Diagnosis and Treatment > Investigation > C3 complement

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Danielle R Bullock Division of Rheumatology, Department of Pediatrics

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Bradley S Miller Division of Endocrinology, Department of Pediatrics

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H Brent Clark Division of Neuropathology, Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, USA

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Patricia M Hobday Division of Rheumatology, Department of Pediatrics

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Summary

IgG4-related hypophysitis is an important diagnostic consideration in patients with a pituitary mass or pituitary dysfunction and can initially present with headaches, visual field deficits and/or endocrine dysfunction. Isolated IgG4-related pituitary disease is rare, with most cases of IgG4-related disease involving additional organ systems. We report the case of a teenage female patient with isolated IgG4-related hypophysitis, diagnosed after initially presenting with headaches. Our patient had no presenting endocrinologic abnormalities. She was treated with surgical resection, prednisolone and rituximab with no further progression of disease and sustained normal endocrine function. This case, the youngest described patient with isolated IgG4-related hypophysitis and uniquely lacking endocrinologic abnormalities, adds to the limited reports of isolated pituitary disease. The use of rituximab for isolated pituitary disease has never been described. While IgG4-related hypophysitis has been increasingly recognized, substantial evidence concerning the appropriate treatment and follow-up of these patients is largely lacking.

Learning points:

  • IgG4-related hypophysitis most often occurs in the setting of additional organ involvement but can be an isolated finding. This diagnosis should therefore be considered in a patient presenting with pituitary abnormalities.

  • Most patients with IgG4-related hypophysitis will have abnormal pituitary function, but normal functioning does not exclude this diagnosis.

  • Corticosteroids have been the mainstay of therapy for IgG4-related disease, with other immunosuppressive regimens being reserved for refractory cases. Further research is needed to understand the effectiveness of corticosteroid-sparing regimens and whether there is utility in using these agents as first-line therapies.

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Hiroto Minamino The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan
Department of Diabetes and Endocrinology, Wakayama Red Cross Hospital, Wakayama, Japan

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Hidefumi Inaba The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

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Hiroyuki Ariyasu The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

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Hiroto Furuta The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

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Masahiro Nishi The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

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Takashi Yoshimasu Department of Dermatology, Wakayama Medical University, Wakayama, Japan

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Akinori Nishikawa Department of Hematology, Wakayama Medical University, Wakayama, Japan

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Masanori Nakanishi Department of Respiratory Medicine & Medical Oncology, Wakayama Medical University, Wakayama, Japan

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Shigeki Tsuchihashi Department of Otolaryngology, Wakayama Medical University, Wakayama, Japan

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Fumiyoshi Kojima Department of Human Pathology, Wakayama Medical University, Wakayama, Japan

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Shin-ichi Murata Department of Human Pathology, Wakayama Medical University, Wakayama, Japan

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Gen Inoue Department of Diabetes and Endocrinology, Wakayama Red Cross Hospital, Wakayama, Japan

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Takashi Akamizu The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

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Summary

A 73-year-old man with Hashimoto's thyroiditis (HT) suffered from purpura on the lower legs. He was diagnosed with IgG4-related disease (IgG4-RD) with serum IgG4 elevation and dacryo-sialadenitis confirmed histologically. Serum Th2 and Treg cytokines, interleukin 7 (IL7), IL8 and Th2 chemokine levels were elevated, while skewed Th1 balance was seen in fluorescence-activated cell sorting (FACS). Therefore, preferential Th1 balance in HT appeared to be followed by IgG4-RD characterized with Th2 and Treg polarization. The commencement of steroid therapy dramatically exacerbated clinical manifestations including IgG4-RD-associated HT. The measurement of cytokine and chemokine levels as well as FACS analysis in the development of IgG4-RD seemed to be beneficial. In conclusion, an innovative association of HT, IgG4-RD and vasculitis was observed. This report also offers novel diagnostic and therapeutic approaches for IgG4-RD.

Learning points

  • Recently, a subtype of HT has been considered to be a thyroid manifestation of IgG4-RD, although the etiology of IgG4-RD is not established yet.

  • Immunologically a close association between HT and vasculitis was reported.

  • Leukocytoclastic vasculitis is a rare skin presentation of IgG4-RD.

  • In the current case, during the course of HT, IgG4-RD and leukocytoclastic vasculitis occurred; thus, innate immunity and acquired immunity seem to be involved in the development of IgG4-RD.

  • The measurement of cytokine and chemokines appeared to be beneficial in the development of IgG4-RD.

  • Remarkably, effectiveness of steroid therapy for HT suggested presence of IgG4-RD-associated HT. Therefore, this report highlights the pathogenesis of IgG4-RD and proposes novel therapeutic mechanisms. Clinicians should pay attention to the development of IgG4-RD and vasculitis during long course of HT.

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Jingjing Jiang Department of Endocrinology and Metabolism, Zhongshan Hospital, Fudan University, Shanghai, 200032, People's Republic of China

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Mei Zhang Department of Endocrinology, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing, 210029, People's Republic of China

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Ronghua He Department of Endocrinology, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing, 210029, People's Republic of China

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Meiping Shen Department of General Surgery, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, People's Republic of China

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Wei Liu Department of Nuclear Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, People's Republic of China

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Summary

Functional parathyroid cysts are a rare cause of primary hyperparathyroidism and are often mistaken for thyroid cysts. Systemic lupus erythematosus (SLE) is also a very rare cause of hypercalcemia. We report the case of a 62-year-old woman, who was diagnosed with SLE 30 years ago, presenting with clinical and biochemical features of primary hyperparathyroidism. Laboratory investigation revealed increased serum calcium and parathyroid hormone (PTH) levels; neck ultrasonography (USG) revealed 40×34×26 mm cystic mass in the left lobe of thyroid gland. PTH level in the cysts was >2500 pg/ml, determined by USG-guided fine-needle aspiration (FNA). In this case, no evidence for potential pathogenic association between parathyroid cyst and SLE was uncovered. However, the recognition of this association is very important because the therapeutical strategy is completely different. Operative management is usually straightforward and alleviates symptoms and any biochemical abnormalities caused by the cyst.

Learning points

  • Functional parathyroid cysts are the rare cause of primary hyperparathyroidism and are often mistaken for thyroid cysts.

  • SLE is also a very rare cause of hypercalcemia.

  • Ultrasound-guided FNA of cystic fluid with assay for PTH level is an accurate method of differentiating parathyroid cyst from thyroid cyst.

  • Appropriate management of functional parathyroid cysts is surgical excision.

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