A Novel Treatment for Skin Manifestations in Dermatomyositis: A Case Report.

We present a case of refractory cutaneous dermatomyositis (DM) in a 51-year-old Hispanic female which failed multiple treatments but found symptomatic relief with anifrolumab. Anifrolumab was the only treatment that was associated with significant improvement in the rash and pruritis of the patient and lowered her corticosteroid needs. To our knowledge, this is the only second case report that has shown success in treating refractory cutaneous symptoms of DM with anifrolumab after failing standard and multiple combinations of therapies. Anifrolumab is a new first-in-class human monoclonal antibody, which inhibits type 1 interferon receptor (IFN-1) and is used to treat systemic lupus erythematosus (SLE). It is FDA-approved for non-renal manifestations of SLE. This IFN pathway seems to be also active in patients with DM. The presence of IFN-1 and IFN-2 has been reported in muscle biopsies of patients with inflammatory myopathies. Moreover, the IFN activation signature is present in the muscle, blood, and skin of patients with DM. IFN-1 has been assumed to activate toll-like receptors which activate the dendritic cells leading to the secretion of cytokines and chemokines. This potential pathophysiological role of IFN in DM may explain the symptom improvement experienced by our patient after starting anifrolumab treatment. Anifrolumab has additionally been shown to have a good safety profile when used to treat patients with SLE with up to three years of treatment on background conventional disease-modifying antirheumatic drug (DMARD) therapies. In conclusion, SLE and DM share similarities in their pathophysiology and cutaneous disease involvement and can be differentiated clinically. Skin manifestations of DM can persist despite combinations of therapies even when weakness resolves. With this case report, we aim to highlight the possibility of utilizing anifrolumab for treating DM skin manifestations, especially in refractory cases. More research is needed to guide where anifrolumab stands in the therapeutic algorithm for DM. It is unknown whether it treats the myositis component, DM-related arthritis, or coexistent rheumatoid arthritis.

Colon Perforation As Initial Presentation of Refractory and Complicated Sclerosing Mesenteritis.

Sclerosing mesenteritis (SM), a benign chronic fibrosing inflammatory disease of the mesentery, is a rare disease discovered in 1924. The prevalence of the disease is less than 1%. The exact etiology of the disease is not clear. It is thought that the integrity of the gastrointestinal lumen may be altered from chronic inflammatory effects. SM may be associated with autoimmune diseases, trauma, malignancy, or surgery. The most common clinical presentation is abdominal pain. Obstructive symptoms may occur. Diagnosis is made by CT abdomen and biopsy. Treatment includes surgical and immunosuppressive medications.

A Rare Case of Central Nervous System Vasculitis in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibodies-associated Interstitial Lung Disease.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing inflammation of the small vessels. Central nervous system (CNS) ANCA-associated vasculitis is a rare manifestation of AAV. Three mechanisms of AAV affecting the CNS have been reported which include contiguous granulomatous invasion from nasal and paranasal sinuses, remote granulomatous lesions, and vasculitis of small vessels. Chronic hypertrophic pachymeningitis (CHP) is the meningeal-site involvement in AAV caused by granulomatous inflammation in the dura mater. We present a case of pachymeningitis manifested with slowly progressive cognitive dysfunction, leptomeningeal enhancement on MRI, and necrotic vessels with surrounding inflammation on biopsy. This case represents a rare development of subsequent CNS AAV in a patient with ANCA-associated interstitial lung disease treated with rituximab with a resolution of leptomeningeal enhancement on a follow-up magnetic resonance imaging (MRI).

Dermatomyositis-Induced Rhabdomyolysis With Features of Necrotizing Myopathy and Acute Inflammatory Demyelinating Polyneuropathy in an Epstein-Barr Virus Infected Patient.

