IgG4-related sclerosing mesenteritis causing bowel obstruction: a case report
- Atsushi Abe†1, 2Email authorView ORCID ID profile,
- Tatsuya Manabe†1,
- Nobuyoshi Takizawa2,
- Takashi Ueki3,
- Daisuke Yamada4,
- Kinuko Nagayoshi1,
- Yoshihiko Sadakari1,
- Hayato Fujita1,
- Shuntaro Nagai1,
- Hidetaka Yamamoto2,
- Yoshinao Oda2 and
- Masafumi Nakamura1
© The Author(s). 2016
Received: 11 August 2016
Accepted: 25 October 2016
Published: 30 October 2016
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. Recently, SM was reported to be closely related to IgG4-related disease (IgG4-RD). This report describes a patient with SM associated with IgG4-RD. A 77-year-old woman with a history of surgery for ectopic pregnancy and wound dehiscence presented with intestinal obstruction. Abdominal enhanced computed tomography (CT) revealed an enhanced, radially shaped, oval mass, 3 cm in diameter, with an unclear rim in the mesentery of the distal ileum, which may have involved the distal ileum. To remove the cause of bowel obstruction, the SM was resected completely and the ileum was resected partially. Histologic examination showed that the mass was composed of spindle cells arranged in a fascicular or storiform pattern; moreover, fibrous stroma was observed, with dense lymphoplasmacytic infiltration and lymphoid follicles. Immunohistochemically, numerous IgG4-positive plasma cells were observed, at a density of 253 per high-powered field, and the IgG4/IgG ratio was about 50 %. Elastica van Gieson (EVG) staining also showed obstructive phlebitis. These findings indicated IgG4-related SM. Although the accurate diagnosis of SM remains difficult without histological analysis, IgG4-RD should be included in the differential diagnosis of unknown mesenteric tumors. Identification of IgG4-RD may prevent unnecessary surgery because corticosteroids may be effective in these patients.
KeywordsIgG4-related SM Sclerosing mesenteritis Intestinal obstruction
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease of unknown etiology that primarily involves the small-bowel mesentery, most frequently observed in middle-aged and older men [1–6]. SM, also called mesenteric fibrosis, mesenteric lipodystrophy, and retractile mesenteritis, is histologically characterized by varying degrees of fibrosis, chronic inflammation, and fat necrosis . On imaging, SM appears as a well- or ill-defined mass in the mesentery, which may be clinically misdiagnosed as a malignant neoplasm [7–9].
SM was recently reported to be closely related to IgG4-related disease (IgG4-RD) [2, 10–13], a systemic syndrome characterized by masses in various organs infiltrated by IgG4-positive plasma cells and high serum IgG4 concentrations [14, 15]. This report describes a patient with IgG4-related SM causing bowel obstruction.
A 77-year-old woman, who had a history of surgery for ectopic pregnancy and wound dehiscence 28 years earlier, presented to another hospital with intermittent abdominal pain. She was diagnosed with an intestinal obstruction and admitted to the hospital. Computed tomography (CT) revealed an irregularly shaped mass, 3 cm in diameter, in the mesentery of the ileum, which was suspected of causing her bowel obstruction. Medical treatment, including long-tube decompression, improved her symptoms, and she was referred to our hospital for further examination and treatment.
This report describes a patient with symptomatic SM as a manifestation of IgG4-RD. Because of its rarity, the etiology of SM remains undetermined [1–6]. Suggested causes include trauma (including surgery), powder on surgical gloves, infection (such as tuberculosis), autoimmune diseases, vascular insufficiency, and retained suture material [1–3]. About 40 to 70 % of patients with SM were found to have undergone previous surgery [1, 2]. Similarly, the patient described in this report had a history of surgery for ectopic pregnancy and wound dehiscence 28 years earlier. During surgery for SM, tight adhesions were seen throughout her abdomen. The pathogenic mechanism of SM seems to be a non-specific response to a wide variety of stimuli.
SM may be an IgG4-RD [10–13], diseases that dramatically respond to corticosteroid treatment [14, 15]. Recently, IgG4-RD was reported to be closely related to multifocal fibrosclerosis . IgG4-RD is characterized by organ enlargement and nodular/hyperplastic lesions in various organs, either concurrently or metachronously, due to marked infiltration of lymphocytes and IgG4-positive plasma cells, as well as to fibrosis of unknown etiology [14, 15]. Although the incidence of SM related to IgG4-RD has not been determined, SM was observed in 2 (4 %) of 57 patients with autoimmune pancreatitis  and marked infiltration of IgG4-positive plasma cells was observed in 4 (33 %) of 12 patients with SM . The comprehensive diagnostic criteria for IgG4-RD  require imaging and serum and histopathological examination. (1) Clinical examination should show characteristic diffuse/localized swelling or masses in single or multiple organs. (2) Hematological examination should show elevated serum IgG4 concentration (≥135 mg/dl). (3) Histopathologic examination should show marked lymphoplasmacytic infiltration, lymphoid follicles, obstructive phlebitis, dense fibrosis, and infiltration of IgG4-positive plasma cells.
