Close, but no cigar: an unfortunate case of primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (PACNS) is an uncommon and misunderstood disease, where little is known regarding its immunopathogenesis and appropriate treatment. Due to the constellation of nonspecific clinical features and imaging findings, PACNS is a diagnostic conundrum for clinicians and can be very difficult to treat. Case Presentation: A 64-year-old male with a history of prostate cancer presented to the emergency department with expressive aphasia and severe headache. Previously, he was diagnosed with ischemic strokes at outside hospitals and was subsequently initiated on anticoagulation medication but was later readmitted with a new onset of nontraumatic subarachnoid hemorrhage and later was found to have ischemic changes in the right temporoparietal lobe. He was suspected to have hypercoagulability of malignancy, as he was unresponsive to a wide variety of anticoagulants and his symptoms continued to deteriorate. On presentation, the physical examination was significant for right homonymous hemianopia, with positive antinuclear antibodies and notable erythrocyte sedimentation rate. The results from the full serologic workup was negative. Subsequent imaging of the brain revealed multifocal stenoses in multiple arteries. On further examination, digital subtraction angiography was concerning for vasculopathy, and was initiated on corticosteroids and cyclophosphamide. Discussion: This is one of the first cases of PACNS in which recurrent strokes were the presenting symptom for PACNS. Vasculitis should be a considered differential in patients with recurrent ischemic strokes and failed anticoagulant therapy. It is important to rule out malignancy and infectious causes due to the wide spectrum of conditions that cause central nervous system vasculitis.

Case of recurring Kikuchi disease and autoimmune hepatitis.

We present a case of a 47-year-old female patient with a history of diagnosed Kikuchi disease and autoimmune hepatitis 13 years ago who presented with recurrent fevers and a desquamative rash on the lower extremities. Computed tomography neck showed enlarged lymph nodes, and with her daily fevers and skin rashes the presentation was concerning for recurrence of her Kikuchi disease. The patient was also found to have an elevated anti-smooth muscle antibody titer, and subsequent liver biopsy confirmed the diagnosis of autoimmune hepatitis. She was started on methylprednisolone with improvement. Our case emphasizes the association of Kikuchi disease with autoimmune conditions other than systemic lupus erythematosus. Given the recurrence of the disease after a decade of quiescence, long-term follow-up of patients with Kikuchi disease should be implemented.

Aortitis presenting as acute myeloid leukemia - A case report.

Introduction: Aortitis can be an initial presentation of many diseases, but most often include large cell vasculitis. Case presentation: We present a case of a 53 year old female who presented with a myriad of symptoms including abdominal pain, nausea, fever, and headaches. CT scan of the chest showed an inflamed aorta with an initial concern for a large cell vasculitis, but found later to be from her underlying acute myeloid leukemia (AML). Discussion/conclusion: Our case emphasizes the importance of aortitis being the initial presentation of a multitude of diseases including malignancy. It remains important, especially for rheumatologists, to consider blood cancers when presented with aortitis. Background/introduction: Aortitis can be an initial presentation of many diseases, but most often include large cell vasculitis. We describe a case of aoritis being the initial presentation of AML. Methods: SCARE 2020 Guidelines.

A rare presentation of necrotizing sarcoidosis.

Sarcoidosis is a disease with unknown aetiology and pathogenesis which affects young adults and is usually a non-necrotizing granulomatous disease seen in histology. Variants of the disease, such as necrotizing sarcoidosis, were first described by Liebow in 1973 and are rarely seen. This case report describes the case of a 60-year-old Bengali female who presented with vague symptoms and was found to have chronic granulomatous inflammation with foci of calcifications involving the lungs, liver and spleen consistent with necrotizing sarcoidosis.

Cerebellar infarction as the initial presentation of IgG4-related disease.

Although IgG4-RD has CNS manifestations, cerebellar involvement has only been reported in three cases. Our patient presented with cerebellar symptoms, several cerebellar infarcts were evident on the brain MRI, and CT abdomen revealed retroperitoneal tumor. Endoscopic biopsy confirmed IgG4-RD. Steroids are the first-line therapy for IgG4-RD, but our patient was lost to follow-up before treatment.

Leukocytoclastic Vasculitic Rash Following Second Dose of Moderna COVID-19 Vaccine.

