No abstract

Encephalitis is an inflammatory process of the brain that is most commonly related to infectious etiology; nonetheless, autoimmune encephalitis has been an increasingly identified entity that can cause it as well and should be considered. N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently identified process but remains less recognized than autoimmune encephalitis. We report a case of an 18-year old female who initially presented with seizures and later developed behavioral symptoms of agitation, crying, screaming, and emotional lability. Ultimately, she was found to have NMDA receptor encephalitis related to ovarian paraneoplastic teratoma. The patient was treated with anti-epileptics and intravenous immunoglobulin and underwent oophorectomy that lead to her recovery. This case highlights the importance of early recognition of NMDA receptor encephalitis to facilitate appropriate investigations and management.

B-cell prolymphocytic leukemia (B-PLL) is a rare leukemia characterized by rapidly increasing leukocytosis with splenomegaly and lymphadenopathy. Treatment strategies are largely based on studies of chronic lymphocytic leukemia (CLL). Antibodies against the cell surface protein CD20 are considered to be first-line therapy. A 76-year-old male with known CLL presented 2 weeks after starting chemoimmunotherapy for newly refractory CLL after failing ibrutinib therapy. White blood cell count was elevated at 226.7 × 103/µL. Fluorescent in situ hybridization analysis of a bone marrow specimen showed new development of complex cytogenetics. Flow cytometry revealed B cells appearing slightly dimmer on CD45 and brighter on CD20 compared with typical B-CLL suggestive of less mature lymphocyte forms. The patient was diagnosed with B-PLL and started on obinutuzumab and venetoclax with rapid normalization of white blood cells. This case recapitulates the challenges in diagnosing and treating B-PLL. Ibrutinib resistance is a growing area of study with several proposed mechanisms of acquired resistance. The pathogenesis of B-PLL is not completely understood, although mutations in MYC are presumed to play a role.

Hepatitis is a rare complication of herpes simplex virus (HSV) which can lead to acute liver failure, liver transplant, or death. This complication is more commonly seen in neonates, immunocompromised, or pregnant patients. Early recognition of disease facilitates prompt treatment with antiretrovirals and prevent its progression. To our knowledge, only 30 cases have been reported. Our patient presented with headaches and elevation of transaminases followed by vesicular rash. Culture tested positive for HSV1 and HSV2 and the patient was successfully treated with Acyclovir.

ObjectiveMen with systemic lupus erythematosus (SLE) are an understudied population. The present study characterized differences between men and women with SLE.MethodsWe examined cross‐sectionally participants with SLE in the All of Us Research Program, a US cohort with a participant survey at enrollment (May 2018 to June 2022) and linked electronic health record (EHR) data. We described and compared characteristics of men and women with SLE encompassing disease manifestations and prescribed medications from EHR data and socioeconomic factors, including health literacy and health care access and utilization, from surveys. We reported racial variations stratified by sex.ResultsOf 1,462 participants with SLE, 126 (9%) were male. Men reported lower educational attainment and less fatigue than women. Myocardial infarction was significantly more common in men. Men had significantly less confidence in completing medical forms than women and exhibited a trend toward requiring more help in reading health‐related materials. Barriers to health care access and utilization were common in both men and women (40% versus 47%, respectively, reporting some reason for delay in care; P = 0.35). Women of race other than Black or African American or White more often reported delaying care due to cultural differences between patient and provider.ConclusionOur study demonstrated major clinical and health literacy differences in men and women with SLE. Socioeconomic factors were significant barriers to health care in both sexes. Our study suggests men have disproportionately poorer health literacy, which may exacerbate preexisting disparities. Further large prospective studies, focusing on recruiting men, are needed to better characterize racial differences in men with SLE.

Monoclonal gammopathy of renal significance is a relatively new diagnosis that attributes kidney disease to damage caused by a monoclonal protein. There is growing recognition of this disease in patients previously diagnosed with monoclonal gammopathy of undetermined significance, as they increasingly develop clinically significant renal impairment requiring treatment. We outline a case of a patient presenting with worsening renal function, found to have a circulating monoclonal protein and ultimately diagnosed with a subtype of monoclonal gammopathy of renal significance referred to as immunotactoid glomerulopathy.

Idiopathic granulomatous mastitis (IGM) is a rare, benign inflammatory disorder of the breast. Clinical features may include painful breasts, erythema, subcutaneous nodules, and ulcerative lesions. It can mimic various other breast pathologies, and it is a diagnosis of exclusion after infection, malignancy, and other inflammatory conditions have been ruled out. In this article, we present a case of IGM developing in a 40-year-old female 3 months after hospitalization for myxedema coma. A contrast-enhanced magnetic resonance imaging of the breasts showed bilateral edema, and a biopsy was negative for malignancy or infection. She was started on prednisone and had noticeable improvement of ulcerations within several weeks. IGM is a rare condition that requires a multimodal treatment approach. Often recalcitrant disease is encountered and requires surgical intervention, immunosuppression, and antimicrobial therapy. The diagnosis should be entertained in patients with bilateral breast inflammation to avoid unnecessary surgical resection early on.

Crescentic glomerulonephritis, also known as rapidly progressive glomerulonephritis, is a syndrome characterized by progressive and rapid deterioration of renal function over the course of weeks to months. Oliguria, hematuria, azotemia, and hypertension are characteristic features of this condition. Crescentic glomerulonephritis is further classified according to the staining pattern on immunofluorescence. In rare instances, a mixed pattern of injury is encountered as in the case of double antibody-positive rapidly progressive glomerulonephritis (RPGN). This case illustrates the challenge in treatment of double antibody-positive RPGN in an elderly female with no previous renal disease. The patient was found to be positive for anti-GBM antibody and MPO-ANCA. Treatment was initially targeted against MPO-ANCA as the biopsy was most consistent with this process; however, the patient failed to respond to treatment and was subsequently transitioned to oral cyclophosphamide directed against anti-GBM disease. In cases of doubly antibody-positive RPGN with anti-GBM disease and ANCA-associated vasculitis, initial treatment should focus on inducing remission of anti-GBM disease as double antibody-positive disease often presents with the aggressive morbidity and mortality seen in anti-GBM disease, and the chronic risk of relapse seen in ANCA-mediated vasculitis.