Retroperitoneal fibrosis associated with immunoglobulin G4-related disease

Table 1 Etiological factors of retroperitoneal fibrosis

Etiological factors
Idiopathic RF
IgG4-related lesions	Frequency unknown
Secondary RF
Drugs	Analgesics, β-blockers, etc.
Malignant diseases	Malignant lymphoma, etc.
Infections	Tuberculosis, etc.
Radiotherapy	Colon cancer, pancreatic cancer, etc.
Surgery	Lymphadenectomy, colectomy, etc.
Others	Trauma, etc.

RF: Retroperitoneal fibrosis.

Table 2 Retroperitoneal fibrosis as extrapancreatic lesions of autoimmune pancreatitis

Cases studied: 51 patients with AIP (2002–2011)
Concurrent RF	8 patients (15.7)
Synchronized onset	5 patients
Heterochronic onset	3 patients
Preceding the diagnosis of AIP	2 patients
Following the diagnosis of AIP	1 patient
Treatment: Steroid therapy at a dose of 30–40 mg per day
Improved	7 patients (87.5)
Unchanged	1 patient (12.5)

RF: Retroperitoneal fibrosis; AIP: Autoimmune pancreatitis.

Table 3 Frequency of concurrent retroperitoneal fibrosis in immunoglobulin G4-related disease

Ref.	Disease	Total number of patients	Patients with RF n	Frequency of concurrent RF
Takuma et al[18]	AIP	56	4	7.1
Kamisawa et al[19]	AIP	459	50	10.9
Hirano et al[20]	AIP	42	8	19.0
Ohara et al[21]	AIP	132	8	6.1
Hamano et al[22]	AIP	64	8	12.5
Ryu et al[23]	AIP	67	2	3.0
Song et al[24]	AIP	25	1	4.0
Chari et al[25]	AIP	29	3	10.3
Raina et al[26]	AIP	26	2	7.7
Maire et al[27]	AIP	28	1	3.6
Ohta et al[28]	IgG4-related sclerosing sialadenitis	10	1	10.0
Zen et al[29]	IgG4-related sclerosing disease	114	13	11.4
Our cases	AIP	51	8	15.7
Total	IgG4-related disease	1103	109	9.9

RF: Retroperitoneal fibrosis; AIP: Autoimmune pancreatitis; IgG4: Immunoglobulin G4.