Does Marine Lenhart Syndrome really exist?

Primary hyperthyroidism is the result of overproduction of thyroid hormone resulting in the classic symptoms of tachycardia, weight loss, diaphoresis, and hyperdefecation. There are multiple common causes to include Graves’ disease, toxic multinodular goiter, and solitary toxic adenomas. Marine Lenhart Syndrome (MLS) is a rare cause of hyperthyroidism, caused by a coexistence of constitutively active thyroid nodules and Graves’ disease. In the original document of Marine and Lenhart, there is no distinction made between the autoimmune phenomenon of Graves’ disease and the solitary toxic nodule of Plummer’s disease. Rather they are both considered to be the manifestation of the same disease. However, in the current era of radionuclide technology, a clear distinction of MLS can be seen with diffuse uptake in the thyroid gland and focused enhancement in the toxic nodules. Therefore what was previously described as one entity is now distinct as Graves’ disease and Plummer’s disease. It is also becoming increasingly clear within the literature that there is also a new phenomenon of post-radioiodine immunogenic hyperthyroidism in patients with toxic nodules and elevated autoantibodies. Therefore in order to properly treat and manage patients, a new definition of MLS may need to be proposed.

Hematologic Autoimmune Manifestation Secondary to Coronavirus Disease 19 Infection – A Single-Center Experience

Introduction: Since December 2019, multiple human cases of novel coronavirus infection were reported, representing with upper respiratory symptoms (influenza-like presentation). The virus was named the Severe acute respiratory system coronavirus 2 (SARS-COV-2). Studies have reported cases of patients with COVID-19 infection, including development of several autoimmune events that suggests that infection with SARS CoV-2 may be associated with initiation of autoimmune hematological autoimmune disorders. Aim: Review the hematological autoimmune phenomenon after infection with SARS-COV-2 in order to assist into the pathogenic mechanisms, clinical manifestations and treatment of this group of patients. Materials and methods: This is a retrospective study that includes 21 patients with autoimmune diseases like secondary immune thrombocytopenia (ITP), autoimmune hemolytic anemia( AIHA) and thrombotic thrombocytopenic purpura (TTP) that have emerged after COVID-19 infection. The patients were diagnosed and treated at the University Clinic of Hematology for a period of time from January 2020 to April 2021. Results: The most common hematologic autoimmune disorder was ITP in 13 cases (62%) followed by AIHA in 5 cases (24%) and TTP in 3 individuals (14%). The mean time of onset of the hematologic auto-immune presentations was 18,4 ± 10,3 days. The therapy of this conditions in patients with COVID-19 infection requires an individualized approach to achieve a precise balance between the risk of severe bleeding and of thromboembolic events. Conclusion: Causal relationship between COVID-19 infection and these autoimmune events still requires further studies. We should all have in mind the risk of development of hematologic autoimmune disorders in infected patients.

Severe systemic scleroderma with multiple organ involvement in a 45-years-old patient

Systemic scleroderma has inflammation and fibrosis playing a crucial role and eventually leading to severe functional failure and damage of multiple organs. Three primary mechanisms are mainly involved in the pathogenesis of scleroderma: vascular anomalies, excess fibrosis, and autoimmune phenomenon. The present case study shows a severe cardiac dysfunction with low cardiac output syndrome (LCOS) with cardiac, hepatic and renal impairment, peripheral ischemia, neurologic symptoms associated or even aggravated by a probable severe toxicity to azathioprine therapy. Currently there is no cure for systemic sclerosis, the aims of treatment are- as recommended also by EULAR: to relieve symptoms, to prevent the condition from progressing, as much as possible, to detect and treat complications early and to minimize any disability.

Autoantibodies against nephrin elucidate a novel autoimmune phenomenon in proteinuric kidney disease

Dysfunction of podocytes, cells critical for glomerular filtration, underlies proteinuria and kidney failure. Genetic forms of proteinuric kidney disease can be caused by mutations in several podocyte genes, including nephrin, a critical component of the kidney filter. In contrast, the etiology of acquired acute-onset nephrotic syndrome has remained elusive. Here we identify autoantibodies against nephrin in serum and glomeruli of a subset of adults and children with non-congenital acute nephrotic syndrome. Our findings align with published experimental animal studies and elucidate a novel autoimmune phenomenon in proteinuric kidney disease interfering with glomerular filter integrity.

