Dry Crusty Nose: Not Just Rhinosinusitis! Hidden IgG4-related Disease Without IgG4 Level Rise.

IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition that can affect various organs. Localized sinonasal IgG4-RD is a rare condition characterized by bone and soft-tissue invasion. In this report, we present a case of a patient initially diagnosed with chronic rhinosinusitis, who underwent endoscopic sinus surgery and was later found to have biopsy proven IgG4-related sinonasal disease despite having normal serum levels of IgG4, resulting in erosion of the right lamina papyracea.

Tissue Plasminogen Activator in Acute Cardiac Arrest.

Tissue plasminogen activator (TPA) is indicated as an empiric therapy for refractory out-of-the-hospital cardiac arrest for suspected pulmonary embolism and myocardial infarction. Intracranial hemorrhage following TPA administration is a rare complication resulting in increased morbidity and mortality. A history of intracranial bleed, oral anticoagulant use prior to hospital admission, low body weight, and unstable hypertension with blood pressure above 180/110 mmHg at the time of presentation are associated with intracranial bleeding following tPA administration. Dedicated imaging including a Computed Tomography of the head without contrast, while feasible for patients presenting with acute stroke, is impractical in the setting of cardiac arrest. Here we report a case of 66 years old patient who presented in context of refractory cardiac arrest with recurrent PEAs with interval return of spontaneous circulation (ROSC) and was given tPA with eventual ROSC. He was subsequently found to have both a subarachnoid and intraventricular hemorrhage.

Reactive Arthritis After mpox Vaccination.

Autoimmune inflammatory reaction after vaccination is a rare clinical entity. Reactive arthritis has been described after various vaccinations, but not after mpox vaccination. Here we present a case of recently diagnosed reactive arthritis after mpox vaccination that presented in the context of unrelenting fever and diarrhea complicated by migratory arthritis and anterior uveitis. We have reported this case to the Vaccine Adverse Event Reporting System (VAERS).

Dry Beriberi Post Roux-en-Y Gastric Bypass Surgery.

Bariatric surgery is an effective strategy for achieving substantial weight loss, prolonging survival, and improving the comorbidities associated with obesity. Nutritional deficiency is a commonly recognized post-procedural complication. Here, we present a case of a patient with paresthesia, lower extremity weakness, and altered mental status one year following Roux-en-Y gastric bypass, who was found to have multiple vitamin and micronutrient deficiencies and was diagnosed with beriberi in the setting of profound thiamine deficiency.

Peritoneal Amyloid as a Presenting Manifestation of AL Amyloid.

Amyloid is a systemic disease characterized by extracellular deposition of misfolded protein. Gastrointestinal and peritoneal deposition of light chain (AL) amyloid is an under-recognized manifestation of this systemic disease, usually as a late sequela. Here we present a case of recently diagnosed AL peritoneal amyloid that presented in the context of recurrent, acute onset abdominal discomfort and was found to have bowel obstruction complicated by perforation in the setting of AL-mediated gastrointestinal tract infiltration and dysmotility.

Coronary artery bypass grafting for triple vessel disease in cardiac amyloidosis.

Cardiac amyloidosis is a rare condition with an estimated incidence of 18-55 per 100 000 person-years. It is associated with either immunoglobulin light chain (AL) or transthyretin amyloid (ATTR), both of which result in a restrictive cardiomyopathy complicated initially by diastolic dysfunction and subsequently followed by biventricular systolic heart failure. Untreated cardiac amyloidosis carries an extremely poor prognosis with an estimated median survival time of less than 1 year in AL and 4 years in ATTR amyloidosis. This is the sixth described report of coronary artery bypass grafting in patients with underlying cardiac amyloidosis.

Hepatic segmental arterial mediolysis: A case report and brief literature review.

Key Clinical message: When evaluating patients with abdominal pain, it is important to consider SAM in the differential diagnosis, along with vasculitis, fibromuscular dysplasia (FMD), atherosclerosis, mycotic aneurysms, and cystic medial degeneration. Abstract: Segmental arterial mediolysis (SAM) is a rare arteriopathy which is an under-recognized and commonly missed diagnosis of abdominal pain. We report a case of a 58-year-old female who presented with abdominal pain and was misdiagnosed with a urinary tract infection. The diagnosis was made with CTA and managed with embolization. Despite appropriate intervention and close hospital monitoring, further complications were inevitable. We conclude that though literature has shown better prognosis and even complete resolution after medical and/or surgical intervention, close follow up and monitoring is needed to avoid unexpected complications.

