Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy.

OBJECTIVES: To describe the clinical features and outcomes of patients with primary central nervous system vasculitis (PCNSV) and cerebral amyloid angiopathy (CAA) from a large cohort of consecutive patients with PCNSV treated at a single institution. METHODS: We identified 101 consecutive patients with PCNSV admitted between January 1983 and December 2003. PCNSV diagnoses were based on findings from a central nervous system (CNS) biopsy (n = 31) and conventional angiography (n = 70). CNS tissue specimens from 49 cases were examined histologically, and 49 were stained for amyloid deposits. Those with vascular amyloid deposits (CAA) were compared with those without histological evidence of amyloid deposition. RESULTS: Eight cases (26%) with CNS biopsy specimens positive for PCNSV also showed findings of CAA. Compared with patients with PCNSV only, these patients were older at diagnosis, predominantly male, had a more acute onset, a higher frequency of cognitive dysfunction and showed prominent gadolinium-enhanced leptomeningeal lesions with MRI. Histologically, all had a granulomatous vascular inflammatory pattern. Six patients responded promptly to therapy. Outcomes at last follow-up were similar in the two groups. CONCLUSIONS: PCNSV with CAA appears to form a clinical subset of PCNSV. The vasculitis influences the clinical findings to a greater degree than the presence of amyloid deposits in the vessels.

Primary CNS vasculitis with spinal cord involvement.

BACKGROUND: Primary CNS vasculitis (PCNSV) is an uncommon disease in which lesions are limited to the brain and spinal cord. Our objective was to evaluate the frequency, clinical features, and outcome of spinal cord involvement in PCNSV. METHODS: We retrospectively identified 101 consecutive patients with PCNSV. Spinal cord involvement was documented for five. Clinical findings, laboratory studies, and outcomes of patients with spinal cord involvement were assessed and compared with those without spinal cord manifestations. RESULTS: Spinal cord symptoms developed before cerebral symptoms in one patient, concurrently in two, and after cerebral symptoms in two. CNS biopsy specimens showed necrotizing vasculitis in three patients and granulomatous vasculitis in two. MRI of the spinal cord showed enhanced thoracic lesions in all five. Cerebral angiograms from four patients had normal findings. One patient had a fatal clinical course. The other four had relapses during follow-up but responded well to therapy and had favorable overall outcomes. At the last follow-up (median, 19 months after diagnosis), the four patients had recovered with slight or moderate residual disability. No significant differences in clinical and laboratory features were observed when comparing patients with or without spinal cord involvement. Cerebral angiograms with evidence of vasculitis were significantly more frequent for patients without spinal cord involvement (p = 0.002). CONCLUSION: Spinal cord involvement was documented in 5% of patients with primary CNS vasculitis. The thoracic cord was the predominantly affected site. Other than myelopathy, clinical characteristics were similar to those of the patients without spinal cord involvement.

18F-fluorodeoxyglucose-positron emission tomography: a new explorative perspective.

18F-Fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) is a new functional imaging technique available for clinical and experimental use. 18F-FDG-PET studies can be used for screening, localization and follow-up of hypermetabolic processes including malignancies, infections and autoimmune processes. For several years it has been applied in oncological, cardiological and neurological patients, but nowadays an increasing number of studies favours its use in patients with autoimmune diseases including large vessel arteritis. From the experimental view, this technique has even become more important since the introduction of a small PET scanner for the use in animal models. This review focuses on technical aspects, clinical experiences and experimental and future perspectives of 18F-FDG-PET, with a special emphasis on large vessel vasculitis and other autoimmune diseases.

Endoscopic YAG laser treatment of watermelon stomach (gastric antral vascular ectasia) in patients with systemic sclerosis.

OBJECTIVE: Gastric antral vascular ectasia (GAVE) has been recognized as a rare but important cause of chronic iron deficiency anemia. A number of reported patients were found to have evidence of autoimmune disorders or connective tissue diseases. We carried out this study in order to determine the clinical, endoscopic, and pathologic features in a large series of patients with systemic sclerosis (SSc) who were diagnosed with GAVE. We also determined the response to YAG laser treatment for chronic blood loss in these patients. METHODS: A retrospective chart review of 20 cases diagnosed over an 11-year period, with diagnoses of both SSc and GAVE. RESULTS: Twenty patients with SSc presented with prominent anemia and were diagnosed with GAVE. Treatment with endoscopic laser therapy was successful in preventing surgery for bleeding in GAVE in 85% of cases. CONCLUSIONS: GAVE should be considered in patients with SSc who develop chronic iron deficiency anemia. YAG laser treatment can be useful in the treatment of chronic blood loss anemia in SSc patients with GAVE.

Chronic obstructive pneumonia caused by a vertebral body osteophyte.

