Case of calcinosis cutis associated with Sjogren's syndrome.

Calcinosis cutis is a chronic condition involving skin and soft tissue deposition of calcium and phosphate. It is associated with several conditions including idiopathic, iatrogenic, malignant metastasis, calciphylaxis, and connective tissue diseases. The most common connective tissue diseases it is associated with include systemic sclerosis and dermatomyositis. We present a case image of a patient with Sjogren's syndrome and calcinosis cutis and its progression over time. The patient was optimized on her current treatment regimen to prevent further progression. Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.

A case of knuckle pad syndrome in a middle-aged man.

Knuckle pads are benign papules, nodules, or plaques overlying joints and typically manifest at the proximal interphalangeal joints (PIPs). They may be confused with other dermatologic or rheumatologic diseases. Treatment options for primary knuckle pads are limited and acquired knuckle pads typically improve with withdrawal of the offending insult.

Pseudoseptic Arthritis in a Patient With Psoriasis.

A 42-year-old male with a history of untreated psoriasis and a previous episode of presumed left knee septic arthritis developed sudden onset of left knee pain, swelling, and a moderate effusion. The pathogen could not be isolated despite extensive inflammation seen in synovial fluid (SF) and synovial tissue biopsy. Whether this is culture-negative septic arthritis or pseudo-septic arthritis is the enigma, given the limited sensitivity of current available SF microbiologic testing. We present a challenging and stimulating case with no current guidelines for an optimal empiric antibiotic regimen or anti-inflammatory therapy.

Utilization of Rituximab for Refractory Rowell Syndrome.

Rowell syndrome describes the occurrence of erythema multiforme-like lesions in patients with cutaneous lesions of lupus erythematosus. The clinical picture of atypical erythema multiforme-like lesions, presence of chilblains, speckled ANA pattern, anti-Ro/SSA, or anti-La/SSB antibodies, and absence of infectious or pharmacologic triggers in a patient with systemic lupus erythematosus are some of the classic clinical and serologic features. Histopathologic and serologic findings can help differentiate this process from erythema multiforme. We present a case of young woman with systemic lupus erythematosus, end-stage renal disease due to lupus nephritis, and a remote history of Steven-Johnson syndrome due to sulfa allergy who presented to the hospital with a recurrent, progressive, targetoid erythematous rash involving more than 60% of her body surface area. Our patient had several hospitalizations in the recent past for this erythematous rash and had failed oral therapy with prednisone 1 mg/kg and hydroxychloroquine. In view of the minimal improvement and increasing severity and patient exhibiting early features of mast cell activation syndrome, the patient was treated with pulse intravenous glucocorticoids followed by rituximab with an excellent response. We highlight a unique case report of progressive Rowell syndrome refractory to standard of care with an excellent response to rituximab.

Diagnostic accuracy of salivary gland ultrasound in Sjögren's syndrome: A systematic review and meta-analysis.

BACKGROUND: To systematically review the diagnostic accuracy of salivary gland ultrasound in primary Sjögren's syndrome (pSS). METHODS: PubMed, Embase, CINAHL, Cochrane Central and Scopus and ClinicalTrials.gov were searched to identify diagnostic or validation studies in patients with pSS meeting the diagnostic criteria. A diagnostic test meta-analysis was performed using a bivariate model to calculate the pooled sensitivity, specificity, positive/negative likelihood ratios, and the diagnostic odds ratio. Meta-regression analyses were done for several pSS covariates. RESULTS: Sixty-five studies met our criteria for the qualitative review. Fifty-four studies with a total of 6087 patients were included in the meta-analysis. Pooled sensitivity for salivary gland ultrasound was 80% [95% confidence interval (CI): 77-83%; I 2 = 78%], and specificity was 90% (95% CI: 87-92%; I 2 = 76%). The pooled positive and negative likelihood ratios were 8 (95% CI: 6.4-10) and 0.22 (95% CI: 0.19-0.25), respectively. The corresponding pooled diagnostic odds ratio (DOR) was 37 (95% CI: 28-48). Separate meta-regression models resulted in similar diagnostic estimates: (a) adjusted for mean age: sensitivity 81% (95% CI:77-84%; I 2 = 99%) and specificity 90% (95% CI: 87-93%; I2 = 99%); (b) adjusted for mean disease duration, sensitivity 79% (95% CI:72-84%; I 2 = 99%), and specificity 90% (89-94%; I 2 = 99%). The diagnostic estimates were robust to sensitivity analyses by quality criteria, pSS diagnostic criteria and ultrasound scoring systems. CONCLUSION: Salivary gland ultrasound is a valuable modality for the diagnosis of Sjögren's syndrome. It is plausible that salivary gland ultrasound can be used as an important criterion for the diagnosis of pSS.

