We report the results of nine patients with inclusion body myositis treated with intravenous immunoglobulin in an open-label uncontrolled study. None of our patients improved on objective manual muscle testing or functional disability scores. One patient developed mild neutropenia, complicating the intravenous immunoglobulin treatment. Our results do not exclude the possibility that intravenous immunoglobulin could be beneficial in some patients by slowing the rate of deterioration or perhaps stabilizing the disease. However, given the lack of objective improvement and high cost of treatment, we would not recommend intravenous immunoglobulin in the treatment of inclusion body myositis unless a blinded, controlled trial demonstrates clear benefit.
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http://dx.doi.org/10.1212/wnl.44.8.1516 | DOI Listing |
Front Immunol
January 2025
Department of Intensive Care Unit, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China.
Background: For severe systemic rheumatic diseases (SRDs), therapeutic plasma exchange (TPE) may be applied as a rescue therapy; it usually combined with intravenous immunoglobulin (IVIG) or intravenous methylprednisolone pulse (IVMP) in severe SRDs. However, the necessity of this combination treatment strategy in SRDs remains uncertain.
Objective: This retrospective study aimed to evaluate the effectiveness of TPE alone versus TPE combined with IVIG/IVMP in treating severe SRDs.
Neurol Sci
January 2025
Department of Infectious Diseases, Shenzhen Nanshan People's Hospital, Shenzhen University Medical School, Shenzhen, 518052, China.
Background: Glial fibrillary acidic protein (GFAP) astrocytosis is a rare autoimmune encephalitis discovered in the last decade. The diagnosis depends on clinical symptoms, imaging, and antibody testing. However, most cases require several months or even longer to make a definite diagnosis.
View Article and Find Full Text PDFDermatologie (Heidelb)
January 2025
Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Leipzig, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland.
Painful ulcerations developed in a 33-year-old woman with anti-NXP-2-positive dermatomyositis in the facial and trunk areas and a 67-year-old woman with TIF1-gamma-positive dermatomyositis on the hands, while undergoing systemic therapy with azathioprine or low-dose methylprednisolone and cyclic administration of intravenous immunoglobulins (IVIG), respectively. In the laboratory workup, the anti-MDA‑5 antibody status remained negative and the creatine kinase (CK) normal in both patients, while histopathological examinations were nonspecific. Intensive topical class 4 corticosteroid therapy and continuation of the immunosuppressive or immunomodulating therapy led to healing of the ulcerative skin lesions.
View Article and Find Full Text PDFMod Rheumatol
January 2025
Department of Pediatrics, Japanese Red Cross Aichi Medical Center, Nagoya Daini Hospital, Nagoya, Aichi, Japan.
Objectives: The early administration of immunoglobulin in Kawasaki disease occasionally results in treatment failure. However, whether this is because severe cases are diagnosed and treated early or due to other factors remains unclear. In this study, we examined the timing of initial immunoglobulin administration and immunoglobulin resistance in cases classified by severity of illness.
View Article and Find Full Text PDFLupus
January 2025
Rheumatology Department, Centro Hospitalar São João, Porto, Portugal.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, autoimmune peripheral neuropathy, rarely reported as being associated with systemic lupus erythematous (SLE). We report the case of 53-year-old women with a long history of SLE, diagnosed with CIDP during a lupus flare beginning with progressive muscle weakness of lower and upper limbs, without affection of the central nervous system. She received treatment with steroids, intravenous immunoglobulins and anifrolumab and showed clinical and immunological improvement.
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