Table 3.
Extraglandular clinical manifestations of Sjögren's syndrome in the 4 Klinelfelter's syndrome compared to randomly selected 46,XY men and 46,XX women.
| KS1 | KS2 | KS3 | KS4 | pSS men (#/%) | pSS women | |
|---|---|---|---|---|---|---|
| (n=32) | (n=32) | |||||
| Lymphadenopathy | - | - | - | - | 9(28.1%) | 6(18.8%) |
| Gland swelling | - | + | - | + | 10(31.3%) | 7(21.9%) |
| Arthralgia/Arthritis | - | - | - | + | 20(62.5%) | 24(75%) |
| Vasculitis | - | - | - | - | 0 | 0 |
| Pulmonary | +1 | - | +1 | +1 | 0* | 0* |
| Raynaud's | - | + | + | - | 8(25.0%) | 13(40.6%) |
| Renal | - | - | - | - | 0 | 0 |
| Myositis | - | - | - | - | 0 | 0 |
| PNS‡ | - | - | - | - | 14(43.8%) | 8(25%) |
| CNS | - | - | - | - | 0 | 0 |
| Hepatitis | - | - | - | - | 1(3.1%) | 0 |
| Hematological | - | +2 | - | - | 7(21.9%)† | 4(12.5%) |
| Elevated IgG | - | + | + | + | 10(31.3%) | 5(15.6%) |
| RF | - | - | + | + | 10(31.3%) | 10(31.3%) |
| ANA | - | + | + | + | 26(81.3%) | 22(68.8%) |
ANA = antinuclear antibody, RF=rheumatoid factor, ND=not done or unknown
= All 3 positive have interstitial lung disease
= This patient has both leukopenia and lymphopenia
No patient had interstitial lung disease but 11 men and 9 had chronic cough. Comparing interstitial lung disease among the 47,XXY men and 46,XY men shows a statistical trend (p=0.11 by Fisher's exact test)
1 each with isolated leukopenia or thrombocytopenia, 3 with isolated lymphopenia, 1 with combined lymphopenia and thrombocytopenia, 1 with combined leukopenia and lymphopenia
Peripheral neuropathy was defined as decreased vibratory, pin prick, and light touch, as we have previously reported [27]. No patient was evaluated by electromyography or nerve conduction studies.