Microvascular remodeling after autologous stem cell transplant in an overlap of systemic sclerosis and systemic lupus erythematosus: A case report.

Microvascular remodeling and capillary repopulation can occur after autologous hematopoietic stem cell transplant (HSCT) in patients with systemic sclerosis and systemic lupus erythematosus (SLE). We aim to report evidence for microvascular remodeling after autologous HSCT as observed by nailfold videocapillaroscopy (NVC). We describe a rare occurrence of features consistent with systemic sclerosis and SLE in a 33-year-old female with a complex clinical course refractory to conventional treatments, ultimately requiring autologous HSCT. We performed NVC before and after HSCT using optical video and light microscopy. At the microvascular level, morphologic changes in the capillary vascular bed were observed after HSCT. Pretransplant damage in capillary structure was noted as evidenced on NVC with architectural loss, ramifications, capillary drop, and decreased density. Posttransplant NVC revealed an increase in capillary density with evidence of microvascular remodeling. Further studies on the clinical use and impact of microvascular remodeling on disease progression are needed and looking into the application of NVC scoring to assess clinical response would be meaningful.

Bilateral Lumbosacral Plexopathy As the Initial Manifestation of Systemic Sarcoidosis: A Case Report.

Neurosarcoidosis is one of the most relevant involvements in systemic sarcoidosis and can be the initial presentation. Its diagnosis is often considered difficult because of unusual clinical manifestations or diagnostic mimics. The peripheral nervous system is less frequently involved than the central nervous system, although it may also lead to irreversible neurologic impairment. Lumbosacral plexopathy in sarcoidosis is a rare presentation and has been scarcely described in anecdotal case reports and small case series. We describe the case of a 61-year-old female who presented with right inguinal pain, right thigh weakness, and gait limitation, with imaging evidence of bilateral lumbosacral plexopathy as the initial manifestation of systemic sarcoidosis and subsequently developed joint and pulmonary involvement. This case report aims to bring awareness of this involvement as a possible initial manifestation of systemic sarcoidosis and mention key features of the differential diagnosis. Prompt recognition and treatment may prevent neurologic impairment.

Submucosal Epstein-Barr Virus Positive Polymorphic B-cell Lymphoproliferative Disorder of the Larynx: A Case Report.

Epstein-Barr virus (EBV) belongs to the group of human herpes virus and can cause clinical and subclinical infections. Although EBV-related disease presentations are similar, they can lead to oncogenic transformation with various clinical manifestations. A thorough workup with morphology, immunohistochemistry, and molecular studies is crucial for the diagnosis of EBV-positive polymorphic B-cell lymphoproliferative disorder, not otherwise specified (NOS), which is a new entity introduced by International Consensus Classification in 2022. We describe an interesting presentation of EBV-positive polymorphic B-cell lymphoproliferative disorder with laryngeal involvement to bring awareness to this entity and we would like to address the need for more accessible treatment options.

Dermatomyositis in Association With SARS-CoV-2 Infection or COVID-19 Vaccine.

OBJECTIVE: New-onset and relapsed dermatomyositis (DM) has been reported following SARS-CoV-2 infection or COVID-19 vaccination. This study aims to show the characteristics of a DM cohort after COVID-19 infection and vaccination. METHODS: A retrospective review was performed on patients treated for DM between March 1, 2020, and October 31, 2022. Charts were evaluated for the presence of new-onset DM or relapse of preexisting DM following either SARS-CoV-2 infection or COVID-19 vaccination. Data on symptom onset, timing of vaccination, type of vaccination, and disease characteristics were collected. RESULTS: Ninety-eight patients treated for DM at our institution in the Division of Rheumatology were included. In total, 12 of 98 patients (12.2%) experienced DM symptoms (either incident or relapse) following either infection or vaccination. Of the 12 patients who developed incident disease or relapse, 7 (58.3%) developed postinfection symptoms, and 8 (66.7%) developed symptoms after vaccination (3 patients had symptoms following both infection and vaccination). The mean onset of symptoms following COVID-19 infection was 3.2 days (median 0.5 days), and mean onset following COVID-19 vaccination was 5.75 days (median 3.5 days). Nine of 12 patients (75%) had a positive myositis-specific antibody, and the remaining 3 (25%) had myositis-associated antibodies. There was no predominant vaccine associated with the development of postvaccination DM symptoms. CONCLUSION: This retrospective review revealed a strong temporal relationship between DM symptoms and COVID-19 infection or vaccination in 12.2% of all patients with DM evaluated in our clinic during the pandemic. Additional studies are required to understand the possible pathophysiology behind this association.