The immunization against coronavirus disease (COVID-19) via vaccination serves as a significant milestone in the fight against the pandemic. Rapid introduction of various COVID-19 vaccines to stem the spread of virus has researchers scrambling to document the adverse effects left in its wake. Thus far, there have been singular examples of cutaneous vasculitis associated with COVID-19. A history of vasculitis leaves little error to miss its inclusion in diagnostic differentials. It also invokes the physiologic possibility that afflicted patients possess a more susceptible landscape for recurrence that was then triggered by the vaccine when compared with those who lack similar history. In our case report, we build on those findings with one of the first documented examples of vaccination-induced vasculitic rash in a previously asymptomatic patient.

Chasing the dragon and stumbling upon an octopus: A case of heroin-induced leukoencephalopathy and reverse takotsubo cardiomyopathy.

The practice of heating heroin and inhaling its vapors, commonly referred to as "chasing the dragon" has been around for decades, but only gained popularity in the United States in the 1990s. Since then, there have been many documented cases of heroin-induced leukoencephalopathy (HIL) and takotsubo cardiomyopathy (TTC). This case highlights a patient with a history of heroin inhalation who presented with multiple neurological features, including bilateral upper and lower extremity weakness, blurry vision and slurred speech. Symptoms progressively worsened over the course of multiple weeks and brain imaging was consistent with toxic leukoencephalopathy secondary to heroin inhalation. Medical course was complicated by a rare associated feature of HIL: reverse Takotsubo cardiomyopathy (rTTC). Transesophageal echocardiogram demonstrated a classic basal hypokinesis and ballooning characteristic of rTTC. The patient's symptoms were treated as currently there is no guideline directed therapy for HIL or rTTC. This case demonstrated a rare and significant complication of heroin inhalation: HIL and rTTC and described potential therapies currently being studied.

Diagnosis and management of unilateral subclavian steal syndrome with bilateral carotid artery stenosis.

Subclavian steal syndrome is a rare phenomenon occurring from retrograde blood flow in the vertebral artery due to proximal stenosis in the subclavian artery. As a result, the arm gets blood supply from the vertebral artery at the expense of the vertebrobasilar system. The patient remains largely asymptomatic until there is an increase demand for blood supply to the arm, resulting in a constellation of symptoms including dizziness, vertigo, blurred vision, diplopia, headache, syncope, postural hypotension, neurologic deficits, and rarely, memory problems. The management approach depends on the severity of clinical symptoms but includes medical treatment, endovascular therapy and lifestyle modifications.

Extended spectrum beta lactamase producing Escherichia coli eustachian valve infective endocarditis.

Endocarditis is an infection of the endocardium caused by a multitude of bacteria, including S. aureus, viridans streptococci, S. bovis, or S. epidermidis, among others. It can cause a variety of physical findings, including new onset murmur, Osler nodes, and Janeway lesions. Endocarditis is diagnosed with multiple positive blood cultures with transesophageal echocardiogram (TEE) showing valvular vegetations. In this article, we present a 47 year old female with a history of ESRD on dialysis who presented with a bleeding fistula found to be in septic shock. Diagnosis of eustachian valve endocarditis with E. Coli ESBL was made through positive blood cultures as well as using TEE. She was started on IV meropenem for seven days, to which the patient completed and eventually was discharged home with resolution of symptoms.

Chilaiditi syndrome: A structural displacement in a heart failure patient.

BACKGROUND: Chilaiditi's sign is often found incidentally on chest or abdominal radiograph and can be accompanied by clinical symptoms such as abdominal pain, gastrointestinal complications, and less commonly associated with dyspnea. CASE PRESENTATION: In this interesting case, we discover lingering dyspnea in our 79 year old male with a past medical history of asthma and heart failure with preserved ejection fraction admitted for acute heart failure exacerbation with reduced ejection fraction along with a new incidental finding of Chilaiditi's sign on chest radiograph. Patient received optimal diuretics and guideline-directed medical treatment for heart failure exacerbation, but mild dyspnea with pleuritic chest pain persisted. Dyspnea with pleurisy was likely attributed to a structural anatomical defect (Chilaiditi's sign) that can be picked up on imaging. CONCLUSION: Chilaiditi syndrome can be an incidental cause of ongoing persistent dyspnea, and if symptoms are severe, intervention can be warranted for symptomatic resolution. LEARNING OBJECTIVE: Chilaiditi syndrome should be considered as a possible diagnosis among patients with a history of heart failure and incidental Chilaiditi's sign on chest radiographic imaging who suffer from persistent dyspnea and pleurisy despite optimal diuretics and guideline-directed medical treatment.