Table 11.

The use of intravenous immunoglobulins in endocrine disorders

Results/response	Number of patients	Intervention including dose and IVIg preparation used	Study design	Ref	Disease
21 of 26 patients showed improvement of the mean Neuropathy Impairment Score (NIS) from 61.5 ± 26.0 to 33 ± 29.6 (p < 0.001); improvement of NIS was more frequent in patients with a conduction block (100%) vs. in those who did not (66.7%; p = 0.03); relapses occurred less in the responders who had a conduction block (9.1%) vs. in those who did not (50%; p = 0.04)	26 patients with DDP evaluated at baseline, at the end of 4 weeks of IVIg therapy, afterwards every few months, mean follow-up period of 25 months.	IVIg 0.4 g/kg for 5 days	Prospective open label	[331]	Diabetic demyelinating polyneuropathy
Reached 80% reduction of pains and significant recovery of muscle strength	A 57-year-old man with type 2 diabetes and painful proximal diabetic neuropathy with muscle weakness and atrophy	IVIg 0.4 g/kg for 5 days	Case report	[332]	Proximal diabetic neuropathy
All defective cranial nerve findings disappeared during the 1st month and did not recur during 8 months of follow-up	A 55-year-old woman, with 12-year history of DM, developed simultaneous right VII and left III, IV, and VI cranial nerve pulses	IVIg 0.4 g/kg for 5 days	Case report	[333]	Cranial nerve neuropathy in diabetes mellitus
5 days after IVIg easy fatigability of thigh disappeared and of the strength of shoulder girdle muscle improved; 1 year after IVIg treatment, there was normal muscle strength and significant improvement of atrophy	A 45-year-old man with IDDM and diabetic amyotrophy	IVIg 0.4 g/kg per day for 5 days	Case report	[334]	Diabetic amyotrophy
Rapid improvement of fasting serum glucose levels, stabilization of renal function, and decrease in the donor-specific class II antibodies	A 44-year-old man with long-standing poorly controlled IDDM	3 doses of 0.5 g/kg per day of IVIg, started on day 14 posttransplant and given every other day adjunctive to standard posttransplant therapy, plasmapheresis, and rituximab	Case report	[335]	Pancreas allograft rejection
Partial response to IVIg and complete resolution of weakness after CP, asymptomatic after 1 year without treatment	A 17-year-old man with known IDDM and a subclinical Hashimoto's thyroiditis and a new onset of asymmetric weakness and atrophy in arms	Monthly infusions of IVIg 0.4 g/kg per day for 5 consecutive days followed by 6 cycles of CP 1 g/m2	Case report	[336]	Multifocal motor neuropathy, type 1 diabetes, and Hashimoto's thyroiditis
Within 10 weeks after IVIg, muscle weakness and sensory disturbances disappeared, but tendon reflexes stayed slightly depressed; the patient was treated with IVIg every 3 months due to IVIg-dependent course	A 64-year-old man with clinical and laboratory findings supportive for MMN, high titers of anti-GM1-Abs and subclinical Hashimoto's thyroiditis	IVIg 0.4 g/kg per day for 5 days, repeated every 3 months due to IVIg-dependent course of the disease	Case report	[337]	Multifocal motor neuropathy and Hashimoto's thyroiditis
Partial response to CS and IVIg and antiepileptic medication (probably due to lowering of seizure threshold by antithyroid drugs and beta-blockers); continued with immunosuppressive therapy (high-dose CS and AZA) but relapsed after 2 months, recovered after thyroidectomy	A 34-year-old woman with encephalopathy and thyrotoxic Hashimoto's thyroiditis	IVIg 2 g/kg in 3 days adjunctive to high-dose CS and plasmapheresis	Case report	[338]	Thyrotoxic autoimmune encephalopathy
See ophthalmology section					Grave's ophthalmopathy
All had normalization of VLCFA within 3 months, but neurological status continued to deteriorate in both groups	6 of 12 patients with X-linked childhood and adolescent adrenoleukodystrophy received IVIg in addition to diet and GTOE supplement	IVIg 1 g/kg in 8–12 h (Gamma globulin; Merieux, France) every 15 days for 3 months and every month for a year, thereafter; both groups received very-long-chain fatty acids (VLCFA)-restricted diet and supplement of 40 mg/day glycerol trioleate and erucic acids (GTOE)	RCT	[339]	Adrenoleukodystrophy
3/9 improved; 3/9 remained stable; 3/9 deteriorated and died	9 adult patients with various X-ALD phenotypes	15 cycles of 1 g/kg 7 S-immunoglobulin (Venimmun, Behring, Germany)	Uncontrolled trial	[340]
Recovery of adrenal function but dependence on dialysis 18 months later	A 20-year-old man with Waterhouse–Friderichsen syndrome and bilateral renal cortical necrosis secondary to meningococcal sepsis	Neither protocol nor IVIg preparation is available; IVIg adjunctive to aggressive treatment with antibiotics, CS, vasopressors, plasmapheresis, and dialysis	Case report	[341]	Waterhouse–Friderichsen syndrome
During IVIg treatment, normalization of thyroid function, some nonovulatory menses, and reduction of antibody titers were observed	A female with autoimmune polyendocrine syndrome type 2	IVIg	Case report	[342]	Autoimmune polyendocrine syndrome