Autoimmune Phenomena Research Articles

A 15-Year Review of Ocular Manifestations of Autoimmune Inflammatory Disorders in a University Hospital

Background: Autoimmune diseases represent a significant health burden for 3% to 9% of the general population, and rheumatology, perhaps more than any other medical subspecialty, encompasses a broad array of such diseases that affect a wide range of organ systems including the eye. It serves as a sensitive barometer of autoimmune phenomena in many of the rheumatic diseases. Objective: The study determined the various ocular manifestations of all autoimmune inflammatory disorders at the University of Santo Tomas Hospital, Section of Rheumatology and the Department of Ophthalmology from 2003 to 2018. Methodology: A retrospective cohort study was done involving chart review of patients from both private division and clinical division. Sex, age, duration of disease, medications given, and ocular manifestations were determined. Statistical analysis included frequencies, percentages, and logistic regression analysis. Results: Sixty-seven patients were included in the study. Of these, 58.2% (39/67) had ocular manifestations secondary to an established autoimmune disease. It was found out that 58.97% (23/39) had Vogt-Koyanagi-Harada syndrome. Other autoimmune diseases with ophthalmologic manifestations were systemic lupus erythematosus at 12.8% (5/39), ankylosing spondylitis at 10.25% (4/39), rheumatoid arthritis and Behcet’s syndrome at 5.12% (2/39), and granulomatosis with polyangiitis, scleroderma and psoriatic arthritis at 2.56% (1/39). Conclusion: In this population, the frequency of ocular manifestations of autoimmune diseases remained low with anterior uveitis as the most common. Close coordination between specialties is very important in the course of treatment and management to lessen its complications and related morbidity.

Autoimmune and inflammatory manifestations associated with acute myeloid leukemia with Trisomy 8-Case series and review of the literature.

AML can be associated with autoimmune or inflammatory phenomena (AIP) occurring prior, concomitantly, or after its diagnosis. Trisomy 8 is one of the most common cytogenetic abnormalities associated with AML. We describe three patients with AML, trisomy 8, and associated AIP and review the known literature on this association. All of our patients had major symptomatic relief when treated with leukemia-directed therapy and corticosteroids. AIP in AML may be an underdiagnosed phenomenon, particularly in patients with trisomy 8.

New Onset of Autoimmune Diseases Following COVID-19 Diagnosis.

There is growing evidence that coronavirus disease 2019 (COVID-19) can lead to a dysregulation of the immune system with the development of autoimmune phenomena. The consequence of this immune dysregulation ranges from the production of autoantibodies to the onset of rheumatic autoimmune disease. In this context, we conducted a systematic review to analyze the current data regarding the new-onset systemic and rheumatic autoimmune diseases in COVID-19 patients. A literature search in PubMed and Scopus databases from December 2019 to September 2021 identified 99 patients that fulfilled the specific diagnostic/classification criteria and/or nomenclature for each rheumatic autoimmune disease. The main diseases reported were vasculitis and arthritis. Idiopathic inflammatory myopathies, systemic lupus erythematosus, and sarcoidosis were also reported in a limited number of patients, as well as isolated cases of systemic sclerosis and adult-onset Still’s disease. These findings highlight the potential spectrum of systemic and rheumatic autoimmune diseases that could be precipitated by SARS-CoV-2 infection. Complementary studies are needed to discern the link between the SARS-CoV-2 and new onset-rheumatic diseases so that this knowledge can be used in early diagnosis and the most suitable management.

Coxiella burnetii Infection Associated With Thromboangiitis Obliterans-like Phenomena With Digital Autoamputation: A Case Report and Review of Q Fever-Associated Autoimmunity.

We present a case of a patient with chronic Q fever who presented with digital necrosis, autoamputations, and positive anticentromere antibody, mimicking a scleroderma vasculopathy or thromboangiitis obliterans. Coxiella burnetii infection has long been associated with the presence of autoantibodies and autoimmune phenomena including vasculitis. Clinicians should consider Q fever testing in patients with new-onset autoimmune diseases or autoantibodies and appropriate exposure histories.

