Granuloma-like lesion at subcutaneous immunoglobulin site in a common variable immunodeficiency patient

Immunotherapy ◽  
2019 ◽  
Vol 11 (14) ◽  
pp. 1177-1180 ◽  
Author(s):  
Ankmalika Gupta ◽  
Beverly Wang ◽  
Sudhir Gupta
Keyword(s):  
Intravenous Immunoglobulin ◽  
Common Variable Immunodeficiency ◽  
Immunoglobulin Therapy ◽  
Subcutaneous Immunoglobulin ◽  
Primary Antibody Deficiencies ◽  
First Case ◽  
Granulomatous Lesions ◽  
Cutaneous Granuloma ◽  
Common Variable ◽  
Switching Treatment

Immunoglobulin therapy is the main stay in the treatment of primary antibody deficiencies. Granulomatous lesions are common complication in patients with common variable immunodeficiency (CVID). We present the first case of cutaneous granuloma-like lesion at site of subcutaneous immunoglobulin injections in a patient with CVID. These lesions resolve overtime following switching treatment to intravenous immunoglobulin. Unlike granulomas associated with CVID, granulomatous lesion in this patient did not require any specific therapy, and resolved over a period of 4 weeks following switching subcutaneous immunoglobulin to intravenous immunoglobulin.

scholarly journals 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

2021 ◽  
Vol 12 ◽  
Author(s):  
Isaac R. Melamed ◽  
Holly Miranda ◽  
Melinda Heffron ◽  
Joseph R. Harper
Keyword(s):  
Adverse Events ◽  
Intravenous Immunoglobulin ◽  
Common Variable Immunodeficiency ◽  
Treatment Phase ◽  
Open Label ◽  
Ivig Infusion ◽  
C1 Esterase Inhibitor ◽  
Pre Treatment ◽  
Common Variable ◽  
Esterase Inhibitor

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 RegistrationClinicalTrials.gov, identifier NCT03576469.

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