The rate of IgG4- and IgG-positive cells diagnostic of IgG4-RD has been defined as >40 to 50 %, with >60 to 100 IgG4-positive cells present per high-powered field (HPF) . The degree of infiltration of IgG4- and IgG-positive plasma cells is analyzed in areas with the highest density of positive cells, with three HPFs evaluated in each patient and averaged . The postoperative serum IgG4 concentration in our patient was 114 mg/dl, higher than the normal range (4.8–105 mg/dl), but lower than the cutoff of 135 mg/dl. Thus, according to the above criteria, this patient should be diagnosed as having “probable” IgG4-RD.
Summary of the clinicopathological features of IgG4-related sclerosing mesenteritis
Serum IgG4 (mg/dl)
IgG4+ plasma cells count (/HPF)
IgG4+/IgG ratio (%)
Other IgG4-related disease
Steroid therapy (before resection)
Most patients with SM are symptomatic, with abdominal pain or a palpable abdominal mass being the most common clinical manifestations [1, 2, 8]. SM symptoms are caused by a direct mechanical effect of the mass on the bowels, vessels, and lymphatics, resulting in abdominal pain, bowel obstruction, ischemia, and ascites. Abdominal CT scanning is important for an accurate diagnosis. In the absence of histological analysis, SM can be diagnosed by CT findings of (1) hyperattenuating mesenteric fat, especially at the root of the small-bowel mesentery, (2) well-defined soft tissue nodules less than 5 mm in diameter surrounded by a fatty halo (fat ring sign), and (3) a tumoral pseudo-capsule . However, the imaging appearances of SM vary depending on the predominant tissue component (fat necrosis, inflammation, or fibrosis) . Therefore, SM may still be very difficult to distinguish accurately from other mesenteric diseases, such as gastrointestinal stromal tumor, malignant lymphoma, metastatic carcinoid tumor, desmoid tumor, and metastatic adenocarcinoma [5, 7–9].
The histological differential diagnosis in our patient included leiomyosarcoma (desmin+), inflammatory myofibroblastic tumor (ALK+/−, IgG4−), desmoid fibromatosis (β-catenin nuclear+), liposarcoma (CDK4+, MDM2+), follicular dendritic tumor (CD21+, CD35+), and malignant lymphoma (T/B cell marker, light chain restriction). A definitive diagnosis of SM requires histological examination of biopsy or surgically excised tissue specimens, unless other organs are apparently affected by IgG4-RD or percutaneous needle biopsy can be easily performed.
No consensus has yet been reached for the treatment for SM. Asymptomatic or mildly symptomatic SM may be left untreated [1, 2]. Surgical exploration is advocated in patients with life-threatening complications, such as bowel obstruction or perforation, or if there is high suspicion of an alternative diagnosis, such as malignancy. Surgical intervention, predominantly incomplete resection of SM, did not resolve symptoms or prevent disease progression . The effects of complete resection remain unknown, as complete resection is frequently prevented by vessel involvement. The patient described in this report underwent complete resection of SM and partial ileal resection, both for accurate diagnosis and for removal of the cause of bowel obstruction. Surgery resulted in good postoperative outcomes, without recurrence of the disease or symptoms after about 4 years of follow-up. Medical treatment, including with corticosteroids, tamoxifen, cyclophosphamide, and azathioprine, has also shown good results [1–3], although medical treatment for SM has not been standardized. If SM is a manifestation of IgG4-RD, corticosteroids may be promising.
SM may be associated with IgG4-RD in some patients. Although the accurate diagnosis of SM remains difficult in the absence of histological examination, IgG4-RD should be included in the differential diagnosis of unknown mesenteric tumors. Corticosteroids may be effective in these patients, thereby avoiding unnecessary surgery.
AA and TM wrote the manuscript. HY, YO, and MN supervised the study. NT, HY, and YO performed the pathological examinations. TU, DY, KN, YS, HF, and SN were attending physicians to the presented patient. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
The patient has consented in writing to the publication of images.
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