Journal of Endocrinology and Metabolism, ISSN 1923-2861 print, 1923-287X online, Open Access |
Article copyright, the authors; Journal compilation copyright, J Endocrinol Metab and Elmer Press Inc |
Journal website http://www.jofem.org |
Original Article
Volume 6, Number 2, April 2016, pages 46-51
Clinical, Endocrinological and Immunological Characteristics of Japanese Patients With Autoimmune Polyglandular Syndrome Type 3a
Tables
Cases [Reference] | Age (years)/sex | Type of diabetes | Anti-GAD antibody | Urinary C-peptide (μg/day) | Positivity for anti-TPO or anti-TG antibody | Thyroid function | Other complication |
---|---|---|---|---|---|---|---|
F: female; GAD: glutamic acid decarboxylase; MRHE: mineralocorticoid-responsive hyponatremia with the elderly; RA: rheumatoid arthritis; ref: reference; RF: rheumatoid factor; SPIDDM: slowly progressive insulin-dependent diabetes mellitus; TG: thyroglobulin; TPO: thyroid peroxidase. Normal ranges of anti-GAD antibody and urinary C-peptide are < 1.5 U/mL and 29.2 - 167.0 μg/day, respectively. | |||||||
Case 1 [4] | 53/F | SPIDDM | 10,000 | 70 | (+) | Euthyroid | Symptomatic Sjogren’s syndrome with anti-SSA/Ro ab (+) and anti-SSB/La ab (+); asymptomatic RA with RF (+) |
Case 2 [7] | 83/F | SPIDDM | 3.2 | 9.1 | (+) | Treated by levothyroxine (25 μg/day) | MRHE |
Case 3 [8] | 68/F | SPIDDM | 34 | 52.9 | (+) | Euthyroid | None |
Case 4 [9] | 84/F | SPIDDM | 65.2 | < 0.3 | (-) | Treated by levothyroxine (50 μg/day) | Sarcoidosis, candidiasis, and liver cirrhosis due to autoimmune hepatitis |
Cases | Age (years)/sex | Type of diabetes | Anti-GAD antibody (U/mL) | Anti-IA2 antibody (U/mL) | HbA1c (%) | Serum CPR (ng/mL) | Urinary CPR (μg/day) | Treatment for diabetes | Anti-TPO antibody (IU/mL) | Anti-TG antibody (IU/mL) | Anti-TR antibody (IU/L) | Thyroid function, findings of thyroid ultrasonography and treatment |
---|---|---|---|---|---|---|---|---|---|---|---|---|
No swelling indicates the absence of thyroid swelling determined by thyroid ultrasonography. AOT1D: acute onset type 1 diabetes; CPR: C-peptide immunoreactivity; F: female; GAD: glutamic acid decarboxylase; GLP-1: glucagon-like peptide-1; IA2: islet antigen 2; M: male; MMI: methylmercapto-imidazole; NGT: normal glucose tolerance; OAD: oral anti-diabetic drug; SPIDDM: slowly progressive insulin-dependent diabetes mellitus; TG: thyroglobulin; TPO: thyroid peroxidase; TR: thyroid stimulating hormone receptor; UD: undetermined. Normal ranges of anti-GAD antibody, anti-IA2 antibody, serum CPR, urinary CPR, anti-TPO antibody, anti-TG antibody and anti-TR antibody are < 1.5 U/mL, < 0.4 U/mL, 0.61 - 2.09 ng/mL, 29.2 - 167.0 μg/day, < 16 IU/mL, < 28 IU/mL and < 2.0 IU/L, respectively. | ||||||||||||
1 | 80/F | UD | 13 | 0.9 | 12.1 | < 0.03 | < 0.5 | Insulin | 11 | 207 | 0.4 | Euthyroidism, no treatment |