Osteophytes associated with spondylosis have been implicated as a cause of multiple extraspinal manifestations. Symptoms are more likely to occur with the large osteophytes associated with diffuse idiopathic skeletal hyperostosis. In the thoracic region, osteophytes have been reported infrequently as a cause of extraspinal complications. We report a case in which an anterior thoracic vertebral osteophyte was responsible for chronic obstructive pneumonia due to obstruction of the right main stem bronchus. The patient's condition improved considerably after surgical resection of the compressing thoracic osteophyte.

Intravascular lymphomatosis. A report of ten patients with central nervous system involvement and a review of the disease process.

The clinical, radiographic, and pathological findings in ten cases of intravascular lymphomatosis with central nervous system involvement seen at our institution over a 15-year period are presented. Nine patients presented with a subacute, progressive multifocal neurologic disorder. Most patients had fever, anemia, and elevation of the erythrocyte sedimentation rate. As the illness evolved, computerized tomography scanning and magnetic resonance imaging showed evidence of multifocal central nervous system disease. Angiography was nondiagnostic but suggested vasculitis in six cases. A response to empiric corticosteroid treatment was typical but usually transient. In six patients, the diagnosis was made antemortem by brain biopsy. The prognosis of patients was primarily dependent on early diagnosis and treatment, before massive central nervous system damage occurred. Treatment with chemotherapy, with or without radiotherapy, was associated with stabilization of the disease in three of five patients.

A study of the cutaneous manifestations of Behçet's disease in patients from the United States.

BACKGROUND: The type and frequency of different manifestations of Behçet's disease (BD) vary in different geographic areas. This variability could affect the ability to diagnose the disease in certain areas by using standardized criteria. The frequency of cutaneous lesions in patients from the United States, where the disease is less prevalent, is not known. OBJECTIVE: We sought to determine the frequency and type of skin lesions in a series of patients with BD from the United States and to identify methods of confirmation of these lesions as part of the disease process. RESULTS: Cutaneous manifestations were present in 64% of patients with BD. Clinicians most often relied on their clinical diagnosis to identify lesions as part of the spectrum of BD. Skin biopsy specimens were generally nonspecific. CONCLUSION: Cutaneous manifestations were common in patients with BD from the United States and usually were necessary to fulfill the diagnostic criteria of the disease in most cases.

Pilot study of antithymocyte globulin in systemic sclerosis.

OBJECTIVE: Between June 1, 1992 and August 31, 1994 we conducted an open pilot study of antithymocyte globulin (ATGAM; Upjohn, Kalamazoo, MI) in 10 patients with early systemic sclerosis (SSc) to assess whether this agent might prevent the progression of cutaneous and pulmonary involvement in this disease. METHODS: Adult patients with early SSc (< 3 years) and evidence of progressive skin and pulmonary disease were enrolled. All patients were hospitalized and received a single course of intravenous ATGAM, at a dosage of 10 mg/kg over 4 hours, on 5 consecutive days. Patients were followed up at weeks 1, 2, 3, and 4, and months 2, 4, 6, and 12. Patients were considered to be improved if the Rodnan skin score decreased > or = 25%, to be worse if the skin score increased > or = 25%, and to be not improved if the skin score was within 25% of baseline. For pulmonary involvement, patients were considered to be improved if either the diffusing capacity for carbon monoxide or the forced vital capacity was increased > or = 10%, worse if decreased by > or = 10%, and stable if within 10% of baseline. RESULTS: Most patients tolerated the treatment well, although 1 patient developed an allergic reaction necessitating discontinuation of treatment, 1 developed a serum sickness reaction after completion of therapy, and 1 developed a central venous access-related axillary vein thrombosis. Two patients died of SSc-related complications during the followup period. At 12 months, only 2 patients showed improvement in both skin and pulmonary function measures, whereas 5 patients were worse and 3 were stable. CONCLUSION: At the dosage administered in this study, ATGAM appears ineffective in improving the skin and pulmonary features of SSc.

HLA-DR locus antigens in polymyalgia rheumatica and giant cell arteritis.

HLA-DR locus antigens were determined in 69 patients with giant cell arteritis, polymyalgia rheumatica, or both. The frequencies of HLA-DR4 and HLA-DRW6 in these patients were increased above normals. But the differences were not significant in the total group nor when the patients were separated according to the presence or absence of polymyalgia rheumatica.

Giant cell arteritis (temporal arteritis) presenting as fever of undetermined origin.

A retrospective study of the histories of 100 patients with biopsy-proven giant cell arteritis was performed. Fifteen of these patients had "fever of unknown origin" as the initial manifestation of this disease. All 15 had normal leukocyte counts; however, they had significantly lower hemoglobulin and albumin levels (P greater than 0.01) and significantly higher platelet counts, erythrocyte sedimentation rates, and alkaline phosphatase values (P congruent to 0.05) compared to the other 85 patients. In 4 patients, random temporal artery biopsies were performed despite persistently negative results from diagnostic evaluations and in the absence of any symptoms or findings suggestive of arteritis.