Diffuse alveolar hemorrhage secondary to sarcoidosis.

Diffuse alveolar hemorrhage (DAH) is a pulmonary condition that can be caused by autoimmune disorders such as lupus, small vessel vasculitis, and antiphospholipid syndrome. Sarcoidosis as a cause of DAH has been reported; however, the literature remains limited. We performed a chart review for patients with a diagnosis of both sarcoidosis and DAH. Seven patients met inclusion criteria. Mean (range) patient age was 54 years (39-72), and 3 patients had a history of tobacco use. Diagnosis of DAH and sarcoidosis were concurrent for 3 patients. Corticosteroids were used for treatment of DAH in all patients; 2 (including 1 with refractory DAH) were successfully treated with rituximab. We believe sarcoidosis-associated DAH is more common than previously reported. It is essential to consider sarcoidosis in the differential diagnosis of immune-mediated DAH. Key Points • Sarcoidosis can cause diffuse alveolar hemorrhage (DAH); more extensive studies are needed to estimate this condition's prevalence. • BMI of 25 or higher appears to be a risk factor for the development of sarcoidosis-associated DAH.

A study of temporal artery biopsy for the diagnosis of giant cell arteritis.

OBJECTIVE: This large, retrospective, multicenter study examines the Mayo Clinic experience with temporal artery biopsies over an 11-year period to help form guidelines that would lead to optimal performance of the technique. METHODS: Pathology records were identified from all patients at all three Mayo Clinic sites (Rochester, MN; Jacksonville, FL; and Scottsdale, AZ) who underwent temporal artery surgery over an 11-year period, from January 1, 1994, to December 31, 2004. From each report, we extracted demographic information, the length of the temporal artery removed in the biopsy from each side, and the pathologic findings in each specimen. We used logistic regression to assess whether biopsy positivity may be associated with the following factors: biopsy length, age, sex, type (unilateral vs. bilateral), and year of study. RESULTS: Our data set included 3817 temporal artery biopsies performed on 2539 patients at Mayo Clinic. Overall, 681 patients (27%) had a positive biopsy on at least one side. Biopsy length was uniformly noted to have no significant effect on biopsy positivity. Of the 603 patients with a bilateral biopsy, 43 (7%) had a negative initial biopsy followed by a positive result on the contralateral side. CONCLUSION: Our results support that one can recommend any length of biopsy, within the range our study, without affecting the rate of positivity. Furthermore, we conclude that if a unilateral biopsy is negative on frozen section, then we recommend a second biopsy on the contralateral side to avoid possible missed diagnoses. Key Points • Our paper is a large retrospective study over all 3 Mayo Clinic campuses investigating the association between temporal artery biopsy length and the positivity rate of biopsies. • We also determined the value of performing bilateral biopsies in which the first biopsy was negative on frozen section. • We believe the strength of our study is having one of the largest sample sizes of biopsies in the literature to date including 3817 biopsies. • The sum of our research supports that we found biopsy positivity is not a function of length and, second, bilateral biopsies diagnose up to 7% of disease not otherwise picked up by unilateral approaches.

Extra-articular involvement of rheumatoid arthritis in three seropositive patients in the absence of initial joint involvement.

INTRODUCTION: Extra-articular involvement (EAI) in rheumatoid arthritis (RA) is rare, severe and usually presents after years of joint involvement. Onset of RA as lung involvement has been published. We describe a series of three patients with strongly positive anti-citrullinated peptide antibodies (ACPA) and rheumatoid factor (RF) positive in the absence of joint symptoms, but with significant EAI. METHODS: This is a descriptive case series of three patients evaluated in an academic medical center rheumatology clinic. RESULTS: A 50-year-old female presented with severe recurrent scleritis in the background of strongly positive ACPA, but no joint involvement. Her ocular disease responded well to rituximab. Four years later, she developed peripheral inflammatory arthritis consistent with RA. A 74-year-old male presented after developing recurrent steroid-dependent serositis (pleuro-pericarditis) and interstitial lung disease (ILD). Serology was notable for strongly positive ACPA, but no joint involvement. Serositis responded well to adalimumab. Two years after initial symptoms, he developed peripheral joint involvement after holding adalimumab. A 56-year-old female presented with an isolated, biopsy-proven subcutaneous rheumatoid nodule. Subsequently, she developed pancytopenia and fatigue. She tested strongly positive for ACPA and RF. Bone marrow biopsy was negative for malignancy and she had no evidence of infection. She responded to steroids and hydroxychloroquine and had not developed joint involvement after 2 years of follow-up. CONCLUSION: EAI as an onset of RA is a complex and not easily recognized entity if typical joints involvement is not yet present. Early diagnosis may help guide specific therapy and prevent sequelae and co-morbidities.