Diagnostic dilemma: drug-induced vasculitis versus systemic vasculitis.

Drug-induced vasculitis can rarely cause inflammation and necrosis of blood vessel walls of both kidney and lung tissue. Diagnosis is challenging because of the lack of difference between systemic and drug-induced vasculitis in clinical presentation, immunological workup and pathological findings. Tissue biopsy guides diagnosis and treatment. Pathological findings must be correlated with clinical information to arrive at a presumed diagnosis of drug-induced vasculitis. We present a patient with hydralazine-induced antineutrophil cytoplasmic antibodies-positive vasculitis with a pulmonary-renal syndrome manifesting as pauci-immune glomerulonephritis and alveolar haemorrhage.

Pulmonary Artery Aneurysm Associated With Aspergilloma in a Patient Diagnosed With Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA) is an autoimmune, necrotizing granulomatous disease that affects small- and medium-sized blood vessels. Aspergilloma is a fungal mass of Aspergillus and usually found in the preexisting cavity in lung parenchyma. Surgical resection is the mainstay of treatment of aspergilloma. In this article, we present a case of a 70-year-old male with GPA and aspergilloma who presented with massive, life-threatening hemoptysis. Further workup with a chest computed tomography (CT) pulmonary angiogram demonstrated a pulmonary artery pseudoaneurysm along the wall of the lung cavity which was emergently managed with embolization and required monitoring in the medical intensive care unit. This case report alerts clinicians to maintain a high level of suspicion for an aneurysm if the degree of hemoptysis is higher than expected.

Unraveling the Puzzle: A Case Report Questioning the Causal Relationship Between Subarachnoid Hemorrhage and Microscopic Polyangiitis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are small-to-medium-vessel vasculitis, which includes granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. MPA predominantly affects the kidneys and lungs. Subarachnoid hemorrhage (SAH), a life-threatening condition, rarely occurs with AAV. In this case, we present a 67-year-old female who presented with a sudden-onset headache after a recent diagnosis of ANCA-associated renal vasculitis. Kidney biopsy revealed pauci-immune glomerulonephritis, and serum was positive for ANCA along with myeloperoxidase antibody. A computed tomography scan of the head revealed both SAH and intraparenchymal hemorrhage. The patient was managed medically for SAH and intraparenchymal hemorrhage. ANCA vasculitis was treated with steroids and rituximab, and the patient showed improvement.

Antisynthetase Syndrome With Paraneoplastic Antibodies.

Antisynthetase syndrome (ASyS) is an uncommon systemic autoimmune disorder characterized by the presence of autoantibodies targeting aminoacyl-transfer RNA (tRNA) synthetase. The syndrome displays a diverse range of clinical manifestations affecting multiple organs, thereby posing a diagnostic challenge. In this report, we present an unusual case of a patient diagnosed with ASyS, displaying positive anti-PL-12 antibodies along with paraneoplastic antibodies. To the best of our knowledge, this is the first documented case in the existing literature describing ASyS with the presence of anti-PL-12 antibodies and concomitant paraneoplastic antibodies in the context of ductal carcinoma in situ.

Overlapping Tubulointerstitial Lupus Nephritis and Immunoglobulin G4-Related Disease: A Case Report.

