Is Satoyoshi syndrome an autoimmune disease? A systematic review

Abstract

Objectives Satoyoshi syndrome (SS) is a rare multisystem disease of presumed autoimmunea aetiology. We carried out a systematic review to evaluate the available evidence to support that autoimmune hypothesis. Methods We searched for SS cases in PubMed, the Web of Knowledge and Scopus up to January 2022, using keywords “Satoyoshi syndrome” or “Komuragaeri disease”. Data on symptoms, associated autoimmune diseases, presence of autoantibodies and response to treatment were collected. Results 77 patients from 57 articles published between 1967 and 2021 were included. 59 patients were women. The mean age at diagnosis was 21.2 years. All cases had painful muscular spasms and alopecia. Frequent manifestations included: diarrhoea, malabsorption, growth retardation, amenorrhea and bone deformity. SS was associated with other autoimmune diseases: myasthenia gravis, autoimmune thyroiditis, idiopathic thrombocytopenic purpura, atopic dermatitis, bronchial and lupus erythematosus. Autoantibody determinations were performed in 39 patients, of which 27 had positive results. The most frequently detected autoantibodies were antinuclear antibodies. Other less frequently found auto-antibodies were: anti-acetylcholine receptor antibodies, anti-DNA antibodies, antithyroid antibodies, anti-GAD and anti-gliadin antibodies. Pharmacological treatment was reported in 50 patients. Most of them improved with corticosteroids, immunosuppressants and immunoglobulins, or a combination of these medications. Conclusion SS is associated with other autoimmune diseases and a variety of autoantibodies. Improvement after corticosteroid or other immunosuppressant treatment was observed in 90% of cases. These data support an autoimmune aetiology for SS. More studies including systematic determination of autoantibodies in all patients with SS will help us advance in our understanding of this disease.

Impending central retinal vein occlusion and granulomatous uveitis in a patient with Satoyoshi syndrome

“…High-dose intravenous immunoglobulin therapy and plasma exchange are administered in severe cases. [3–6]…”

“…A total of 77 cases have been reported worldwide thus far. [1][2][3][4] Satoyoshi syndrome is considered an acquired disease as no familial onset has been reported. [7] Painful muscle spasms are the most common initial symptoms.…”

“…High-dose intravenous immunoglobulin therapy and plasma exchange are administered in severe cases. [3][4][5][6] There have been case reports of Satoyoshi syndrome occurring in autoimmune and immune disorders. For example, Satoh et al [8] reported a case who was complicated with systemic lupus erythematosus, myasthenia gravis, and Satoyoshi syndrome.…”

“…[1,2] Although this disease was initially reported in 1967, only 64 cases have been reported worldwide as of 2018, and 77 cases as of 2021. [3,4] Corticosteroid therapy is effective for suppressing the muscle spasms associated with this disease to some extent; hence, autoimmune mechanisms may be associated with its pathogenesis. However, the underlying mechanisms of this disease have not yet been elucidated.…”

“…have not yet been elucidated. [2][3][4][5][6] In terms of treatment, and muscle relaxants, anticonvulsants, corticosteroids, immunosuppressants, and immunoglobulin therapy have been reported to be effective in various case reports. [3][4][5][6] Herein, we report a case of granulomatous pan-uveitis and impending central retinal vein occlusion (CRVO) co-occurring with Satoyoshi syndrome.…”