Identifying Clinicoradiological Phenotypes in Diffuse Idiopathic Skeletal Hyperostosis: A Cross-Sectional Study.

Background and objectives: Diffuse idiopathic skeletal hyperostosis (DISH) is a bone formation disease in which only skeletal signs are considered in classification criteria. The aim of the study was to describe different phenotypes in DISH patients based on clinicoradiological features. Materials and Methods: We evaluated 97 patients who met the Resnick or modified Utsinger classification criteria for DISH and were diagnosed at our hospital from 2004 to 2015. Patients were stratified into: (a) peripheral pattern (PP)-Resnick criteria not met but presenting ≥3 peripheral enthesopathies; (b) axial pattern (AP)-Resnick criteria met but <3 enthesopathies; and (c) mixed pattern (MP)-Resnick criteria met with ≥3 enthesopathies. Statistical analysis was carried out to identify variables that might predict classification in a given group. Results: Fifty-six of the 97 patients included (57.7%) were male and 72.2% fulfilled the Resnick criteria. Applying our classification, 39.7% were stratified as MP, 30.9% as AP and 29.4% as PP. Clinical enthesopathy was reported in 40.2% of patients during the course of the disease. Sixty-eight patients were included in a comparative analysis of variables between DISH patterns. The results showed a predominance of women (p < 0.004), early onset (p < 0.03), hip involvement (p < 0.003) and enthesitis (p < 0.001) as hallmarks of PP. Asymptomatic patients were most frequently observed in AP (28.6%, MP 3.8%, PP 5.0%) while MP was characterized by a more extensive disease. Conclusions: We believe DISH has distinct phenotypes and describe a PP phenotype that is not usually considered. Extravertebral manifestations should be included in the new classification criteria in order to cover the entire spectrum of the disease.

Hiperostosis esquelética difusa idiopática: ¿existe un patrón de predominio periférico? / Diffuse idiopathic skeletal hyperostosis: can we identify a peripheral pattern?

La hiperostosis esquelética difusa idiopática es una enfermedad caracterizada por la osificación de ligamentos vertebrales y de entesis periféricas. Actualmente, siguen utilizándose los criterios clasificatorios clásicos que se basan en hallazgos radiológicos vertebrales, pero que no incluyen otras manifestaciones clinicopatológicas que conforman el espectro de la enfermedad. Diversos autores abogan por la revisión y actualización de dichos criterios. A continuación describimos 3 casos clínicos en los que no se podría establecer un diagnóstico de hiperostosis esquelética difusa idiopática pero que presentan unas manifestaciones clínico-radiológicas totalmente compatibles con la enfermedad hiperostosante

Diffuse idiopathic skeletal hyperostosis is a bone disease characterized by ossification of spinal ligaments and peripheral entheses. Currently, we still use the classic classification criteria that do not include extraspinal manifestations. A number of authors agree on the need to revise them. We present 3 patients in which a diagnosis of diffuse idiopathic skeletal hyperostosis could not be established if we apply those criteria, but they have fully compatible clinical and radiological manifestations of hyperostotic disease

Diffuse idiopathic skeletal hyperostosis: Can we identify a peripheral pattern? / Hiperostosis esquelética difusa idiopática: ¿existe un patrón de predominio periférico?

Diffuse idiopathic skeletal hyperostosis is a bone disease characterized by ossification of spinal ligaments and peripheral entheses. Currently, we still use the classic classification criteria that do not include extraspinal manifestations. A number of authors agree on the need to revise them. We present 3 patients in which a diagnosis of diffuse idiopathic skeletal hyperostosis could not be established if we apply those criteria, but they have fully compatible clinical and radiological manifestations of hyperostotic disease.

Nailfold capillaroscopic findings in primary Sjögren's syndrome with and without Raynaud's phenomenon and/or positive anti-SSA/Ro and anti-SSB/La antibodies.

The aim of this study was to assess nailfold capillaroscopic (NC) findings in patients with primary Sjögren's syndrome (PSS) with and without Raynaud's phenomenon (RP) as well as in the presence of positive anti-SSA/Ro and anti-SSB/La antibodies. Videocapillaroscopy was performed in 150 patients with PSS. Data collected included demographics, presence of RP, PSS symptoms, antinuclear antibodies, rheumatoid factor, anti-Ro, anti-La, anti-CCP, salivary scintigraphy, labial biopsy, and NC findings. RP was present in 32% of PSS, keratoconjunctivitis sicca in 91%, oral xerosis in 93%, and skin or genital xerosis in 53%. In patients with positive anti-SSA/Ro (75%) and positive anti-SSB/La (40%), NC showed normal findings in 53% of cases and non-specific in 36%. In patients with PSS, NC was normal in 51% of cases and non-specific in 34%. Scleroderma pattern was found in 14 patients. RP associated with PSS had non-specific capillaroscopy in 40% of cases (p = 0.1). Pericapillary haemorrhages (p = 0.06) and capillary thrombosis (p = 0.2) were not increased, but more dilated capillaries were detected in 48% of cases. Patients with positive anti-Ro and/or anti-La have not a distinct NC profile. Patients with RP associated with PSS had more dilated capillaries, but neither pericapillary haemorrhages nor capillary thrombosis was observed.