COVID-19 Vaccine Does Not Increase the Risk of Disease Flare-Ups among Patients with Autoimmune and Immune-Mediated Diseases.

Background: Reports describing post-vaccine autoimmune phenomena, in previously healthy individuals, increased the concerns regarding the risk of disease flare-ups in patients with immune diseases. We aimed to assess the potential risk of disease flare-up, after receiving the COVID-19 (Coronavirus disease 2019) vaccine, during a follow-up period of 6 months. Methods: We performed a prospective cohort study, enrolling the patients with autoimmune- and immune-mediated diseases who voluntarily completed our questionnaire, both online and during hospital evaluations. Based on their decision to receive the vaccine, the patients were divided into two groups (vaccinated and non-vaccinated). Participants who chose not to receive the vaccine served as a control group in terms of flare-ups. Results: A total of 623 patients, 416 vaccinated and 207 non-vaccinated, were included in the study during hospital evaluations (222/623) and after online (401/623) enrolment. There was no difference concerning the risk of flare-up between vaccinated and non-vaccinated patients (1.16, versus 1.72 flare-ups/100 patients-months, p = 0.245). The flare-ups were associated with having more than one immune disease, and with a previous flare-up during the past year. Conclusions: We did not find an increased risk of flare-up following COVID-19 vaccination in patients with autoimmune-/immune-mediated diseases, after a median follow-up of 5.9 months. According to our results, there should not be an obvious reason for vaccine hesitancy among this category of patients.

Helicobacter pylori Resistance to Triple Therapy in a Multicultural Population in New York City.

Introduction: Helicobacter pylori infection (HPI) has become a worldwide concern due to its associations with intestinal and extraintestinal disease including cancer, autoimmune phenomena, and vitamin deficiencies. HPI has been found to affect Hispanics at higher rates compared with non-Hispanics in the USA. Hispanics comprise most of the patient population at Metropolitan Hospital in New York City. Growing concerns about antibiotic resistance led to the reconciliation of treatment guidelines with the consensus of bismuth quadruple therapy as the first-line treatment, replacing clarithromycin-based triple therapy. We conducted a retrospective study to explore the resistance rate of Helicobacter pylori to triple therapy in patients at Metropolitan Hospital.Objective: To explore the resistance rates of Helicobacter pylori in infected patients treated with clarithromycin-based triple therapy in Metropolitan Hospital over a five-year period.Materials and Methods: Charts of all patients who underwent upper endoscopy during a five-year period were retrospectively reviewed. Overall, 2000 patients were screened for presence of HPI. We included 322 patients with a demonstrated HPI obtained from biopsies taken during upper endoscopy within the study period. Inclusion criteria were patients older than 18 years old with positive HPI who were prescribed therapy. Exclusion criteria were patients with positive HPI who did not receive treatment for the infection and patients without a confirmatory diagnosis of infection. We further reported on three groups based on the implemented therapy. Each treated group was divided into three subgroups based on eradication testing. Treatment compliance was documented. The patient population was demographically characterized by ethnicity, age at diagnosis, body mass index (BMI), and sex.Results: Of the 322 patients included in the study, 258 were Hispanics (80%). The eradication rate among patients treated with selected clarithromycin-based therapies was found to be statistically significant when compared with other HPI therapies. There was no statistically significant difference between the studied group with respect to age, sex, ethnicity, and BMI. In the group of patients with suspected clarithromycin resistance, antimicrobial sensitivity testing was ordered in one case.Discussion: HPI varies with race and ethnicity. Within the USA, the prevalence is lowest among non-Hispanics. Ethnicity and age, sex, and BMI were not factors that impacted treatment outcomes. We found that triple therapy with a proton pump inhibitor, amoxicillin, and clarithromycin (PAC) was used as a first-line treatment, consistently showing a low rate of resistance. The eradication rate among patients treated with PAC was found to be statistically significant when compared with all other therapies. It is significant for the hospitals with limited resources, where initial treatment follows the “test-and-treat” strategy. Quadruple therapy as the first-line treatment raises concerns about medication costs, insurance coverage, side effects, and dosing, which may have a significant impact on patient compliance.Conclusion: Our study showed that selected clarithromycin-based therapies were superior for HPI eradication when compared with non-clarithromycin-based triple therapy in low-resistance communities. Culture with antimicrobial susceptibility testing was used in a de minimis number of cases, which raises awareness for future study.

