In view of the presence of autoantibodies against inner ear antigens, the pathogenesis of sudden deafness (SD) and progressive sensorineural hearing loss (PSNHL) is suggested to be of an autoimmune nature. However, microthrombosis of the inner ear may result from pathogenic anti-cardiolipin antibody (aCL) activity. We studied 30 patients (17 females and 13 males, age range 20-52 y), of whom 11 suffered from SD and 19 from PSNHL. All were clinically and serologically evaluated for association with autoimmune disorders (serological examination included: aCL, ANA, ENA, ANCA, proteinelectrophoresis, and complement levels). Twenty healthy matched subjects served as controls. None of the control group were aCL positive, whereas 8 out of 30 (27%) patients demonstrated low-moderate titers (P < 0.02), of whom 5 out of 8 suffered from SD. In addition, 2 aCL negative patients with PSNHL demonstrated hypergammaglubolinemia accompanied by hypocomplementemia, whereas none with SD had such abnormalities. Our data suggests that aCL is detected in patients with sudden sensorineural hearing loss and therefore may play an important role in the pathogenesis of this disability. If sustained by additional studies, these findings would warrant the consideration of anticoagulant therapy.

We have previously demonstrated that IgG autoantibodies derived from SLE patients are capable of penetrating into nuclei of living COLO-16 cells, in vitro. To address the possible correlation in Lupus Erythematosus (LE) between the in vivo ANA binding to nuclei of epidermal cells and the presence of intranuclear penetrating antibodies in sera of those patients, 25 consecutive patients were studied. Out of 25 skin biopsies, 11 specimens (8 of SLE and 3 of DLE) showed by immunofluorescent microscopy extensive in vivo presence of IgG in epidermal nuclei, whereas all sera of these patients stained nuclei of living COLO-16 cells, in vitro. Such penetration was also observed in additional 6/25 sera of patients, but with in vivo negative biopsies. This in vitro nuclear binding, which was unrelated to clinical symptoms of patients or their serological autoantibody profile and titer, was reproduced following cross-linking of intracellular protein by PLP fixation. Likewise, western blotting (immunoblotting) analysis, demonstrated the intranuclear presence of IgG in all in vitro intranuclear IgG staining sera. Furthermore, this in vitro presence, which neither affects cell viability nor DNA synthesis, is time-dependent and of a transient nature: nuclear staining disappears within 48 h following removal of the penetrating sera from medium. In conclusion, since the COLO-16 in vitro assay mirrors exactly the in vivo situation, and because of its higher sensitivity, it provides an excellent tool for the study of non-degraded autoantibody penetration into the nuclei of living cells.

Out of 280 immunotherapy (IT)-treated patients in our allergy clinic, 37 (13%) developed systemic adverse reactions. Parietaria judaica (Pj) extract, a highly allergenic pollen in northern Israel, was part of the IT regimen in 46% of treated patients who developed systemic adverse reactions. Twenty-six (70%) of systemic adverse reactions occurred during the buildup phase, whereas 11 (30%) occurred in the maintenance phase of treatment. Mild systemic reactions developed in 15/37 (40%), moderate in 20/37 (54%), and severe in 2/37 (5%) of patients. In 22/37 (59% of our IT-treated patients, adverse reactions developed within 30 min after injection. Among these were the two patients with severe systemic reactions. In 19%, moderate adverse reactions appeared at 30-60 min; in 22%, mild to moderate reactions appeared after 1-2 h. Our study concludes that severe systemic reactions to IT usually appear within 30 min after injection. In Israel, IT with highly allergenic pollens such as Pj frequently causes systemic reactions, even during the maintenance phase of treatment. In such cases, the reduction of IT dosage should be more than 50% during the pollen season, and a waiting period of 1 h should also be considered.

A 40y old woman with primary Sjögren's syndrome developed elevated purpura, peripheral neuropathy, muscular tenderness, abdominal pain, heart failure, and convulsive spells. The hallmarks of this disease were high titers of anti-Ro antibodies and low complement levels in the serum, leukocytoclastic small vessel vasculitis in the cutaneous biopsy specimen, and a life threatening clinical course. Echocardiography revealed left ventricular hypokinesis with low ejection fraction, which is unlike the more common features of cardiomyopathy complicating Sjögren's syndrome. The rapidly deteriorating heart failure and other systemic complications remitted on pulse corticosteroid and cyclophosphamide therapy. The pathogenesis of heart failure, which appeared concurrently with vasculitis and was reversed on immunosuppressive therapy, is explained in the context of the systemic disease. Leukocytoclastic vasculitis might be at the origin of this rare variant of acute, severe but reversible cardiomyopathy in pSS.

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