Snorting the Brain Away: Cerebral Damage as an Extension of Cocaine-Induced Midline Destructive Lesions

Cocaine consumption is associated with a variety of clinical manifestations. Though cocaine intranasal inhalation always determines nasal mucosal damages, extensive septum perforations, and midline destructions—known as cocaine-induced midline destructive lesions (CIMDL)—affect only a limited fraction of patients. CIMDL is viewed as a cocaine-associated autoimmune phenomenon in which the presence of atypical anti-neutrophil cytoplasmic antibody (ANCA) promotes and/or defines the disease phenotype. A 51-year-old man presented with an intracranial tumor-like lesion by its space-occupying effect. CT also revealed the destruction of the nasal septum and skull base. A diagnosis of CIMDL was made in light of the patient’s history as well as findings of the physical and endoscopic examinations, imaging studies, and laboratory testing. There was no evidence of other pathologies. Histopathological results from cerebral biopsy led us to consider the intracranial pathology as an extension of the CIMDL. CIMDL is the result of a necrotizing inflammatory tissue response triggered by cocaine abuse in a subset of predisposed patients. The reported case is the first CIMDL consistent with brain extension mimicking a tumor-like lesion. While the presence of atypical ANCA seems to promote and/or define the disease phenotype, the specific role of these and other circulating autoantibodies needs further investigation.

Lens and Intraocular Lens-Induced Uveitis; Clinic, Diagnosis, and Treatment

Lens-induced uveitis is a rare form of uveitis. It is an autoimmune phenomenon secondary to lens proteins that go out of the lens capsule due to exogenous or iatrogenic (surgical) trauma. The classic history and eye examination findings are typical for diagnosis. For the treatment, surgery is usually chosen in the forefront. Intraocular lens-induced uveitis is a rare form of uveitis that can be seen as a result of micro-and macro trauma of intraocular lens implants to intraocular tissues (iris, ciliary body, iridocorneal angle, etc.). Although this entity can be regressed by medical treatment, the definitive treatment is the removal or repositioning of the intraocular lens according to the etiological event. With the development of minimal traumatic surgical techniques and the use of new intraocular lens implants, its frequency decreases. The aim of this review is to summarize the general features, diagnosis, and treatment of uveitis due to lenses and intraocular lenses.

Morphea after Borrelia-induced facial nerve palsy

Morphea, also known as localized scleroderma, is characterized by inflammation and fibrosis of the skin. The exact pathogenesis of morphea is unknown, but generally includes genetic predisposition to autoimmunity combined with an environmental insult. Previous cases have been associated with active Borrelia infection; however, Borrelia infection as a direct cause of morphea was not generalizable to most patients. Within endemic areas, Borrelia burgdorferi is the most common cause of facial nerve paralysis, another autoimmune phenomenon. We report a case of facial morphea in a young man with family history of autoimmune disease who developed morphea in the same location as two previous episodes of Borrelia-induced facial nerve palsy. This case is remarkable because it suggests Borrelia burgdorferi induced loss of local immune tolerance to host antigens, first with facial nerve palsy and followed years later by development of morphea.

Drug-Induced Lupus Erythematosus Associated with Proton Pump Inhibitor

Drug-induced lupus erythematosus is an autoimmune phenomenon where the drug exposure leads to the development of systemic lupus erythematous like clinical features. Drug-induced lupus erythematosus can be divided into systemic lupus erythematous, subacute cutaneous lupus erythematous, and chronic cutaneous lupus erythematous. Here, we report a case of a 29-year-old female presented with systemic lupus erythematous due to chronic use of proton pump inhibitors, which is considered to be very rare.

Autoimmune Complications in Chronic Lymphocytic Leukemia in the Era of Targeted Drugs

Autoimmune phenomena are frequently observed in patients with chronic lymphocytic leukemia (CLL) and are mainly attributable to underlying dysfunctions of the immune system. Autoimmune cytopenias (AIC) affect 4–7% of patients with CLL and mainly consist of autoimmune hemolytic anemia and immune thrombocytopenia. Although less common, non-hematological autoimmune manifestations have also been reported. Treatment of CLL associated AIC should be primarily directed against the autoimmune phenomenon, and CLL specific therapy should be reserved to refractory cases or patients with additional signs of disease progression. New targeted drugs (ibrutinib, idelalisib and venetoclax) recently entered the therapeutic armamentarium of CLL, showing excellent results in terms of efficacy and became an alternative option to standard chemo-immunotherapy for the management of CLL associated AIC. However, the possible role of these drugs in inducing or exacerbating autoimmune phenomena still needs to be elucidated. In this article, we review currently available data concerning autoimmune phenomena in patients with CLL, particularly focusing on patients treated with ibrutinib, idelalisib, or venetoclax, and we discuss the possible role of these agents in the management of AIC.