Immunoglobulin G4-related disease (IgG4-RD) in conjunction with systemic lupus erythematosus (SLE) is a rare occurrence. In this case report, we present the case of a 50-year-old female who was diagnosed with SLE based on clinical and laboratory criteria. The patient exhibited pericardial effusion necessitating pericardiocentesis, pleural effusion requiring thoracentesis, and impaired renal function necessitating dialysis. Renal biopsy revealed findings consistent with tubulointerstitial lupus nephritis and IgG4-related disease. Additionally, elevated levels of serum IgG4 were detected. The patient received intravenous pulse dose steroids and oral steroids, which were tapered gradually, followed by daily hydroxychloroquine treatment and two doses of rituximab every two weeks. Consequently, the patient experienced an improvement in renal function and no longer needed dialysis. To our knowledge, only a few reports of this overlap exist. This late diagnosis of SLE could be explained by the fact that IgG4 is associated with milder renal disease in lupus patients, due to its inability to activate the classical complement pathway. IgG4-RD/SLE overlap patients usually respond well to a combination of steroids and other immunosuppressants used to treat SLE. However, our experience with treating this disease overlap remains limited due to its extreme rarity.

Spontaneous Retroperitoneal Hematoma in Vitamin C Deficiency.

Retroperitoneal hematoma is rare but potentially life-threatening. It is commonly caused by traumatic or iatrogenic vascular injury, retroperitoneal neoplasm, coagulopathy, chronic anticoagulation, or fibrinolytic therapy. However, retroperitoneal hematoma due to vitamin C deficiency is rare. Here, we report a case of 40 years old man who developed retroperitoneal hematoma in context of very low vitamin C. To our knowledge, this is the second described case of retroperitoneal hematoma from vitamin C deficiency.

Superior Mesenteric Artery Thrombosis and Intestinal Ischemia as a Consequence of COVID-19 Infection.

COVID-19-associated arterial and venous thrombotic events are multifactorial in origin, resulting in significant morbidity and mortality. Intestinal ischemia due to thrombus is a rare manifestation of COVID infection. Here, we report the case of a patient who presented with fever, malaise, and diarrhea, and was found to be COVID-19 positive; his clinical course was further complicated by devastating thrombosis of the superior mesentery artery (SMA) associated with COVID-19 infection.

Acute Presentation of Primary CNS Lymphoma Mimicking Toxoplasma in HIV Infection.

Primary CNS lymphoma (PCNSL) accounts for up to 15% of non-Hodgkin lymphomas in HIV patients and is the second most common cause of space-occupying brain lesions in HIV patients after CNS toxoplasmosis. Differentiation of PCNL and CNS toxoplasmosis is crucial as PCNL carries a poor prognosis with survival time of 2-4 months without treatment but can be improved with prompt initiation of chemotherapy. These two entities often present clinically in a similar manner, and conventional imaging can also be a diagnostic challenge due to overlapping imaging characteristics. Thus, definitive diagnosis of PCNSL relies on histopathologic confirmation. Here, we present a case of intracranial lesion that presented acutely in the context of headache and left sided body weakness and was found to have PCNSL.

Surgical Emergencies among Gynecological Surgeries in a Tertiary Care Center: A Descriptive Cross-sectional Study.

INTRODUCTION: The management of gynecological emergencies is essential for the preservation of the life of affected woman, her sexual functions and fertility particularly in disease conditions that threaten her life. The main objective of the study is to determine the proportion of the surgical emergencies among gynecological surgeries in a tertiary care center. METHODS: This is a descriptive cross-sectional study conducted in the department of gynecology and obstetrics in Shree Birendra Hospital, Kathmandu, Nepal from April 2013 till March 2017. Ethical approval was taken from the Institutional Review Committee (IRC) in November 2019. This study was conducted among 515 gynecological surgeries by using convenience sampling methods. Point estimate at 95% Confidence Interval was calculated along with frequency and proportion for binary data. Data were analyzed using Excel software. RESULTS: In our study, the proportion of surgical emergencies among total gynecological surgeries performed in the department of gynecology and obstetrics in Shree Birendra Hospital was 120 (23.30%). The highest number of surgical emergencies was observed in the age group of 20-29 years old, followed by less than 19 years of old age group. Ectopic pregnancy accounting for 85 (70.83%) is found to be the most common surgical emergencies in our study. Out of all surgical emergency cases, most of them underwent salpingectomy 65 (54.16%) followed by salpingectomy with tubal ligation 20 (16.16%). CONCLUSIONS: Surgical emergencies among gynecological surgeries are found to be in greater proportion in the department of gynecology and obstetrics in Shree Birendra Hospital. Ectopic pregnancy accounted for more than half of the diagnoses in this study.