Neuropsychological deficit profiles in systemic lupus erythematosus.

Although neuropsychological deficits have been reported in several cognitive domains in patients with systemic lupus erythematosus (SLE), there is considerable variability in the literature about which neuropsychological domains are most affected. Similar to studies that demonstrated that specific profiles of neuropsychological deficits exist for those with traumatic brain injury (TBI; Johnstone, Hexum, & Ashkanazi, 1995), this study examined whether a specific pattern of deficits is present in SLE. By comparing reading scores (as estimates of premorbid ability) to tests of concurrent cognitive abilities (i.e., memory, attention, etc.), it was determined that SLE presents a profile distinct from TBI, with the most significant impairments noted in expressive language (Zdiff = -1.39), attention (Zdiff = -0.41), and speed of processing (Zdiff = -0.40). In contrast to TBI, no impairment was noted in intelligence, memory, or cognitive flexibility. Results suggest that memory problems reported by individuals with SLE may be related to inattention. Clinical implications are discussed.

Analysis of autoantibodies against RNA polymerases using immunoaffinity-purifed RNA polymerase I, II, and III antigen in an enzyme-linked immunosorbent assay.

Autoantibodies against RNA polymerases (RNAP) have been reported to occur in patients with a wide variety of connective tissue diseases (CTD), including systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD). The frequency of anti-RNAP antibodies has been reported to vary widely between different CTD diseases in studies examining different patient populations. Furthermore, these studies have been limited by the fact that methods have not previously been available for detecting antibodies against RNAP which are both rapid and quantitative. We have developed an enzyme-linked immunosorbent assay (ELISA) for rapidly quantitating antibodies against RNAP I, II, and III. We have utilized both the ELISA and the immunoprecipitation of 35S-labeled HeLa cells to analyze sera from a large cohort of well-characterized Caucasian CTD patients for the presence of anti-RNAP antibodies. We found excellent concordance for the presence of anti-RNAP antibodies using immunoprecipitation and ELISA. Anti-RNAP antibodies occurred predominantly among female patients with the diffuse form of SSc and were detected in 8/36 (22%) of Caucasian patients with diffuse SSc and 1/53 (2%) with limited SSc. Anti-RNAP antibodies occurred in 1/42 (2%) of patients with SLE. Anti-RNAP antibodies did not occur in MCTD (0/49). Antibodies against RNAP were rare among antinucleolar-reactive sera, occurring in only 3/200 (1.5%). The RNAP ELISA provides a validated method which can be rapidly utilized in a clinical diagnostic laboratory setting to identify SSc patients who are at risk for developing diffuse SSc with multiorgan involvement and hypertensive renal crisis.

Ruptured intrahepatic aneurysm as the initial manifestation of polyarteritis nodosa.

polyarteritis nodosa (PAN) is characterized by panarteritis involving all layers of the vessel wall of medium and small arteries and adjacent veins leading to thrombosis and aneurysmal dilatation. We present the case of a 21-year-old Caucasian man with an acute abdomen with a massive gastrointestinal (GI) hemorrhage caused by a large intrahepatic aneurysmal rupture needing surgery. This was the initial manifestation of underlying PAN, which had not been diagnosed before the event. This patient's postoperative course was complicated by continued hemorrhage prompting further diagnostic abdominal arteriography, which revealed multiple art-eurysms along the superior mesenteric artery. The aneurysmal wall biopsy revealed lymphocytic infiltrates, scattered giant cells, and minimal necrosis. A diagnosis of PAN was entertained based on arteriographic findings, although no pathognomonic demonstration of multiple visceral aneu-rysms is a characteristic finding of PAN, The patient survived the catastrophic event, and treatment with corticosteroids was initiated, which led to an uncomplicated hospital stay. Furthermore, outpatient follow ups showed continued clinical improvement with corticosteroid therapy and a repeat angiogram performed elsewhere demonstrated resolution of most of the aneurysms. Our case represents an unusual initial clinical manifestation of PAN and is one of the few cases reported with a torrential (GI) hemorrhage resulting from a rupture of an intrahepatic aneurysm, which is associated with increased mortality. The clinical significance of abdominal pain is not always apparent. It may occur in the absence of any gross intra-abdominal lesions, or, as in our patient, may herald an acute abdominal catastrophe. The clinician should consider PAN in the differential diagnosis of a massive GI hemorrhage. It is also important to note that early diagnosis followed by aggressive treatment can be lifesaving.