IgG4-related Disease as Systemic Disease

We are reporting a case of a 63-year-old Chinese female who presented to the rheumatology clinic with positive antinuclear antibody and unintentional weight loss along with lymphadenopathy. Further workup revealed eosinophilia, elevated anti–double stranded DNA, serum protein, and serum IgG4 (immunoglobulin G4). The patient was diagnosed with systemic lupus erythematosus. Due to the raised IgG4 level along with eosinophilia and diffuse lymphadenopathy, IgG4-related systemic disease was suspected. It was confirmed with IgG4 staining on lymph node biopsy. Our case is presenting the fact that systemic lupus erythematosus and IgG4-related disease can be present in the same patient with multiple overlapping features making accurate diagnosis challenging.

Download Full-text

IgG4-related thyroiditis: a case report and review of literature

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-14-0037 ◽

2014 ◽

Vol 2014 ◽

Cited By ~ 1

Author(s):

Mahmud Abo Salook ◽

Carlos Benbassat ◽

Yulia Strenov ◽

Amit Tirosh

Keyword(s):

Hashimoto’S Thyroiditis ◽

Systemic Disease ◽

Hashimoto's Thyroiditis ◽

List Type ◽

Review Of Literature ◽

Related Disease ◽

Igg4 Related Disease ◽

Aggressive Form ◽

Learning Points ◽

Very High

Summary A 55-year-old male, with a positive medical history for hypothyroidism, treated with stable doses for years was admitted with subacute thyroiditis and a feeling of pain and pressure in the neck. Laboratory tests showed decrease in TSH levels, elevated erythrocyte sedimentation rate, and very high antithyroid antibodies. Owing to enlarging goiter and exacerbation in the patient's complaints, he was operated with excision of a fibrotic and enlarged thyroid lobe. Elevated IgG4 plasma levels and high IgG4/IgG plasma cell ratio on immunohistochemistry led to the diagnosis of IgG4-mediated thyroiditis. We concluded that IgG4-thyroiditis and IgG4-related disease should be considered in all patients with an aggressive form of Hashimoto's thyroiditis. Learning points IgG4-related disease is a systemic disease that includes several syndromes; IgG4-related thyroiditis is one among them. IgG4-thyroiditis should be considered in all patients with an aggressive form of Hashimoto's thyroiditis. Patients with suspected IgG4-thyroiditis should have blood tested for IgG4/IgG ratio and appropriate immunohistochemical staining if possible.

Download Full-text

IgG4-related Disease - A Systemic Disease that Deserves Attention Regardless of One's Subspecialty

Internal Medicine ◽

10.2169/internalmedicine.9533-17 ◽

2018 ◽

Vol 57 (9) ◽

pp. 1201-1207 ◽

Cited By ~ 12

Author(s):

Hideaki Hamano ◽

Eiji Tanaka ◽

Nobukazu Ishizaka ◽

Shigeyuki Kawa

Keyword(s):

Systemic Disease ◽

Related Disease ◽

Igg4 Related Disease

Download Full-text

PS01.097: A CASE OF IGG4-RELATED SCLEROSING ESOPHAGITIS

Diseases of the Esophagus ◽

10.1093/dote/doy089.ps01.097 ◽

2018 ◽

Vol 31 (Supplement_1) ◽

pp. 77-77

Author(s):

Hirokazu Miki ◽

Taku Michiura ◽

Hiromi Mukaide ◽

Kentaro Inoue ◽

Keigo Yamamichi ◽

...

Keyword(s):

Plasma Cells ◽

Conflicts Of Interest ◽

Systemic Disease ◽

Esophageal Resection ◽

Esophageal Disease ◽

Resected Specimen ◽

Thoracic Esophagus ◽

Related Disease ◽

Igg4 Related Disease ◽

Mucosal Erosion

Abstract Background IgG4-related disease (IgG4-RD) is a systemic disease characterized by fibrosing inflammation with an abundance of IgG4-positive plasma cells. IgG4-RD has been found to affect various organs. Many cases of IgG4-RD affecting the extrapancreatic organs have been reported in patients without pancreatic lesions. Methods Sclerosing esophagitis with IgG4-positive plasma cell infiltration is very rare disease. We report a case of esophageal benign stricture that was difficult to diagonose. Results A 76-year-old woman had been experiencing slight difficulty in swallowing in the 3 years prior to this presentation. Her dysphagia had progressed and she began to vomit frequently after meals and lost weight while hospitalized after surgery for cervical cancer. Esophagogastroduodenoscopy and an esophagram showed circumferential erosion and a stricture of the thoracic esophagus. No malignant cells were identified. However, because it was difficult to exclude a malignant tumor, she underwent esophageal resection. The resected specimen showed stricture with mucosal erosion and transmural wall hypertrophy. On histological examination, transmural hyperplasia consisting of inflammatory granulation tissue with abundant infiltration of IgG4-positive plasma cells and lymphocytes was observed. Conclusion A probable diagnosis of IgG4-related disease was made because comprehensive diagnostic criteria for this condition were met. IgG4-related esophageal disease presenting as esophageal lesions alone is very rare. Disclosure All authors have declared no conflicts of interest.

Download Full-text

Immunoglobulin G4-Related Disease Mimicking Asthma

Canadian Respiratory Journal ◽

10.1155/2013/619453 ◽

2013 ◽

Vol 20 (2) ◽

pp. 87-89 ◽

Cited By ~ 16

Author(s):

Hiroshi Sekiguchi ◽

Ryohei Horie ◽

Timothy R Aksamit ◽

Eunhee S Yi ◽

Jay H Ryu

Keyword(s):

Airway Inflammation ◽

Submandibular Gland ◽

Autoimmune Pancreatitis ◽

Plasma Cells ◽

Systemic Disease ◽

Immunoglobulin G4 ◽

Related Disease ◽

Igg4 Related Disease ◽

Organ Manifestations

Immunoglobulin (Ig) G4-related disease (also known as ‘IgG4-related sclerosing disease’, ‘IgG4-related systemic disease’ or ‘hyper-IgG4-disease’) is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

Download Full-text