Purinergic signalling in systemic sclerosis.

SSc is a chronic autoimmune rheumatic disease that involves numerous organs and presents major management challenges. The histopathologic hallmarks of SSc include vasculopathy, fibrosis and autoimmune phenomena involving both innate and adaptive immune systems. Purinergic signalling is a pathway that may be implicated in the pathophysiology of several of these disease manifestations. Extracellular purines are potent signalling mediators, which have been shown to be dysregulated in SSc. As examples, purines can exacerbate vasculopathy and provoke platelet dysfunction; as well as contributing to immune dysregulation. Elements of purinergic signalling further promote organ and tissue fibrosis in several disease models. Here, we provide an overview of extracellular purine metabolism in purinergic signalling and link disorders of these to the molecular pathology of SSc. We also discuss targeting the purinergic signalling and explore the translational applications for new therapeutic options in SSc.

Seronegative Erosive Arthritis Following SARS-CoV-2 Infection.

Coronavirus disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) affecting mostly the respiratory system, but several other organs and systems can be involved. Extrapulmonary manifestations and autoimmune phenomena following SARS-CoV-2 infection are frequent events occurring during the first 2 weeks or in later stages of the disease course. These can be expressed as an isolated discovery of autoantibodies, mostly antinuclear or antiphospholipid antibodies, through to full-blown autoimmune organ-specific and systemic diseases. Joint pain is a frequent complain in most patients, but to our knowledge, frank arthritis has not been reported so far. A 46-year-old woman developed symmetrical polyarthritis 2 months after SARS-CoV-2 infection. Laboratory tests showed high acute phase reactants, while the immunological profile was negative. Hand and wrists X-rays revealed soft tissue swelling as well as bone erosions at the ulnar base of the third and fourth metacarpophalangeal joint of the right hand and carpal bones. The patient responded well to small doses of prednisone and methotrexate and after 4 months she had a sustained clinical and laboratory improvement. This is the first report making an association between SARS-CoV-2 infection and erosive polyarthritis. Physicians dealing with patients infected from SARS-CoV-2 should be aware for the possible development of musculoskeletal disorders, among them symmetrical polyarthritis. Thus, a close follow-up and monitoring is mandatory.

COVID-19 vaccination can occasionally trigger autoimmune phenomena, probably via inducing age-associated B cells.

COVID-19 vaccination can occasionally trigger autoimmune phenomena, probably via inducing age-associated B cells.

SOCS1 Haploinsufficiency Presenting As Incidental Refractory Thrombocytopenia in a Pediatric Patient with Inflammatory Symptoms and History of Sars Cov-2 Infection