2 [4] | 53/F | SPIDDM | 10,000 | 11 | 10.2 | 0.26 | 70 | Insulin | > 600 | 298 | 0.7 | Euthyroidism, no treatment |
3 | 49/M | SPIDDM | 4,140 | 11 | 5.8 | 1.86 | UD | Insulin + OAD | 454 | 546 | 11.3 | Euthyroidism, no treatment |
4 | 81/F | UD | 49,000 | 2.9 | 9.8 | < 0.03 | 1.2 | Insulin | < 5 | 82 | 0.4 | Euthyroidism, no swelling, and no treatment |
5 | 72/F | AOT1D | 970 | 8.4 | 10 | < 0.03 | 1.7 | Insulin | 109 | 21 | 25.2 | Hyperthyroidism, treated by MMI |
6 [8] | 68/F | SPIDDM | 34 | < 0.4 | 8.3 | 4.77 | 52.9 | Insulin + OAD + GLP-1 analog | 71 | 497 | 0.5 | Euthyroidism, no swelling, and no treatment |
7 | 62/F | SPIDDM | 1,300 | < 0.4 | 8.7 | < 0.03 | UD | Insulin | 195 | 23 | 0.4 | Euthyroidism, no treatment |
8 | 71/F | SPIDDM | 6.3 | < 0.4 | 11.9 | 6.04 | 36.4 | Insulin | 26 | 17 | < 1 | Euthyroidism, no swelling, and no treatment |
9 | 50/M | SPIDDM | 2.4 | > 0.4 | 8.5 | 4.75 | UD | OAD | > 600 | 411 | 0.4 | Euthyroidism, no treatment |
10 | 80/F | SPIDDM | 53 | < 0.4 | 6.8 | 2.64 | UD | OAD | 62 | 11 | 0.6 | Euthyroidism, no treatment |
11 | 69/M | SPIDDM | 36 | < 0.4 | 6.5 | 3.84 | UD | OAD | 502 | 480 | 0.7 | Euthyroidism, no treatment |
12 [9] | 84/F | SPIDDM | 65.2 | UD | 5.9 | < 0.03 | < 0.3 | Insulin | < 5 | < 10 | UD | Hypothyroidism, treated by levothyloxine |
13 [7] | 83/F | SPIDDM | 3.2 | < 0.4 | 6.3 | 0.84 | 9.1 | Diet + exercise | 8 | < 10 | UD | Hypothyroidism, treated by levothyloxine |
14 | 47/M | NGT | 2.9 | < 0.4 | 5.6 | 3.0 | UD | No treatment | 73 | 488 | 0.4 | Hypothyroidism, treated by levothyloxine |
15 | 42/M | AOT1D | 39 | < 0.4 | 10.5 | 0.05 | 4.9 | Insulin | 163 | 23 | 10.2 | Hyperthyroidism, treated by MMI |
16 | 43/M | AOT1D | 155 | < 0.4 | 8.6 | 0.18 | UD | Insulin | 251 | 946 | 1 | Hypothyroidism, treated by levothyloxine |
17 | 76/F | SPIDDM | 57 | < 0.4 | 7.2 | 1.87 | UD | OAD | > 600 | 12 | 0.4 | Subclinical hypothyroidism, no treatment |
18 | 67/M | SPIDDM | 2.2 | < 0.4 | 6.9 | 1.65 | UD | OAD | 9 | 182 | 0.4 | Euthyroidism, no swelling, and no treatment |
19 | 60/F | SPIDDM | 550 | < 0.4 | 6.6 | 3.41 | 30.9 | OAD | 16 | 76 | 2.6 | Hyperthyroidism, treated by iodine isotope treatment |
20 | 83/F | SPIDDM | > 2,000 | < 0.4 | 9.6 | < 0.03 | < 0.9 | Insulin + OAD | 417 | 121 | < 0.3 | Euthyroidism, no swelling, and no treatment |
21 | 67/F | UD | < 0.3 | 1 | 7.5 | < 0.03 | 9.8 | Insulin | 20 | 818 | < 0.3 | Euthyroidism, no treatment |
22 | 42/F | AOT1D | 0.7 | 3.9 | 8.7 | < 0.03 | UD | Insulin | 8 | 12 | 164 | Hyperthyroidism, treated by MMI |
23 | 53/F | SPIDDM | < 0.3 | 0.5 | 6.6 | 2.64 | 153 | Diet + exercise | 8 | 14 | 4.5 | Hyperthyroidism, treated by MMI |
24 | 53/F | UD | 1.1 | < 0.4 | 9.7 | 0.08 | UD | Insulin | 392 | 151 | 0.5 | Subclinical hypothyroidism, no treatment |
