Antisynthetase syndrome with refractory lung involvement and myositis successfully treated with double filtration plasmapheresis

Abstract

Antisynthetase syndrome (ASS) is characterized by inflammatory muscle disease, pulmonary and joint involvement, and antisynthetase autoantibodies, with anti-Jo-1 antibody being the most common. Despite the use of immunosuppressive drugs, the prognosis of lung involvement seems poor. Herein, we report a case of refractory ASS, which maintained long-term remission by double filtration plasmapheresis (DFPP) combined with immunosuppressive therapy. For a 65-year-old woman, who was diagnosed with ASS, immunosuppressive therapy was initiated and plasmapheresis (PP) was performed five times due to acute interstitial pulmonary disease and inflammatory myopathy. She remained in remission for eight months following PP. Increase in interstitial involvement was identified by lung tomography when the patient presented again with complaint of progressive increase in dyspnea and muscle pain. Although the immunosuppressive therapy was increased for the patient with elevated creatine phosphokinase (CPK) (2776 IU/mL), a rapid decrease in diffusion capacity of the lung for carbon monoxide (DLCO) was observed and the patient underwent PP. After four sessions of therapy, insufficient clinical and laboratory response was obtained (control CPK 1797 IU/mL) and because of that issue DFPP using a 2A filter was performed to the patient. There was a marked improvement in complaints of the patient, DLCO, and laboratory findings (control CPK 508 IU/mL) after three sessions of DFPP. The patient, who continued the immunosuppressive therapy after DFPP procedure, is being followed for 12 months in remission. Although our experience is limited with only one patient, DFPP seems promising as a treatment option for ASS with severe lung involvement.

Therapeutic plasma exchange in antisynthetase syndrome with severe interstitial lung disease

“…The clinical presentation and treatment outcome in our patient were similar to that of the case reported by Bozkirli et al . They managed a 65‐year‐old woman with severe ILD associated ASS that was refractory to steroids and intermittent cyclophosphamide with TPP using fresh frozen plasma as replacement fluid.…”

“…Like we did, Bozkirli et al used TPP as an emergency intervention in the acute phase to save time till the immunosuppressive became effective. We used albumin as replacement fluid because of the risks associated with the use of blood products and the fact the 2013 apheresis guideline and earlier control trials used albumin as replacement fluid in majority of the autoimmune and immune complex diseases .…”

“…However, for steroid refractory cases and those with severe or progressive disease, beneficial effects of immunosuppressive agents such as cyclophosphamide, IV immunoglobulins, cyclosporine, azathioprine, tacrolimus and rituximab have been shown . A case report has shown that therapeutic plasma exchange and double filtration plasmapheresis may possibly be used as a rescue therapy in the acute phase of lung involvement .…”

Diagnostic Utility of Auto-Antibodies in Inflammatory Muscle Diseases

“…Indeed, rituximab may be efficacious in refractory IIM [83] and presence of anti-MI-2 or anti-synthetase syndrome predict clinical improvement [84]. Removing antibodies (plasma exchanges) in the acute phase of auto-immune myopathy with MSA also showed positive results [85,86]. The immuno-modulator treatment: intra-venous immunoglobulins (IVIg) could modulate effects of pathogenic antibody by inhibiting activation of complement and/or fixation to their cognate antigens (anti-idiotypic effect) [87].…”

Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review

“…The role of plasmapheresis is still unclear. Its use has been reported in two cases of ASSD-ILD as an emergency intervention before immunosuppressive therapy became effective [129,130].…”