Esophageal dysmotility and lung disease in patients with systemic sclerosis: is there a possible association or correlation? A retrospective chart review.

OBJECTIVE: Our aim was to evaluate the relationship between esophageal dysmotility and lung disease by correlating the results of Echocardiogram and Pulmonary Function Test (PFT) with Esophageal Transit Study (ETT). METHODS: Charts of Systemic Sclerosis (SSc) patients fulfilling 2013 ACR/EULAR classification criteria seen in Rheumatology clinics were reviewed and their demographics, ETT result, PFT, and echocardiogram data were collected at baseline, years 1, 3, 5, and 10. Patients were divided based on their ETT status and were compared with respect to each variable using a two-sided two-sample t test for continuous variables and a Fisher's exact test for categorical variables. RESULTS: 130 patients were identified with either limited cutaneous SSc (109) or diffuse cutaneous SSc (21) with a mean age of 52.65 years. The mean DLCO was statistically worse in abnormal ETT patients [p value = 0.0004] as were the progression rates per year for DLCO at - 2.25 (p value = 0.019). Progression rate of FVC per year was statistically significant in the abnormal ETT group, although the mean value was not. The number of patients with abnormal PASP was not statistically different between the two groups (p values 0.104, 0.178, 0.653 at baseline, years 3 and 5, respectively). CONCLUSION: The presence of esophageal dysmotility was associated with increased pulmonary involvement in the form of abnormal DLCO with worsening progression rates per year. There was no statistically significant difference in PASP and FVC between the two groups; however, the progression rate for FVC was worse in adjusted models.

Systemic Vasculitis Associated With Immune Check Point Inhibition: Analysis and Review.

PURPOSE OF REVIEW: Immunotherapy with immune checkpoint inhibitors (ICIs) has become a well-established modality to treat a number of different malignancies, especially in cases with advanced stages and/or recurrent diseases. These agents have been associated with development of a variety of autoimmune disorders as immune-related adverse events (IRAEs or irAEs). This review focuses on development of vasculitis with use of ICI. RECENT FINDINGS: Available information on vasculitis associated with immune checkpoint inhibition is limited primarily to case reports at this time. Most immune-related adverse events will not present as vasculitis, and it is an uncommon manifestation and/or is under-reported. There are no current well-established guidelines for treating vasculitis associated with ICIs; initial management would usually start with consideration of discontinuing the ICI and administering corticosteroids. Collaboration between treating oncologists and rheumatologists is necessary for a combined approach to management. While arthralgias, myalgias, and inflammatory arthritis frequently occur as irAEs, vasculitis is an uncommon presentation. Vasculitis has been reported with all of the available ICI agents, and there seems to be no clear difference in the risk based on small numbers. Large vessel vasculitis and vasculitis of the nervous system were the most commonly reported types of vasculitis but cases of vasculitis involving medium and small vessels have also been reported. It is challenging to know if the underlying disease or ICIs are the main culprit in development of vasculitis and requires a collaborative relationship between the treating oncologist and rheumatologist. Except in very mild cases, development of vasculitis during ICI therapy requires temporary or permanent discontinuation of ICI.

Rheumatoid Vasculitis: A Diminishing Yet Devastating Menace.

PURPOSE OF REVIEW: Rheumatoid vasculitis (RV) is an unusual complication of long-standing rheumatoid arthritis, which is characterized by the development of necrotizing or leukocytoclastic vasculitis involving small or medium-sized vessels. In this review, we aim to provide an update on the epidemiology, pathogenesis, clinical presentation, and management of this challenging extra-articular manifestation. RECENT FINDINGS: RV is heterogenous in its clinical presentation depending on the organ and size of blood vessels involved. The most common organs involved are the skin and peripheral nerve. Based on recent population studies, the incidence has significantly decreased with early recognition and the advent of immunosuppressive drugs and biologics; however, the mortality rates remain high. RV remains a serious extra-articular manifestation of RA that needs to be promptly recognized and treated. No consensus is available on treatment, given the ongoing debate of whether the biologics can trigger or treat RV.

Obstetric outcomes in women with rheumatoid arthritis: Results from Nationwide Inpatient Sample Database 2003-2011✰.

OBJECTIVE: Fertility is reduced in patients with Rheumatoid Arthritis due to unknown cause. Few studies have addressed pregnancy outcomes in RA. This study was undertaken to determine the frequency of complications occurring during pregnancy for women with RA and compare with the general obstetric population by using the largest inpatient care database. METHODS: By using the 2003-2011 Nationwide Inpatient Sample of Healthcare Cost and Utilization Project, we estimated the number of obstetric hospitalization in women with RA between the age group 18-50 years. Demographic characteristics and in-hospital obstetric complications for all pregnancy-related admissions for women with and without RA were compared. Multivariate logistic regression analysis was used to obtain adjusted odds ratio. RESULTS: The total number of obstetric hospitalization was 42.32 million of which 31,439 were women with RA. The maternal age of RA population was higher (30.5 years) than that in the control group (27 years). After adjusting for potential confounders, maternal RA population had a significantly higher prevalence of hypertensive diseases, premature rupture of membranes, antepartum hemorrhage, preterm delivery, intrauterine growth retardation and cesarean delivery. The prevalence of postpartum hemorrhage and the risk of inpatient mortality were not different between two groups. CONCLUSION: Women with RA have a higher risk of adverse outcomes of pregnancy and thus close antenatal and post-delivery monitoring need to be performed in order to reduce complications. Further studies are needed to examine these findings in relation to severity of disease, medications used and the presence of other comorbidities.

