Subcutaneous immunoglobulins for the treatment of a patient with antisynthetase syndrome and secondary chronic immunodeficiency after anti-CD20 treatment: a case report

Abstract

BackgroundAntisynthetase syndrome is a rare and debilitating multiorgan disease characterized by inflammatory myopathy, interstitial lung disease, cutaneous involvement, and frequent chronic inflammation of the joints. Standard treatments include corticosteroids and immunosuppressants. In some cases, treatment resistance may develop. Administration of immunoglobulins intravenously is recommended in patients with drug-resistant antisynthetase syndrome.Case presentationHere, we describe the case of a 56-year-old woman of Algerian origin. She is the first case of a patient with multidrug-resistant antisynthetase syndrome featuring pulmonary involvement and arthropathy, and chronic secondary immune deficiency with recurrent infections, after anti-CD20 treatment, in which her primary antisynthetase syndrome-related symptoms and secondary immune deficiency were treated successfully with subcutaneous administration of immunoglobulin. The administration of immunoglobulin subcutaneously was introduced at a dose of 2 g/kg per month and was well tolerated. Clinical improvement was observed within 3 months of initiation of subcutaneous administration of immunoglobulin. After 22 months of treatment, she showed a significant improvement in terms of muscle strength, pulmonary involvement, arthralgia, and immunodeficiency. Her serum creatine phosphokinase and C-reactive protein levels remained normal. Finally, she was compliant and entirely satisfied with the treatment.ConclusionsTaken together, these observations suggest that administration of immunoglobulin subcutaneously may be a useful therapeutic approach to tackle steroid-refractory antisynthetase syndrome while ensuring minimal side effects and improved treatment compliance. This treatment also allowed, in our case, for the regression of the chronic immunodeficiency secondary to rituximab treatment.

Pulmonary Manifestations of Predominantly Antibody Deficiencies

“…This increased susceptibility might be due to suppressive effect of anti-CD20 antibodies on type 2 helper T (Th2) responses, e.g., production of cytokines interleukin (IL)-4, IL-5, and IL-13 [202]. Moreover, there is a report of antisynthetase syndrome after treatment with rituximab, a CD20 monoclonal antibody [204]. The patient was presented with recurrent respiratory infections, arthropathy, and interstitial pneumonitis.…”

Intravenous Immunoglobulin Therapy for Maintenance Therapy of Interstitial Lung Disease Due to Antisynthetase Syndrome

“…The use of intravenous immunoglobulins in treating antisynthetase syndrome is limited to case reports, showcasing patients in whom it was used either acutely in the hospital [1], or when the clinician's hand was forced due to side-effects or infection. There is a growing body of evidence proving its utility in improving myositis, dermatological and neurological complications associated with DM/PM, but it is yet unproven for treatment of the deadliest complication-ILD [2,3].…”