Good syndrome and polymyositis.

Abstract

Good syndrome (GS)wasfirst described byDrRobert Good in 1954 in cpm [reference range, >188,800 cpm]; pokeweed mitogen: 8,311 an adult patient with thymoma and immunodeficiency.1 The immunodeficiency found in GS typically demonstrates low to absent B cells in the peripheral blood, hypogammaglobulinemia, and defects in cell-mediated immunity in patients 40 to 70 years of age.2e4 Infections associated with both Band T-cell defects are common.5 Autoimmune disorders and their complications have been associated with GS. Although dysregulation of T-cell function has been implicated, the pathogenesis remains unclear.5,6 In a systematic review by Kelesidis and Yang6 of 152 patients diagnosed as having GS, pure red blood cell aplasia and myasthenia gravis were the most prevalent comorbidities, followed by oral lichen planus, aplastic anemia, macrocytic anemia, autoimmune hemolytic anemia, monoclonal gammopathy, diabetes mellitus, polyarthropathy, and myelodysplastic syndrome. Polymyositis has never been reported in a patient with GS. Polymyositis is an inflammatory myopathy that is thought to be caused by a T-cellemediated cytotoxicity.7 The clinical presentation includes progressive muscle weakness, fatigue, dysphagia, and arthralgias. Diagnosis is based on characteristic abnormalities of serummuscle enzymes, electromyography, andmuscle biopsy after other myopathies have been ruled out specifically by clinical manifestations and diagnostic testing. Patients may be treated with corticosteroids, immunosuppressive medications, immunosuppressant, and/or immunomodulatory modalities. The patient initially developed radiographic evidence of a mediastinal mass at the age of 65 years. Further evaluation and biopsy revealed the presence of a thymoma. Three years after the resection of the thymoma, the patient developed recurrent thrush, esophageal candidiasis, and onychomycosis. The patient was hospitalized for pneumonia 4 times per year before treatment. At the age of 73 years,with no prior historyofmuscle disease, he developed proximal muscle weakness that involved, most prominently, the muscles of his neck and shoulders. Elevated muscle enzyme levels (creatine phosphokinase, aldolase, and serum glutamic oxaloacetic transaminase) suggested a myopathy. A left deltoid muscle biopsy specimen revealed inflammatory myopathy without inclusive bodies. Degeneration and regeneration of muscle fibers were noted accompanied by focal perimysial and endomysial lymphatic infiltrates. Electromyographic analysis suggested myopathic changes. Laboratory evaluation performed at 75 years of age revealed a low total absolute CD3þ cell count of 570 cells/mm3 (reference range, 625-2400 cells/mm3), an absolute CD3þ CD4þ cell count of 414 cells/mm3 (reference range, 400-1400 cells/mm3), and an absolute CD3þ CD8þ cell count of 141 cells/mm3 (reference range, 160-880 cells/mm3). The response to mitogen and antigen stimulation was markedly diminished (phytohemagglutinin: 10,870

DOI: 10.1016/j.anai.2014.03.001

Cite this paper

@article{Frith2014GoodSA, title={Good syndrome and polymyositis.}, author={John A Frith and Erin Toller-Artis and Haig Tcheurekdjian and Robert W. Hostoffer}, journal={Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology}, year={2014}, volume={112 5}, pages={478} }