Acral vascular necrosis associated with immune-check point inhibitors: case report with literature review.

BACKGROUND: Treatment of solid malignancies has been revolutionized with the introduction of immune checkpoint inhibitors (ICIs) and their use is being expanded in therapy of different cancers. However, immune related adverse events (IRAEs) can occur during treatment. These side effects occur due to stimulation of the innate and adaptive immune system and can lead to serious complications. Recently, acral ischemia has been reported in some cases during treatment with programmed death-1 (PD-1) and cytotoxic T lymphocyte associated antigen-4 (CTLA-4) inhibitors. Here, we discuss a case in which acral necrosis developed after initiation of a PD-1 inhibitor. We offer a review of the existing literature on the pathophysiology, clinical course and treatment outcomes. CASE PRESENTATION: A 68-year-old female was diagnosed with stage IV non-small cell lung adenocarcinoma and was started on pembrolizumab. The patient developed sudden onset numbness and discoloration of fingertips bilaterally at week 25 after initiation of ICI treatment. Extensive workup to rule out hypercoagulable, autoimmune and vascular disease was unremarkable except for mild elevation of ANA and ESR. The symptoms quickly progressed into dry gangrene within four weeks and did not respond to medical or surgical treatment. Pembrolizumab was subsequently discontinued due to progression of metastatic disease. The patient refused further interventions and transitioned to hospice care where she expired after two months. CONCLUSION: Acral ischemia can develop during treatment of malignancies. This complication, although uncommon, canresult in digital amputation. Physicians should be aware of the possible progression of acral vascular necrosis when Raynaud's like symptoms develop. Larger studies are needed to confirm the role of ICIs in the pathogenesis of acral vascular necrosis.

A Case of Bilateral Hyperglycemic Nonketotic Chorea in an 83-year-old Woman With Normal Brain Imaging.

Bilateral hyperglycemic nonketotic chorea is a rare complication of hyperglycemia. In most cases, the literature illustrates patients presenting with unilateral chorea with image findings significant for hyperintense lesions in the basal ganglia on magnetic resonance imaging (MRI) or hyperdensities on computerized tomography (CT). Here, we present a case of an 83-year-old patient who was admitted to the hospital due to acute onset of orofacial and bilateral upper extremity chorea. She had no previous history of infection, genetic mutation, neoplasms, neurodegeneration, stroke, metabolic disease, drug exposure, or autoimmune disease. Surprisingly, her MRI showed only chronic microvascular changes in periventricular white matter without basal ganglia abnormalities. However, she was noted to have marked worsening of her glycemic control over the preceding 12 months based on worsening glycated hemoglobin (HbA1c) levels and elevated serum glucose on presentation. A literature review indicates that chorea caused by hyperglycemia is at times reversible with glycemic control, but as demonstrated in our patient, this is not always necessarily the case. A similar course has only been elaborated in a few other cases in the literature. We will also review the pathogenesis, the usual disease clinical course and standard treatment from the literature.

A Diagnostic Dilemma: Adult-Onset Still's Disease With Secondary Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome?

Adult-onset Still's disease (AOSD) is a rare autoinflammatory condition. It is a diagnosis of exclusion by ruling out all related infectious, inflammatory, autoimmune, and malignant diseases. We present a case of a 23-year-old Caucasian male who presented with fever, night sweats, joint pain, weight loss, and diarrhea. The initial presentation delayed the diagnosis. Upon further investigation, we formulated the diagnosis of AOSD. In sporadic cases, AOSD with secondary hemophagocytic lymphohistiocytosis (HLH), also known as macrophage activation syndrome (MAS), is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation. In case of suspected secondary complications, timely involvement of a multidisciplinary team and starting of appropriate medications is necessary.

A Unique Case of Bannwarth Syndrome in Early Disseminated Lyme Disease.

Lyme borreliosis is a multisystem inflammatory disease caused by the spirochete Borrelia burgdorferi (B. burgdorferi) and transmitted through the Ixodes tick. Nervous system involvement is known as Lyme neuroborreliosis; it only occurs in disseminated Lyme disease and is manifested by the classic triad of meningitis, cranial neuritis, and radiculoneuritis. Timeline is a significant factor when staging Lyme manifestations. However, certain cases do not follow the typical presentation timelines described in most literature. We report a case of a 66-year-old male who presented with progressively worsening generalized body aches, severe fatigue, and new-onset urine retention for two weeks. Physical examination revealed severe pain with neck flexion and lying supine and diminished deep tendon reflexes bilaterally. Laboratory data revealed a positive Lyme immunoglobulin (Ig) M antibody with lymphocytic pleocytosis on lumbar puncture. He was treated with intravenous (IV) ceftriaxone for early disseminated Lyme disease. His radicular pain responded well to therapy, and he regained full bladder function. Bannwarth syndrome (BWS) is a term applied to the constellation of painful radiculoneuritis characterized as severe, burning, often dermatomal pain. In most cases, BWS affects the limbs, with only a few reported cases of sacral radiculitis causing neurogenic urinary dysfunction. Early recognition of this rare presentation associated with Lyme disease and treatment with antibiotics can prevent disease progression and detrimental neurological sequelae.

SARS-CoV-2 Vaccination-Induced Transverse Myelitis.

