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We describe the first case of bilateral hypothenar hammer syndrome (HHS) followed by systemic sclerosis (SSc) that was associated with silica exposure (Erasmus syndrome). The patient was a woman smoothing tiles in an earthenware factory who presented with bilateral digital ischemia associated with Raynaud's phenomenon. HHS was diagnosed, based on an angiographic study showing aneurysm of the ulnar arteries and occlusions of multiple digital arteries. Pulmonary silicosis was also diagnosed on pulmonary tomodensitometry. Two years later digital swelling with acroosteolysis developed. The FANA test was positive (titer 1:640) and anticentromere antibody tests were also positive. Esophageal manometry showed dysmotility of the lower esophagus. These findings were consistent with a diagnosis of SSc.

Esophageal involvement is considered a major cause of morbidity and an indicator of poor prognosis in polymyositis (PM) and dermatomyositis (DM). We describe 3 patients with steroid resistant PM/DM with life threatening esophageal involvement, resulting in impossible oral feeding and enteral nutrition with a gastric tube. All patients had both dramatic and rapid improvement of all clinical manifestations after initiation of intravenous immunoglobulin (IVIG) therapy. Swallowing disorders completely disappeared after the second infusion of IVIG, which permitted normal oral feeding and ablation of the gastric tube. Our findings suggest IVIG should be considered the treatment of choice in such cases.

Objective: To assess prevalence, characteristics, and predictive factors of pulmonary involvement in polymyositis (PM) and dermatomyositis (DM)., Methods: The medical records of 55 consecutive patients with PM and DM between 1983 and 1996 were reviewed. The criteria for diagnosis of PM and DM were based upon Bohan and Peter criteria., Results: Twenty-two patients (40%) developed lung impairment with a mean of 17 months after onset of PM and DM. The 3 main types of pulmonary disorders were interstitial lung disease (41%), ventilatory insufficiency with bronchopneumonia (22.8%), and alveolitis (with pulmonary function tests revealing restrictive pattern, low diffusing capacity, and normal computerized tomography) (36.2%). Both morbidity and mortality rates were as high as 40 and 27%, respectively, in PM and DM patients with lung involvement. Moreover, for the group with pulmonary diseases, serum GOT and ferritin levels were higher (p = 0.01 and p = 0.02) and the presence of anti-Jo-1 antibody and characteristic microangiopathy were more frequent (p < 0.05 and p = 0.0002)., Conclusion: This series reveals both high prevalence (40%) and severity of pulmonary involvement in PM and DM. Our findings also suggest that high serum GOT and ferritin levels, presence of anti-Jo-1 antibody and characteristic microangiopathy may have predictive value and should encourage the search for pulmonary dysfunction and interstitial lung disease in patients with PM and DM.

We describe a case of Takayasu's arteritis discovered at the early systemic phase. Ultrasonography and computed tomography show thickening of the walls of the superior mesentery and common carotid arteries despite normal findings on angiography. Diagnosis was confirmed by arterial biopsy. We emphasize the importance of noninvasive vascular investigation to support the diagnosis of Takayasu's arteritis.

We describe 4 cases of focal myositis of the lower leg in which magnetic resonance imaging (MRI) was useful and sensitive in clearly revealing the detailed anatomic extent of muscle change and in guiding muscle biopsy. We also observed a correlation between MRI findings and severity of both clinical and histological manifestations, suggesting that MRI may provide prognostic criteria in focal myositis. MRI may be a helpful, noninvasive test for the followup of focal myositis, in assessing disease progression or resolution.

Objective: Histological analysis of giant cell arteritis (GCA) reveals a granulomatous reaction around the internal elastic lamina. Elastolysis by multinucleated giant cells has also been reported. We investigated elastin derived peptides as putative recall antigens for peripheral blood mononuclear cells (PBMC) from patients with GCA., Methods: PBMC were collected from 17 patients with GCA (Group 1), 17 patients with vascular diseases, connective tissue diseases, or polymyalgia rheumatica without GCA (Group 2), and 17 healthy controls (Group 3). Cultures of PBMC with different elastin derived peptides or elastase were analyzed., Results: A proliferative response was obtained only with elastate derived elastin peptides in 12/13 untreated patients with GCA. Steroid treatment was believed to abolish this proliferative response in 4 patients with GCA. PBMC from only 3/34 non-GCA subjects responded to these antigens. No proliferative response was obtained for other elastin derived peptides or elastase in any subject., Conclusion: Degradation of native elastin by leukocyte elastase can provide elastin derived peptides that act as autoimmune targets for T cells in GCA.

We describe a patient with polyarteritis nodosa who developed a large retroperitoneal hemorrhage from a ruptured suprarenal capsule artery aneurysm. Platinum coils were embolized selectively during arteriography and successfully controlled the aneurysmal bleeding. The vasculitis was controlled ten weeks later but our patient developed anemia and computerized tomography scan revealed a large retrogastric hemorrhage. A further arteriogram confirmed the presence of a ruptured gastric coronary artery aneurysm. A second embolization was performed with complete success. Selective embolization of small arteries with coils should be considered a good alternative to surgery in patients with polyarteritis nodosa and hemorrhage from a ruptured aneurysm; arteriography may be considered a potent diagnostic and therapeutic tool in the management of the treatment of polyarteritis nodosa.