25 | 52/F | AOT1D | < 0.3 | < 0.4 | 8.0 | < 0.03 | < 0.9 | Insulin | 188 | 577 | 0.7 | Euthyroidism, no treatment |
26 | 51/M | AOT1D | < 0.3 | < 0.4 | 12.7 | 0.43 | UD | Insulin | 27 | 12 | < 0.3 | Euthyroidism, no treatment |
GAD: glutamic acid decarboxylase; GLP-1: glucagon-like peptide-1; IA2: islet antigen 2; OAD: oral anti-diabetic drug; SPIDDM: slowly progressive insulin-dependent diabetes mellitus; TG: thyroglobulin; TPO: thyroid peroxidase; TR: thyroid stimulating hormone receptor. | |
Sex (male/female) | 8/18 |
Autoimmune diabetes | |
SPIDDM | 15/26 (57.7%) |
Acute onset type 1 diabetes | 6/26 (23.1%) |
Normal glucose tolerance | 1/26 (3.8%) |
Anti-GAD antibody (+) | 20/26 (76.9%) |
Anti-IA2 antibody (+) | 8/26 (30.8%) |
Treatment for diabetes | |
Insulin | 14/26 (53.8%) |
Insulin + OAD | 2/26 (7.7%) |
Insulin + OAD + GLP-1 analog | 1/26 (3.8%) |
OAD | 6/26 (23.1%) |
Diet + exercise | 2/26 (7.7%) |
No treatment | 1/26 (3.8%) |
Autoimmune thyroid diseases | |
Possible Hashimoto thyroiditis | 21/26 (80.8%) |
Overt Hashimoto thyroiditis | 4/26 (15.4%) |
Subclinical hypothyroidism due to Hashimoto thyroiditis | 2/26 (7.7%) |
Immunological Hashimoto thyroiditis | 15/26 (57.7%) |
Anti-TPO antibody (+) | 19/26 (73.1%) |
Anti-TG antibody (+) | 15/26 (57.7%) |
Possible Graves’s disease | 6/26 (23.1%) |
Overt Grave’s disease | 5/26 (19.2%) |
Immunological Grave’s disease | 1/26 (3.8%) |
Anti-TR antibody (+) | 6/26 (23.1%) |
Mean ± SD | Range | |
---|---|---|
When we determined mean ± SD of each variable including variables under the lower limit and above the upper limit, we used variables of upper and lower limit. CPR: C-peptide immunoreactivity; GAD: glutamic acid decarboxylase; fT4: free thyroxine; fT3: free triiodothyronine; IA2: islet antigen 2; TG: thyroglobulin; TPO: thyroid peroxidase; TR: thyroid stimulating hormone receptor; TSH: thyroid stimulating hormone. Normal ranges of anti-GAD antibody, anti-IA2 antibody, serum CPR, urinary CPR, anti-TPO antibody, anti-TG antibody, anti-TR antibody, TSH, fT3 and fT4 are < 1.5 U/mL, < 0.4 U/mL, 0.61 - 2.09 ng/mL, 29.2 - 167.0 μg/day, < 16 IU/mL, < 28 IU/mL, < 2.0 IU/L, 0.54 - 4.26 μIU/mL, 2.39 - 4.06 pg/mL and 0.71 - 1.52 ng/dL, respectively. | ||
Age | 63.0 ± 14.2 | 42 - 86 |
HbA1c | 8.4 ± 2.0 | 5.6 - 12.7 |
Anti-GAD antibody (U/mL) | 2,632.0 ± 9,685.0 | < 0.3 - 49,000 |
Anti-IA2 antibody (U/mL) | 1.86 ± 3.26 | < 0.4 - 11.0 |
Serum CPR (ng/mL) | 1.48 ± 1.84 | < 0.03 - 3.84 |
Urinary CPR (μg/day) | 29.9 ± 55.2 | < 0.5 - 153 |
Anti-TPO antibody (IU/mL) | 185.4 ± 215.7 | < 5.0 - > 600 |
Anti-TG antibody (IU/mL) | 232.5 ± 275.1 | < 10.0 - 946 |
Anti-TR antibody (IU/L) | 9.47 ± 33.4 | < 0.3 - 25.2 |
TSH | 3.54 ± 6.09 | < 0.03 - 29.91 |
fT3 | 3.65 ± 2.03 | 2.09 - 5.92 |
fT4 | 1.15 ± 0.75 | 0.67 - 1.74 |