BACKGROUNDImmune thrombocytopenia purpura (ITP) has a complex pathogenesis and may be a primary diagnosis or secondary to an underlying condition 1. Evaluation for underlying diagnoses in patients presenting with atypical features of classic ITP is key, as this can impact treatment decisions, therapy response, and prognosis. Genetic variants that predispose patients to ITP are especially important to investigate as patients may be at risk for additional autoimmune phenomenon or malignancy. The SARS CoV-2 pandemic has added further complexity as reports suggest the infection can lead to autoimmunity in those with genetic predispositions 2,3. Loss of the suppressor of cytokine signaling 1 (SOCS1) function has been described to manifest with autoinflammatory syndrome, with or without immunodeficiency 4,5. Reports of autoimmunity developing in patients with SOCS1 haploinsufficiency after SARS CoV-2 infection are documented, including multi-system inflammatory syndrome (MIS-C) 2. A proposed mechanism of this virus-triggered autoimmunity includes a transient innate and adaptive immunodeficiency 3. This raises the question whether patients harboring genetic variants with risk of autoimmunity are placed at an even higher risk for ITP in the wake of SARS-CoV2 infection. CASE PRESENTATIONWe present a 6-year-old female with isolated thrombocytopenia of 4,000/uL identified during evaluation for severe arthralgias unresponsive to corticosteroid treatment (maximum dose 1mg/kg/day) over a 6-month period. Laboratory results at presentation were consistent with ITP, including presence of platelet autoantibodies. Evaluation revealed hypocellularity for age (~50%) on bone marrow evaluation as well as elevated IgE (2080 kU/L), with IgA, IgM, and IgG levels within reference range. She had a remote history of SARS CoV-2-like illness and SARS CoV-2 antibodies were found present in serologic assay, without a history of vaccination. Genetic testing, including chromosomal microarray from peripheral blood and marrow, was included in the diagnostic workup given concern for a history of developmental delays with macrocephaly and necessity to rule-out malignancy with the patient noted to have a 5 mega-base deletion at 16p13.2p13.11, which includes the SOCS1 gene. Comprehensive next generation sequencing for additional immune dysregulation/primary immunodeficiency associated variants was unremarkable. Functional studies of surface expression of interferon-inducible genes (CD169 (SIGLEC-1)) and STAT1 phosphorylation via analysis of CD14+ monocytes revealed excess interferon signaling previously described in patients with SOCS1 haploinsufficiency (Figure 1). Measurements of B-cell-activating factor were also found to be extremely elevated at 6432 pg/mL. The patient's ITP course was complicated by hematuria, melena and refractory platelet response to first line therapy consisting of intravenous immunoglobulin 1 g/kg x2 doses and 2 mg/kg/day prednisolone. She required escalation to high dose methylprednisolone (30mg/kg), rituximab 375 mg/m 2/weekly x4 doses, and concurrent romiplostim (2 doses) for control of thrombocytopenia and bleeding manifestations. Her rheumatologic symptoms subsided with initiation of corticosteroids, and she has subsequently completed a prolonged corticosteroid taper. She currently has a normal platelet count with non-steroidal anti-inflammatory therapy utilized for arthralgia management with plan to transition to JAK inhibition for maintenance therapy. CONCLUSIONThis case highlights the potential impact of investigating for susceptibility genes for ITP with consideration for broader testing including targeted next generation sequencing panels or microarray analysis in patients with atypical ITP presentations or response to therapy, as knowledge of this patient's underlying genetics led to earlier treatment and use of alternative agents. Additionally, the case adds the novel finding of bone marrow hypocellularity to the clinical phenotype of SOCS1 haploinsufficiency, as this has not yet been reported and contributes to the literature on the relationship of autoimmunity and SARS CoV-2 infections in patients with predisposing genetic variants. [Display omitted] DisclosuresWalkovich: Horizon Pharmaceuticals: Honoraria, Membership on an entity's Board of Directors or advisory committees; Pharming: Honoraria, Membership on an entity's Board of Directors or advisory committees; Swedish Orphan Biovitrum AB (Sobi): Consultancy, Honoraria; X4 Pharmaceuticals: Other: Local PI for clinical trial involving mavorixafor and patients with neutropenia.

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

Splenic kaposiform hemangioendothelioma presenting as insidious consumptive coagulopathy

CD4+ cells in autoimmune thyroid disease.

