Sj?gren's syndrome associated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) treated with autologous and subsequently allogeneic haematopoietic SCT (HSCT)

摘要

Sjogren's syndrome (SS) is an autoimmune disease that may infrequently be associated with occasionally severe nervous system complications, similar to the neuropsychiatric complications of systemic lupus erythematosus. This patient developed a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) refractory to i.v. high-dose immunoglobulins, considered the gold standard therapy for this condition. CIDP is an autoimmune disease consisting of a sensory-motor neurological syndrome thought to be due to the interaction of cellular and humoral autoaggressive responses. The result is demyelination, leading to axonal degeneration and long-term disability. In view of the results obtained by means of autologous HSCT in a great variety of severe autoimmune diseases, the procedure has also been utilized in severe refractory cases of CIDP. The first report was published in 2002 with initially good results, but the patient relapsed 5 years later. Other cases, including three of his own, have been reviewed by Kazmi et al.