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We report the unusual case of a 31-year-old woman referred for inflammatory pain of the long bones associated with diffuse focal osteolysis, elevated erythrocyte sedimentation rate (ESR) and hypercalcemia. Corticosteroid therapy produced dramatic symptomatic improvement, and biological parameters returned to normal. Biochemical markers of bone remodeling, particularly those of resorption, were strikingly elevated. Parathyroid hormone, parathyroid hormone-related protein, nephrogenous cyclic adenosine monophosphate and calcitriol levels were normal. Investigations for infectious disease and malignancy were negative. The histology of the lesions consisted of cortical osteolysis caused by intense osteoclastic resorption, with several foci of osteogenesis, without malignant cells. The patient died 9 months after the onset of symptoms, following a "malignant" course: severe hypercalcemia, extension of lytic bone lesions and spontaneous fractures. To our knowledge, such a peculiar picture with quite unusual radiologic findings, disseminated focal cortical lesions and a progressive course, without evidence for a tumoral process, has never been described. This case suggests either the presence of an unknown systemic osteoclast-activating factor from an occult tumor or a primary malignant hyperosteoclastosis.

A 49-yr-old male was admitted to the Pulmonology Unit of Bichat Hospital (Paris, France) in February 2001 due to minor haemoptysis. He had a history of right upper lobe tuberculosis in 1970, complicated with secondary aspergilloma in 1999 and treated with itraconazole between April and October 1999. In November 1999, he presented with right pleural empyema (local samples yielded Pseudomonas aeruginosa and Streptococcus sanguis ) due to a bronchopleural fistula (fig. 1a⇓). Right open thoracostomy was performed in December 1999 and daily local care provided for 14 months until admission. On questioning, he denied experiencing fever, weight loss and shortness of breath. Fig. 1.— Chest radiographs on a) diagnosis of empyema and before thoracostomy (November 1999) and b) admission (January 2001). Physical examination results were normal. The pleural cavity was clean and sterile and the bronchopleural fistula still open. Laboratory results, including white blood cell count and erythrocyte sedimentation rate, were normal. The chest radiograph is shown in figure 1b⇑. Computed tomography was also performed (fig. 2⇓). Fig. 2.— Computed …

OBJECTIVE: To assess the recently proposed preliminary criteria for the classification of Sjogren9s syndrome (SS) in a multicentre European study of a new series of clinically defined cases. METHODS: The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, five with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. RESULTS: The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. CONCLUSION: This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.

Objective-To assess the recently proposed preliminary criteria for the classification of Sjogren’s syndrome (SS) in a multicentre European study of a new series of clinically defined cases. Methods-The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, dive with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. Results-The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (Limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. Conclusion-This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.