Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition. Its common manifestations include type I autoimmune pancreatitis and retroperitoneal fibrosis (RPF). We present a rare case involving a 43-year-old female who presented with left lower quadrant (LLQ) pain. Imaging of the abdomen and pelvis revealed left hydroureteronephrosis to the level of an inflammatory process in the left adnexal region, possibly reflecting a tubo-ovarian abscess (TOA). The gynecologic evaluation concluded that the mass was unlikely of gynecologic sources. Transgluteal biopsy of the mass was highly suggestive of IgG4-RD. The patient received prednisone and rituximab (RTX), resulting in complete resolution of the mass, which was confirmed on repeat imaging. This case report provides a valuable addition to the literature to highlight that the diagnosis of IgG4-RD is based on the combination of characteristic clinical, serologic, radiologic, and histopathologic findings. Also, it underlines that the management of the disease is through glucocorticoids (GCs) as the first-line agent for remission induction in all patients with active, untreated IgG4-RD. RTX therapy is an effective treatment for IgG4-RD that is refractory to GCs. Recent studies have suggested that RTX monotherapy can be used to induce and maintain remission in patients with IgG4-RD.

Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organ systems. Due to the heterogeneity of its presentation, it is challenging for clinicians to diagnose and manage the symptoms. SLE has a wide range of presentations from mild to severe and involves various organ systems like mucocutaneous, musculoskeletal, cardiopulmonary, renal, gastrointestinal, and central nervous system. Various novel treatment modalities are being used based on clinical presentation. Prednisone and methylprednisolone are commonly used as needed for acute flares of SLE. Some patients may need a low dose of oral prednisone to keep their SLE under control, which carries a risk of coronary artery disease (CAD) and many other metabolic side effects of steroids. Other long-term medications that are commonly used include hydroxychloroquine, methotrexate, azathioprine, mycophenolate, cyclosporine, and cyclophosphamide. Intravenous cyclophosphamide is used only in severe lupus with renal, pulmonary, or CNS involvement. Rituximab is a human monoclonal B-cell cluster of differentiation (CD)20 receptor antibody used for severe SLE not responding with other medications. Other newer medications are belimumab and anifrolumab. Anifrolumab is a fully human monoclonal antibody that binds to subunit 1 of the type I interferon receptor. We present a case of a 25-year-old female with a chronic history of SLE presented to the outpatient clinic with abdominal distension that needed frequent abdominal paracenteses. She was using hydroxychloroquine, mycophenolate mofetil, and prednisone, but her symptoms were not adequately controlled. After we started the patient on monthly intravenous belimumab, her symptoms and the frequency of visits for paracentesis gradually reduced. B-cells are known to play an essential role in the pathogenesis of SLE, and the use of belimumab, an anti-BLys (B-lymphocyte stimulator) human monoclonal antibody that inhibits B-cell growth, can play a significant role in the management of SLE associated chronic serositis.

Pulmonary manifestations can be present in 20-80% of patients having mixed connective tissue disorder (MCTD) and are usually subacute. MCTD when associated with polymyositis can rarely involve the diaphragm, causing respiratory failure.We present herein the case of a 49-year-old female having MCTD with a component of polymyositis who presented with bilateral diaphragmatic paralysis followed by heart failure requiring respiratory support with non-invasive mechanical ventilation. We are aware of only one prior instance of MCTD associated with unilateral diaphragmatic weakness causing mild respiratory dysfunction. To the best of our knowledge, this is the second reported case of diaphragmatic involvement in the MCTD population, with bilateral diaphragmatic paralysis causing severe respiratory failure. This is also the first reported case of such an unusual initial presentation in this patient group. Pulmonary involvement has a poor prognosis. Early diagnosis with the initiation of therapy can improve mortality outcomes in this patient population.

Background: In the spring of 2021, coronavirus disease 2019 (COVID-19) vaccines were approved and distributed in the United States for the public to combat the COVID-19 pandemic, but their rapid development leaves some questions unanswered. Vaccine efficacy has always been a point of interest for individuals with rheumatological diseases that take immunosuppressants. This study investigates the vaccine efficacy of two COVID-19 mRNA-based vaccines, Moderna and Pfizer, in subjects in West Texas patients with autoimmune diseases. Materials and Methods: Blood was collected from Texas Tech University employees who received both doses of COVID-19 vaccines within the past nine months. Subjects were separated into either a group with a known history of rheumatic disease (n=18) or those without (n=18). The samples were analyzed for serum immunoglobulin A (IgA), immunoglobulin G (IgG), and immunoglobulin M (IgM) levels using specific enzyme-linked immunoassay kits, and a neutralizing antibody test using a surrogate virus was conducted as well. Results were analyzed using the Mann-Whitney U test (unpaired, two-tailed). Results: There was no significant difference in serum IgG and IgA levels between the control and rheumatologic disease groups, but there were significant differences in serum IgM levels. All subjects cleared the threshold for the neutralizing antibody test. Conclusion: The relatively similar serum IgG levels and the 100% detection rate of effective neutralizing antibodies across both groups indicate promising signs of serological response for subjects with autoimmune conditions, but the relatively low serum IgA and IgM levels of the study the group warrants further investigation.

A 56-year-old Hispanic female presented with six weeks of progressive dysphagia, proximal muscle weakness, erythematous rash, bilateral upper extremity pitting edema, and left lower extremity pitting edema. She had preserved heart function and a normal echocardiogram (ECG). She presented with elevated creatine kinase (CK) and aldolase, with normal renal function. Muscle biopsy suggested idiopathic polymyositis. No blood clot was seen on deep vein thrombosis (DVT) ultrasound. The myositis antibody panel showed the NXP-2 antibody, which is usually seen in pediatric dermatomyositis cases. In our literature search, extremity pitting edema is an unusual way of presentation in dermatomyositis. She responded with intravenous immunoglobulin (IVIg) and high-dose intravenous steroids. We used azathioprine for remission maintenance; her rash recurred after tapering steroids. We resumed tapering steroid therapy and started her on weekly methotrexate along with daily azathioprine. With this combination therapy, her rash and muscle function improved. We successfully tapered her steroids. In our literature search, combination therapy with azathioprine and methotrexate was not reported. Our patient is tolerating this therapy very well.