Systemic Lupus Erythematosus Is Associated With a High Risk of Venous Thromboembolism in Hospitalized Patients Leading to Poor Outcomes and a Higher Cost: Results From Nationwide Inpatient Sample Database 2003-2011.

OBJECTIVE: Venous thromboembolism (VTE) is a major cause of mortality and morbidity in hospitalized patients, particularly those with autoimmune disorders. The Nationwide Inpatient Sample (NIS) database was analyzed to determine trends in the rate of hospitalization, mortality from VTE, epidemiology, and outcomes in hospitalized patients with systemic lupus erythematosus (SLE) to assess its impact. METHODS: The 2003-2011 NIS database of the Healthcare Cost and Utilization Project was queried to identify all adults (age 18 years and older) hospitalized with SLE and VTE. Demographic characteristics and in-hospital outcomes of this population were compared with those of patients with SLE without a VTE diagnosis. A multivariate logistic regression analysis was used to obtain the adjusted odds ratio (OR). RESULTS: The total number of hospitalized patients with SLE was 299 595, of whom 9175 (3.06%) had VTE. After adjusting for potential confounders, compared with those without VTE, patients with SLE and VTE had significantly higher inpatient mortality (5% vs. 2.0%; OR 2.35 [95% confidence interval (CI) 2.10-2.62]; P < 0.001), greater disability at discharge (34% vs. 26%; OR 1.53 [95% CI 1.46-1.62]; P < 0.001), a longer length of stay (LOS) by 3.57 days, and higher cost of hospitalization by $25 400. In this database, patients with SLE and VTE were younger and of male sex. Also, African American race and a higher number of comorbidities were associated with an increased risk of VTE in patients with SLE. CONCLUSION: VTE in hospitalized patients with SLE is associated with significantly higher inpatient mortality, greater disability at discharge, an increased LOS, and higher cost of hospitalization. This cross-sectional study helps with quantifying the risk of VTE in hospitalized patients with SLE and provides information on the immense human and material cost this complication leads to. These data can be very useful in the development and implementation of appropriate prophylactic strategies in the high-risk population with SLE.

Rheumatoid meningitis: successful remission with rituximab.

A 53-year-old male with rheumatoid arthritis presented with recurrent headaches, seizures and right-sided lower extremity paralysis while on antiepileptic medications. Work up revealed pachymeningeal and leptomeningeal enhancement on brain MRI. Differential diagnosis included a variety of infections, neoplasm and vasculitis. Histopathology showed findings consistent with rheumatoid meningitis (RM). Ultimately based on symptoms, MRI findings and tissue pathology, he was diagnosed with RM. Intravenous pulse dose steroids were initiated followed by rituximab every 6 months, resulting in significant improvement of the brain MRI findings. Patient has remained seizure free.

When a wound is the harbinger of a serious underlying systemic illness.

A 55-year-old woman presented with progressive enlarging and painful non-healing ulcers on her bilateral lower extremities; biopsy was consistent with pyoderma gangrenosum. Workup for an underlying illness revealed a cavitary lung nodule and an ulcerating mass in the anal canal. Patient did not have any respiratory or gastrointestinal symptoms. Differential diagnosis included inflammatory bowel disease, rectal carcinoma or infection such as tuberculosis, fungal process. Histopathology did not reveal any malignancy, inflammatory bowel disease or infection. Serological studies were positive for perinuclear antineutrophil antibodies specific to proteinase-3 antigen, and the patient was ultimately diagnosed with granulomatosis with polyangiitis. Intravenous pulse dose steroids were initiated followed by monthly pulse cyclophosphamide for 6 months, resulting in rapid and significant improvement of the wounds.

A Brain Ring-Enhancing Lesion.

Neuropsychiatric systemic lupus erythematosus (NPSLE) presents a diagnostic challenge as there is no unified pathophysiological process driving its presentation. Case reports are limited in detailing manifestations and outcomes of NPSLE. This case highlights a unique presentation of NPSLE and discusses challenges associated with diagnosis. A 27-year-old man with systemic lupus erythematosus presented with altered mentation. Initial laboratory results and computed tomography of the brain were unremarkable, but magnetic resonance imaging of the brain revealed ring-enhancing lesions reported as NCC. This led to an extensive infectious disease evaluation, but ultimately there was no evidence of infection. The patient was diagnosed with NPSLE; treatment with intravenous glucocorticoids and cyclophosphamide led to dramatic clinical improvement. Repeat brain magnetic resonance imaging showed resolution of the ringed lesions. This case illustrates the importance of thorough evaluation in immunocompromised patients and warns of the risk of anchoring bias that can lead to diagnostic delays.