Pyoderma Gangrenosum as a Presenting Feature of Undifferentiated Spondyloarthropathy with Erosive Inflammatory Arthritis.

Pyoderma gangrenosum is a rare inflammatory condition with varying clinical presentations and severity. It is commonly seen in association with an underlying condition, most common of which is inflammatory bowel disease. We report a case of a 26-year-old male who came to the emergency department with increasing lower extremity ulcers, intermittent hematochezia, and pain in the small joints of his hands. After excluding a broad list of differentials for lower extremity ulcers, the diagnosis of pyoderma gangrenosum was made. He was also found to have erosive changes at multiple proximal interphalangeal joints and jug-like syndesmophytes at T12 and L1 on CT scan. Although there was evidence of a spondyloarthropathy, there was no evidence of inflammatory bowel disease on colonoscopy, psoriasis, or sexually transmitted infections. After multiple failed trials of medications including azathioprine and sulfasalazine, 4 weeks of Adalimumab resulted in rapid healing of pyoderma gangrenosum lesions and improvement in his synovitis. Coupled together, this suggests a diagnosis of pyoderma gangrenosum associated with undifferentiated spondyloarthropathy and erosive inflammatory arthritis. This case is suggestive of spondyloarthropathy going underdiagnosed and untreated in other patients with pyoderma gangrenosum as lower extremity ulcerations can be the primary complaint for seeking treatment. Although rare, axial spondyloarthropathy associated with pyoderma gangrenosum should be kept as an associated differential diagnosis when faced with pyoderma gangrenosum.

Metastatic ductal adenocarcinoma of the breast presenting with pericardial effusion-Challenges in the diagnosis of breast cancer.

Breast cancer is a common entity that can be difficult to diagnose. This case demonstrates the limitations of breast cancer diagnostics. Particularly, how the available imaging techniques and even biopsy can potentially miss a malignancy. It exemplifies the role immunohistochemistry staining plays in the diagnosis of cancers of unclear origin.

Effectiveness of healthcare educational and behavioral interventions to improve gout outcomes: a systematic review and meta-analysis.

BACKGROUND: We aimed to systematically review the effectiveness of healthcare behavioral and education interventions for gout patients on clinical outcomes. METHODS: We searched multiple databases to identify trials or observational studies of educational or behavioral interventions in gout. Risk of bias was assessed with the Cochrane tool for randomized control trials (RCTs) and the Newcastle-Ottawa Scale for observational studies. We estimated odds ratios (ORs) for categorical and standardized mean difference (SMD) for continuous measures using a random-effects model. RESULTS: Overall, eight (five RCTs and three observational) studies met the inclusion criteria and examined pharmacist-led interventions (n = 3), nurse-led interventions (n = 3) and primary care provider interventions (n = 2). Compared with the control intervention (usual care in most cases), a higher proportion of those in the educational/behavioral intervention arm achieved serum urate (SU) levels <6 mg/dl, 47.2% versus 23.8%, the OR was 4.86 [95% confidence interval (CI), 1.48, 15.97; 4 RCTs] with moderate quality evidence. Compared with the control intervention, a higher proportion of those in the educational/behavioral intervention arm were adherent to allopurinol, achieved at least a 2 mg/dl decrease in SU, achieved an SU < 5 mg/dl, had a reduction in the presence of tophi at 2 years, had improved quality of life as assessed with SF-36 physical component scores, had a higher knowledge about gout and higher patient satisfaction (moderate-low quality evidence). CONCLUSION: Educational and behavioral interventions can improve gout outcomes in the short-intermediate term. Randomized trials are needed to assess its impact on long-term gout outcomes.