While mass immunization against coronavirus disease 2019 (COVID-19) rolls out around the globe, safety concerns and adverse events that need prompt evaluation are also emerging. We report a case of transverse myelitis and Bell's palsy after receiving Johnson and Johnson COVID-19 vaccination under the emergency use authorization in a healthy young woman with no past medical history. Other possible etiologies of her symptoms were ruled out, and she was treated successfully with steroids and plasma exchange.

Lung Adenocarcinoma Presenting as Malignant Pericardial Effusion/Tamponade.

Lung cancers are the most common primary tumors that involve the pericardium with a prevalence of up to 50%. Usually, pericardial involvement goes undetected with almost 10%-12% found among all cancer related autopsies. Rarely pericardial effusions can be the initial site of metastasis and initial manifestation of a primary tumor. In our case, we report a 57-year-old female presenting with cardiac tamponade and subsequent testing was done which revealed lung adenocarcinoma. Malignant pericardial effusions are often silent, but certain times can present with symptoms of shortness of breath, chest pain, cough, arrhythmias, and rarely as pericardial tamponade. A high index of suspicion is required when a patient presents with tamponade to diagnose malignancy. Emergent pericardiocentesis may be warranted depending on the clinical presentation but quite often, patients tend to have a poor prognosis despite therapy given the extent of disease.

Syncope as the Initial Manifestation of a Late-Stage Renal Cell Carcinoma With Metastasis to the Brain.

Renal cell carcinoma (RCC) is the most common neoplasm that arises from renal parenchyma. About one-third of patients with RCC develop metastatic spread, with common sites including the lung, liver, bone, adrenal gland, and brain. Distant metastases can be difficult to detect unless symptoms appear. We report a case of a 56-year-old female who presented to the emergency department with the unresponsiveness of unknown duration. She underwent a thorough laboratory workup, and the computed tomography (CT) scan revealed a retroperitoneal mass originating from the right kidney and a large hemorrhagic brain mass in the left frontal lobe. The patient underwent emergent full craniotomy for tumor removal, and histology confirmed metastatic RCC. Since several patients with RCC are asymptomatic, the slow growth of tumors leading to distant metastasis can be overlooked. Thus, this case demonstrates the importance of early detection of RCC to help prevent or delay further disease progression.

Early Thrombosis of Splenic Artery Stent Graft.

Splenic artery aneurysms (SAAs) are among the most common visceral aneurysms behind aortic and iliac arteries. Certain factors like aneurysm size (especially giant SAAs), hypertension (HTN), symptomatology, pregnancy, portal hypertension (pHTN), and liver transplantation increase the risk of rupture. Most often found incidentally, but when symptomatic, can present with nonspecific symptoms like nausea, vomiting, anorexia, and epigastric/left upper quadrant pain. Diagnosis can be accomplished with different modalities of CT or MRI and digital subtraction angiography (DSA) being the gold standard for diagnosis. Treatment is usually preferred for aneurysms >2 cm, symptomatic cases, and pregnant women. Various surgical/interventional procedures can be performed and selected based on the patient's sex, age, location of the aneurysm, size of the aneurysm, and presenting complaints/complications. Endovascular techniques with or without stent-graft placement are being used more, given the minimally invasive nature of these procedures. No clear guidelines exist on initiation of dual antiplatelet therapy (DAPT), but based on guidelines from visceral arterial stenting (especially iliac arteries and renal arteries), multiple case reports/series on SAAs, we highly recommend the usage of DAPT pre- and post-stent-graft placement to improve patency.

Vaping-Induced Lung Injury: An Uncharted Territory.

Vaping-associated lung injury (VALI) presents with symptoms ranging from lower respiratory tract involvement (shortness of breath, fever, and cough) to gastrointestinal involvement (vomiting and diarrhea). Based on the longitudinal analysis, VALI is associated with increased risk for respiratory disease, making it paramount for medical professionals to understand this disease process and be familiar with its varied presentations. Our case study is a presentation of two relatively young patients with VALI, with a varied clinical course and distinct levels of severity. VALI still remains uncharted territory. Case reports, such as ours, have the potential to invoke randomized controlled clinical trials to better understand the disease etiology, pathology, and management.

Pregnancy unmasking symptoms of undiagnosed lymphangioleiomyomatosis: Case report and review of literature.

Cystic lung diseases are a group of disorders that appear similar on radiological studies on chest computed tomography. Each disorder is characterized by its own etiology, pathophysiology, course of progression and manifestation. Lymphangioleiomyomatosis (LAM) is one of the cystic lung diseases that can either be hereditary or sporadic. The sporadic form is a rare disease with no accurate prevalence reported but is believed to be less than 10 per million. LAM is associated with inappropriate activation of mammalian target of rapamycin (mTOR) signaling which regulates cellular growth. The sporadic form is almost confined to premenopausal female population and estrogen is believed to play an important role in the pathogenesis. Pregnancy and use of estrogen based oral contraceptives can aggravate symptoms of already existing LAM. Here we describe a case of LAM that was previously treated as asthma and was diagnosed after exacerbation of respiratory symptoms after pregnancy. We offer a review of the medical literature regarding the etiology, clinical course, diagnosis and treatment of LAM.