Rituximab Associated Hypogammaglobulinemia in Autoimmune Disease

ObjectiveTo evaluate the characteristics of patients with autoimmune disease with hypogammaglobulinemia following rituximab (RTX) and describe their long-term outcomes, including those who commenced immunoglobulin replacement therapy.MethodsPatients received RTX for autoimmune disease between 2003 and 2012 with immunoglobulin G (IgG) <7g/L were included in this retrospective series. Hypogammaglobulinemia was classified by nadir IgG subgroups of 5 to <7g/L (mild), 3 to <5g/L (moderate) and <3g/L (severe). Characteristics of patients were compared across subgroups and examined for factors associated with greater likelihood of long term hypogammaglobulinemia or immunoglobulin replacement.Results142 patients were included; 101 (71%) had anti-neutrophil cytoplasm antibody (ANCA) associated vasculitis (AAV), 18 (13%) systemic lupus erythematosus (SLE) and 23 (16%) other conditions. Mean follow-up was 97.2 months from first RTX. Hypogammaglobulinemia continued to be identified during long-term follow-up. Median time to IgG <5g/L was 22.5 months. Greater likelihood of moderate hypogammaglobulinemia (IgG <5g/L) and/or use of immunoglobulin replacement therapy at 60 months was observed in patients with prior cyclophosphamide exposure (odds ratio (OR) 3.60 [95% confidence interval (CI) 1.03 – 12.53], glucocorticoid use at 12 months [OR 7.48 (95% CI 1.28 – 43.55], lower nadir IgG within 12 months of RTX commencement [OR 0.68 (95% CI 0.51 – 0.90)] and female sex [OR 8.57 (95% CI 2.07 – 35.43)]. Immunoglobulin replacement was commenced in 29/142 (20%) and associated with reduction in infection rates, but not severe infection rates.ConclusionHypogammaglobulinemia continues to occur in long-term follow-up post-RTX. In patients with recurrent infections, immunoglobulin replacement reduced rates of non-severe infections.

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Long-term follow up of patients with common variable immunodeficiency treated with intravenous immunoglobulin: Reevaluation of intravenous immunoglobulin replacement therapy

Biotherapy ◽

10.1007/bf01877733 ◽

1993 ◽

Vol 7 (2) ◽

pp. 101-107 ◽

Cited By ~ 3

Author(s):

Kyosuke Mushiake ◽

Fumiaki Motoyoshi ◽

Naomi Kondo ◽

Hiroyuki Shimizu ◽

Tadao Orii

Keyword(s):

Replacement Therapy ◽

Intravenous Immunoglobulin ◽

Common Variable Immunodeficiency ◽

Immunoglobulin Replacement Therapy ◽

Long Term Follow Up ◽

Immunoglobulin Replacement ◽

Common Variable

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AB0528 Efficacy and Safety of Rituximab in the Treatment of Neuropsychiatric Systemic Lupus Erythematosus During Long-Term Follow-Up

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2015-eular.4734 ◽

2015 ◽

Vol 74 (Suppl 2) ◽

pp. 1076.3-1077 ◽

Cited By ~ 2

Author(s):

M. Tsanyan ◽

S. Soloviev ◽

E. Aleksandrova ◽

E. Nasonov

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Efficacy And Safety ◽

Systemic Lupus ◽

Neuropsychiatric Systemic Lupus Erythematosus ◽

Long Term Follow Up

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Stevens‐Johnson syndrome/toxic epidermal necrolysis‐like cutaneous lupus erythematosus. A case series with long‐term follow‐up

Journal of the European Academy of Dermatology and Venereology ◽

10.1111/jdv.17698 ◽

2021 ◽

Author(s):

Ignasi Marti‐Marti ◽

Daniel Morgado‐Carrasco ◽

Cristina Carrera ◽

Francesc Alamon‐Reig ◽

Laura Serra‐García ◽

...

Keyword(s):

Toxic Epidermal Necrolysis ◽

Lupus Erythematosus ◽

Cutaneous Lupus Erythematosus ◽

Case Series ◽

Stevens Johnson Syndrome ◽

Johnson Syndrome ◽

Long Term Follow Up ◽

Cutaneous Lupus

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Serious Infectious Events and Immunoglobulin Replacement Therapy in Patients With Autoimmune Disease Receiving Rituximab: A Retrospective Cohort Study

Clinical Infectious Diseases ◽

10.1093/cid/ciaa127 ◽

2020 ◽

Cited By ~ 1

Author(s):

Sarah Stabler ◽

Jonathan Giovannelli ◽

David Launay ◽

Angélique Cotteau-Leroy ◽

Marion Heusele ◽

...

