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.

Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature.

Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.