confirmed at 44 days of age, after seroreversion of hepatitis B core antibodies. Passive transfer of antibodies after intravenous immunoglobulin infusion can lead to a misleading diagnosis if not recognized. In our patient it could have been especially harmful had it prevented heart transplant. Screening for hepatitis B should be performed at least 1 month after intravenous immunoglobulin infusion.

A transferência passiva de anticorpos secundária à infusão de imunoglobulina endovenosa é um efeito secundário raro, mas importante, que pode levar a um diagnóstico e decisões terapêuticas erradas. Nunca foi descrito num recém-nascido. Um recém-nascido do sexo masculino, vacinado contra a hepatite B e diagnosticado com miocardiopatia dilatada, apresentou anticorpos anti-core do vírus da hepatite B aos 12 dias de vida. A exclusão da infecção por hepatite B foi obrigatória, pois seria uma contra-indicação ao transplante cardíaco. A transferência de anticorpos através de imunoglobulina endovenosa foi confirmada aos 44 dias de idade, após sero-reversão dos níveis de anticorpos anti-core do vírus da hepatite B. A transferência passiva de anticorpos após a infusão de imunoglobulina endovenosa pode levar a um diagnóstico errado se não for reconhecida. Neste doente poderia ter sido especialmente prejudicial caso tivesse impedido o transplante de coração. O rastreio para hepatite B deve ser realizado pelo menos um mês após a infusão.