Dermatomyositis (DM) is an autoimmune inflammatory myopathy characterized by features of a typical rash, proximal muscle weakness, and evidence of muscle inflammation. Acute inflammatory demyelinating polyneuropathy (AIDP) is an autoimmune peripheral nerve disease characterized by myelin damage and progressive areflexic weakness and sensory changes. AIDP can be precipitated by viral infections such as Epstein-Barr virus (EBV). We present a case of DM with rhabdomyolysis and necrotizing features, along with AIDP in the setting of EBV viremia. DM and AIDP rarely coincide together. The patient was treated with a combination therapy of methylprednisolone, azathioprine, and intravenous immunoglobulins (IVIGs), which led to significant improvement in his symptoms.

Cervical CT-Dependent Diagnosis of Crowned Dens Syndrome in Calcium Pyrophosphate Dihydrate Crystal Deposition Disease.

PURPOSE: The purpose of this study is to assess the presence of crowned dens syndrome in patients with calcium pyrophosphate disease. We report 34 patients with crowned dens syndrome in one of the largest series from a single tertiary medical center in North America. METHODS: A retrospective chart review was conducted at the University of Kansas Medical Center from November 1, 2005-November 1, 2017. A total of 191 patients with calcium pyrophosphate disease were identified. The available cervical computed tomography scans were analyzed by a musculoskeletal radiologist for the presence of periodontoid calcifications and erosions. RESULTS: Of the 191 patients with calcium pyrophosphate disease, 57 had cervical computed tomography scans; 34 of them (34/57, 59.64%) had periodontoid calcifications. Only 12/34 patients were formally evaluated and diagnosed by rheumatologists with crowned dens syndrome. Twenty-two of 34 were either not seen by a rheumatologist or were not diagnosed with crowned dens syndrome. The median age was 78.5 years, with 73.52% over 70 years old; 24/34 (70.58%) were female; 17/34 patients (50%) were symptomatic; 28/34 (82.35%) had additional sites of chondrocalcinosis on available radiographs; 8 (28.57%) had 3 or more sites of chondrocalcinosis in typical calcium pyrophosphate disease locations. Six patients did not have any radiographs. CONCLUSION: Crowned dens syndrome is an under-recognized entity that should be considered in elderly patients with neck pain in the setting of calcium pyrophosphate disease. Our data demonstrated a high percentage (about 60%) of patients with calcium pyrophosphate disease who had cervical computed tomography findings consistent with crowned dens syndrome. This underscores the importance of performing cervical computed tomography when evaluating patients with neck pain and calcium pyrophosphate disease.

Nivolumab-induced new-onset seronegative rheumatoid arthritis in a patient with advanced metastatic melanoma: A case report and literature review.

Immune-related adverse events have been reported in patients treated with anti-programmed death-1 receptor drugs such as nivolumab. We present a case of a new-onset seronegative rheumatoid arthritis in a patient with metastatic melanoma treated with nivolumab.

Vancomycin and the Risk of AKI: A Systematic Review and Meta-Analysis.

BACKGROUND AND OBJECTIVES: Vancomycin has been in use for more than half a century, but whether it is truly nephrotoxic and to what extent are still highly controversial. The objective of this study was to determine the risk of AKI attributable to intravenous vancomycin. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a systematic review of randomized, controlled trials and cohort studies that compared patients treated with intravenous vancomycin with a control group of patients given a comparator nonglycopeptide antibiotic and in which kidney function or kidney injury outcomes were reported. PubMed and Cochrane Library were searched from 1990 to September of 2015. Two reviewers extracted data and assessed study risk of bias, and one reviewer adjudicated the assessments. A meta-analysis was conducted on seven randomized, controlled trials (total of 4033 patients). RESULTS: Moderate quality evidence suggested that vancomycin treatment is associated with a higher risk of AKI, with a relative risk of 2.45 (95% confidence interval, 1.69 to 3.55). The risk of kidney injury was similar in patients treated for skin and soft tissue infections compared with those treated for nosocomial pneumonia and other complicated infections. There was an uncertain risk of reporting bias, because kidney function was not a prespecified outcome in any of the trials. The preponderance of evidence was judged to be indirect, because the majority of studies compared vancomycin specifically with linezolid. CONCLUSIONS: Our findings suggest that there is a measurable risk of AKI associated with vancomycin, but the strength of the evidence is moderate. A randomized, controlled trial designed to study kidney function as an outcome would be needed to draw unequivocal conclusions.