Primary Immune Thrombocytopenic Purpura (ITP) and ITP Associated with Systemic Lupus Erythematosus: A Review of Clinical Characteristics and Treatment Modalities.

Immune thrombocytopenic purpura (ITP) is an immune-mediated disorder characterized by the destruction of platelets and megakaryocytes due to autoantibodies against the platelet surface proteins. ITP without any apparent cause of thrombocytopenia is defined as primary ITP, and ITP in the setting of SLE is secondary ITP, which can be diagnosed after excluding other causes of thrombocytopenia by history, physical examination, and laboratory testing. Patients with ITP associated with SLE have higher median platelet count and less bleeding manifestations compared to the patients with primary ITP. It can be very challenging to diagnose primary ITP in SLE patients as other causes of thrombocytopenia including drug-induced thrombocytopenia, antiphospholipid syndrome, and thrombotic microangiopathic process should be ruled out. Corticosteroids are the main modality of treatment. IVIG can be used in severe cases. Splenectomy was found to be less effective in ITP associated with SLE compared to primary ITP. Control of disease activity with immunosuppressive therapy can be helpful in some cases associated with active disease flares in SLE patients.

Epidemiology and outcomes of emergency department visits in systemic lupus erythematosus: Insights from the nationwide emergency department sample (NEDS).

BACKGROUND: Systemic lupus erythematosus (SLE) patients are prone to frequent emergency department (ED) visits. This study explores the epidemiology and outcomes of ED visits by patients with SLE utilizing the Nationwide Emergency Department Sample (NEDS). METHODS: Using NEDS (2019), SLE ED visits identified using ICD-10 codes (M32. xx) were compared with non-SLE ED visits in terms of demographic and clinical features and primary diagnoses associated with the ED visits. Factors associated with inpatient admission were analyzed using logistic regression. Variations in ED visits by age and race were assessed. RESULTS: We identified 414,139 (0.35%) ED visits for adults ≥ 18 years with SLE. ED visits with SLE comprised more women, Black patients, ages 31-50 years, Medicare as the primary payer, and had higher comorbidity burden. A greater proportion of Black and Hispanic SLE patients who visited the ED were in the youngest age category of 18-30 years (around 20%) compared to White patients (less than 10%). Non-White patients had higher Medicaid utilization (27%-32% vs 19% in White patients). Comorbidity patterns varied based on race, with more White patients having higher rates of hyperlipidemia and ischemic heart disease (IHD) and more Black patients having chronic kidney disease (CKD), hypertension, and heart failure. Categorizing by race, SLE/connective tissue disease (CTD) and infection were the most prevalent primary ED diagnosis in non-White and White patients, respectively. Age ≥ 65 years, male sex, and comorbidities were linked to a higher risk of admission. Black race (OR 0.86, p = .01) and lowest income quartile (OR 0.78, p = .003) had lower odds of inpatient admission. CONCLUSION: Infection and SLE/CTD were among the top diagnoses associated with ED visits and inpatient admission. Despite comprising a significant proportion of SLE ED visits, Black patients had lower odds of admission. While the higher prevalence of older age groups, hyperlipidemia, and IHD among White patients may partly explain the disparate results, and further study is needed to understand the role of other factors including reliance on the ED for routine care compared among Black patients, differences in insurance coverage, and potential socioeconomic biases among healthcare providers.

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.

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.

Neuromyelitis Optica in a Nepalese Man.

BACKGROUND: Neuromyelitis optica is a severely disabling inflammatory disorder of the central nervous system of autoimmune etiology that mainly affects the optic nerves and spinal cord. Here, we present a case report detailing a patient with tingling and weakness of right upper and lower limbs who was neuromyelitis optica immunoglobulin G-positive. CASE PRESENTATION: A 46-year-old Nepalese man presented to the hospital with a history of tingling and weakness of right upper and lower limbs that developed over a period of two months. Clinical evaluation showed diminished power across all major muscle groups in the right upper and lower limbs. Magnetic resonance imaging of his cervical spine showed T1 iso- to hypointense signal and T2 hyperintense signal in central cervical spinal cord from first to sixth cervical level, probably suggestive of myelitis or demyelination. The patient was immediately started on intravenous methylprednisolone. The diagnosis of neuromyelitis optica was later confirmed with strongly positive neuromyelitis optica immunoglobulin G. CONCLUSION: In resource limited setting, in the absence of tests for neuromyelitis optica immunoglobulin G, treatment was started and the patient's condition started to get better. Hence, early initiation of aggressive immunosuppressive treatment is essential in such cases.