Intensive Multicomponent Fibromyalgia Treatment: A Translational Study to Evaluate Effectiveness in Routine Care Delivery.

OBJECTIVE: The current study was designed to evaluate the translation of clinical trial outcomes and clinical guidelines for the treatment of fibromyalgia (FM) into an intensive multicomponent clinical program embedded in routine care delivery. The study aimed to assess the adaptation of these recommended strategies into routine clinical care while evaluating their effectiveness and durability in improving functional status and level of distress in a large clinical sample of FM patients. METHODS: Four hundred eighty-nine patients with FM completed a 2-day program that incorporated best practice recommendations for the treatment of FM. Patients completed the Fibromyalgia Impact Questionnaire-Revised, the Center for Epidemiologic Studies Depression Scale, and the Pain Catastrophizing Scale at admission to the program and at follow-up on average 5 months posttreatment. RESULTS: Significant improvements were seen in functional status (p < 0.0001), depressive symptoms (p < 0.0001), and pain catastrophizing (p < 0.0001) after participation in the intensive multicomponent treatment program. CONCLUSIONS: The present study shows that an intensive multicomponent treatment program embedded in routine care delivery is effective in significantly improving functional status and psychological distress in a large sample of FM patients. The significant improvements were durable and maintained at follow-up.

Factors associated with mortality and infections in patients with systemic lupus erythematosus with diffuse alveolar hemorrhage.

OBJECTIVE: To evaluate factors associated with mortality and infections in patients with systemic lupus erythematosus (SLE) and diffuse alveolar hemorrhage (DAH). METHODS: A retrospective chart review was carried out for medical admissions of patients with a diagnosis of SLE and DAH in 9 hospitals. Clinical and laboratory data were recorded for each patient at DAH diagnosis. RESULTS: We included 57 episodes of DAH of 50 patients (7 recurrences), 49 women (86%), 14 juvenile SLE (24.6%); 24 had died (42.1%). In the chart review we detected infection in 22 episodes (38.6%): 8 invasive fungal infections, 16 bacterial infections, and 2 patients had both types. In the bivariate analysis, factors associated with mortality were high Acute Physiology and Chronic Health Evaluation II scores, requirement of mechanical ventilation (OR 15.0, 95% CI 1.9 to 662.2), infections (fungal or bacterial; OR 3.2, CI 0.9 to 11.1), renal failure (OR 4.9, CI 1.4 to 18.0), and thrombocytopenia (OR 4.3, CI 1.2 to 15.6). We found similar mortality between children and adults. Infections were associated with treatment for SLE, requirement of mechanical ventilation, hypocomplementemia, and high levels of C-reactive protein. CONCLUSION: Infection is a frequent finding in patients with DAH and SLE; we found similar mortality between adult SLE and juvenile SLE. Factors that we describe associated with infections may influence the therapeutic selection for these patients.

Tocilizumab, an effective treatment for relapsing giant cell arteritis.

OBJECTIVES: Patients with giant cell arteritis (GCA) often respond to corticosteroid (CS) therapy; however, the majority of patients relapse when CS therapy is tapered or withdrawn. The purpose of this study was to assess the efficacy of tocilizumab (TCZ) in patients with relapsing GCA. METHODS: Four patients with relapsing GCA received TCZ monthly (4mg/kg or 8mg/kg). Disease activity and drug tolerability were evaluated clinically and via laboratory test results at the beginning of the study and every 3 months until the publication of this study. All four patients were still receiving TCZ monthly at the time of manuscript submission. RESULTS: All four patients treated with TCZ achieved clinical and laboratory response. No adverse events were detected. CONCLUSIONS: In our small case series, TCZ was efficacious and well tolerated in patients with relapsing GCA. Proper randomised controlled trials are required to achieve confident conclusions regarding the safety and efficacy of TCZ in GCA.