Keyword(s):

Risk Factors ◽

Cohort Study ◽

Autoimmune Disease ◽

Replacement Therapy ◽

Systemic Vasculitis ◽

Immunoglobulin Replacement Therapy ◽

History Of ◽

Immunoglobulin Replacement ◽

Cd4 Lymphocyte ◽

Autoimmune Cytopenia

Abstract Background Rituximab (RTX) is widely administered to patients with autoimmune disease (AID). This study aimed to estimate the incidence of serious infectious events (SIEs) after RTX initiation in patients with AID. We also described the characteristics and risk factors of SIEs, and immunoglobulin replacement therapy (IgRT) strategies. Methods Patients treated between 2005 and 2016 were included in this retrospective monocentric cohort study. An RTX course was defined as the complete RTX treatment regimen received by a given patient for AID. SIEs and IgRT were right-censored at 24 months after RTX initiation. Results Two hundred twenty-one patients were included (corresponding to 276 RTX courses). Reasons for RTX initiation included connective tissue disease (38%), systemic vasculitis (36%), and autoimmune cytopenia (22%). The 1- and 2-year incidences of SIEs were 17.3 (95% confidence interval [CI], 12.0–22.5) and 11.3 (95% CI, 8.1–14.5) per 100 person-years, respectively. Forty-seven SIEs were observed, mostly comprising pneumonias (45%) and bacteremias (21%). When documented, the microorganisms were bacterial (55%) and fungal (12%). Identified risk factors of SIEs were age, history of diabetes, history of cancer, concomitant steroid treatment, and low CD4 lymphocyte count at RTX initiation. IgRT was started in 22 RTX courses (8%). Conclusions In patients with AID treated with RTX, the 1- and 2-year incidence of SIE was 17.3 and 11.3 per 100 person-years, respectively. Reports of SIE characteristics, risk factors, and IgRT strategies highlight the need for an appropriate and individualized assessment prior to and following RTX to prevent SIEs, particularly in patients with comorbidities.

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P.351Gene-replacement therapy (GRT) in spinal muscular atrophy type 1 (SMA1): long-term follow-up from the onasemnogene abeparvovec phase 1/2a clinical trial

Neuromuscular Disorders ◽

10.1016/j.nmd.2019.06.513 ◽

2019 ◽

Vol 29 ◽

pp. S184

Author(s):

J. Mendell ◽

R. Shell ◽

K. Lehman ◽

M. McColly ◽

L. Lowes ◽

...

Keyword(s):

Clinical Trial ◽

Spinal Muscular Atrophy ◽

Replacement Therapy ◽

Muscular Atrophy ◽

Phase 1 ◽

Spinal Muscular Atrophy Type ◽

Long Term Follow Up

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Kikuchi-Fujimoto disease: unusual presentation of rare disease

Clinics and Practice ◽

10.4081/cp.2016.828 ◽

2016 ◽

Vol 6 (1) ◽

Author(s):

Duy Vu ◽

Srini Reddy ◽

Lynn Day ◽

Nail Aydin ◽

Subhasis Misra

Keyword(s):

Lupus Erythematosus ◽

Unusual Presentation ◽

Chronic Infections ◽

Young Females ◽

Inappropriate Treatment ◽

Increased Risk ◽

Long Term Follow Up ◽

Autoimmune Processes

Kikuchi-Fujimoto disease (KFD) is a rare, benign disorder that typically follows a selflimiting natural course and was initially described in young females of Asian descent. Its clinical presentation may mimic lymphoproliferative disorders, connective tissue disorders, and chronic infections. This often leads to misdiagnosis and inappropriate treatment. The exact cause of this condition remains unknown although autoimmune processes and certain infectious agents have been associated with the disease. The diagnosis of KFD is made histopathologically. Treatment is supportive and long-term follow-up is recommended due to increased risk of future development of systemic lupus erythematosus. Here we are presenting a case of a patient with an unusual presentation of KFD.

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Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura

Blood ◽

10.1182/blood-2013-04-496752 ◽

2013 ◽

Vol 122 (12) ◽

pp. 2023-2029 ◽

Cited By ~ 74

Author(s):

Cassandra C. Deford ◽

Jessica A. Reese ◽

Lauren H. Schwartz ◽

Jedidiah J. Perdue ◽

Johanna A. Kremer Hovinga ◽

...

Keyword(s):

Lupus Erythematosus ◽

Complete Recovery ◽

Systemic Lupus ◽

Thrombocytopenic Purpura ◽

Risk Of Death ◽

Chronic Disorder ◽

Key Points ◽

Long Term Follow Up

Key Points After recovering from TTP, the prevalence of hypertension, depression, and systemic lupus erythematosus and risk of death are increased. TTP may be a more chronic disorder rather than a disorder of acute episodes and complete recovery.

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