Silicone-Induced Foreign Body Reaction: An Unusual Differential Diagnosis of Posterolateral Hip Pain.

BACKGROUND: Silicone injection is commonly used for soft tissue augmentation for esthetic purposes. It is not without complications. CASE PRESENTATION: We present a case of a 31-year-old woman presenting with refractory left lateral hip pain. Magnetic resonance imaging of the patient's pelvis revealed innumerable small low signal foci throughout the gluteus maximus and overlying subcutaneous fat bilaterally consistent with injectable material, possibly silicone. CONCLUSIONS: This case report emphasizes that silicone-induced granulomatosis must be considered in the differential diagnosis of hip pain when evaluating a patient who has had access to plastic surgery or clandestine operators.

Factors associated with calcium absorption in postmenopausal women: a post hoc analysis of dual-isotope studies.

Reduced calcium absorption is a risk factor for osteoporosis. This study examined factors associated with fractional calcium absorption (FCA) and net calcium absorption in postmenopausal women in a post hoc analysis of three completed dual-isotope studies. Data were analyzed from 50 postmenopausal women undergoing 121 inpatient research visits in three studies evaluating changes in FCA related to correction of vitamin D insufficiency (n=19), use of proton pump inhibitors (n=21), and use of aromatase inhibitors to treat breast cancer (n=10). Net calcium absorption was the product of FCA and total calcium intake in milligrams per day. Variables included subjects' age, race, body mass index, serum calcium, creatinine, parathyroid hormone, 1,25-dihydroxyvitamin D, 25-hydroxyvitamin D, and habitual intake of kilocalories, protein, fat, carbohydrate, fiber, calcium, iron, magnesium, oxalate, phosphorus, potassium, and vitamin D based on outpatient diet diaries. In multivariate models, subjects' age, dietary intake of kilocalories, carbohydrates, fat, fiber, calcium, and potassium were significant predictors of FCA. In multiple variable models predicting net calcium absorption, dietary intake of kilocalories, fat, fiber, calcium, potassium, and serum 1,25-dihydroxyvitamin D were significant. The square of the correlation between actual and predicted values (an approximation of R(2)) was 0.748 for FCA and 0.726 for net calcium absorption. Similar to other studies, this study found that age, 1,25-dihydroxyvitamin D, and dietary calcium and fat were associated with calcium absorption. Dietary intake of kilocalories, carbohydrates, and potassium were new factors that were significantly associated with FCA and net calcium absorption. In summary, the study suggests that several dietary habits play a role in calcium absorption, beyond vitamin D and calcium.

Do HbA1C Levels Correlate With Delayed Gastric Emptying in Diabetic Patients?

BACKGROUND/AIMS: Gastroparesis is characterized by delayed gastric emptying without obstruction. Diabetes is frequently associated with poor glycemic control and delayed gastric emptying. Gastric emptying scintigraphy (GES) is the standard for measuring gastric emptying. Serum hemoglobin A1C (HbA1C) measures prolonged glycemic control with normal as < 7% glycated. To date, no correlation of serum HbA1C level with gastric emptying, demonstrated by GES, in diabetics has been performed. The aim of the present investigation is to determine if a relationship exists between serum HbA1C levels and gastric emptying, assessed by GES, in diabetics. METHODS: All diabetics, having both GES and serum HbA1C level within 3 months from July 1, 2003 - June 30, 2008 were eligible for study. Demographic data collected included gender, age and ethnicity. Abnormal gastric emptying was defined as T½ > 120 minutes and serum HbA1C as percent glycated. RESULTS: Nuclear Medicine GES database review revealed 431 examinations performed during the study interval. A total of 181 were not eligible due to the following: 29 duplicates, 22 diabetes not documented and 130 without HbA1C levels, resulting a study group of 250 cases. No significant correlation was observed between gastric emptying time, HbA1C or age. Among patients with HbA1C ≥ 7%, HbA1C was inversely related to age with a coefficient of correlation of r = -0.175 (p = 0.038). CONCLUSIONS: There is no correlation observed between gastric emptying time, using GES, and serum HbA1C levels. In diabetics, serum HbA1C is not as important as daily glycemic control regarding gastric emptying.