The phenomenon of autoimmunity develops as a result of the triggering factor released by damaged cells. This leads to an infiltration of CD4+ cells involved in stimulating the effector cells cytotoxicity and stimulating the humoral response. One of the most common autoimmune disorders are autoimmune thyroid diseases, including Hashimoto's thyroiditis and Graves's diseases. Helper T lymphocytes, which are divided into Th1, Th2, Tregs, and the relatively new groups Th17, Th22, and Th9, are involved in the pathogenesis of AITD. CD4+ cell subtypes mature and differentiate by specific transcription factors and in a specific interleukin environment. Not only are Th1 and Th2 cells involved in the development of AITD, but also Th17, Th22, and Th9 lymphocytes and their correlation to Tregs lymphocytes. The plasticity of the CD4+ cells is very important, affecting the balance between these cells, as well the factors modulating their phenotypic variability. Patients with AITD have an increased percentage of Th17, Th22, and Th9 cells as well as defective function of Tregs lymphocytes. The balance between Th17 cells (and also other cytotoxic T cells) and Treg cells is also very important. Understanding the role of CD4 cells in the pathogenesis of AITD may be important not only for the development of the knowledge, but also for determining therapeutic targets.

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.

S2153 A Case of Proton Pump Inhibitor Causing Systemic Drug-Induced Lupus

Introduction: Drug induced lupus erythematosus (DILE) is a well documented autoimmune phenomenon that can be triggered by exposure to certain medications. Presented here is a case of systemic drug induced lupus erythematosus from a proton pump inhibitor (PPI), which represents a fairly uncommon reaction to this medication. Case Description/Methods: A 63 year old female with history of gastroesophageal reflux disease (GERD) presented to clinic complaining of typical heartburn pain and globus sensation for which she was prescribed pantoprazole. After 2 weeks of daily use, she developed pain and swelling of bilateral metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints, photosensitive malar rash, patchy alopecia, ‘gnawing’ diffuse abdominal pain (different than GERD symptoms), and oral ulcers. Her pantoprazole was stopped, and 2 weeks later her symptoms resolved. Review of the her medical record revealed that she had prior exposure to 2 other PPI's in the past that both were associated with development of similar symptoms. Use of omeprazole caused pain and swelling of her right MCP joints, and several years later use of esomeprazole caused severe pain and swelling of the left elbow. In both cases, discontinuation of her PPI caused resolution of symptoms. Her labs were notable for positive anti histone antibody and negative ANA, dsDNA, RF, anti CCP. Discussion: DILE has three basic variants; systemic, subacute cutaneous, and chronic cutaneous. The type of drug induced lupus and severity of symptoms is strongly associated with the specific drug of exposure. PPI's are most associated with subacute cutaneous DILE, although this remains an overall rare adverse reaction. Subacute cutaneous DILE presents with formation of annular or papulosquamous skin lesions and mild (if any) systemic symptoms. Our case is more consistent with systemic DILE. Review of literature reveals few case reports of systemic DILE from PPI use. Systemic DILE presents with multi-system involvement with constitutional symptoms, myalgias, arthralgias, and serositis. Symptoms resolve with withdrawal of the agent. The case above clearly demonstrates this illness script through multiple exposures to PPI. Classic SLE is ruled out in this case due to this temporal relationship with drug exposure, as well as her antibody profile positive for anti-histone and negative for other SLE-associated antibodies. This case highlights the importance of considering atypical drug reactions, even when prescribing relatively common medications.

Hemolytic Anemia in Inflammatory Bowel Disease: Our "Gut" Tells Us to Blame the Drug.

Several studies determined that autoimmune hemolytic anemia (AIHA) is a rare extraintestinal IBD manifestation, related to the underlying disease activity. However, evidence linking biologic therapy to AIHA is sparse. This article reviews the evidence on the association of these clinical phenomena. There are two retrospective studies and a few case reports linking biologic therapies to AIHA. While some autoimmune phenomenon triggered by our biologic therapies have been well recognized, we do not find the evidence associating these therapies to AIHA sufficiently compelling.

GUILLAIN-BARRÉ SYNDROME AND HEMOLYSIS IN COVID-19

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: The spectrum of autoimmune phenomena associated with Coronavirus disease 2019 (COVID-19) continues to expand. Guillain-Barre Syndrome (GBS) manifesting as a post-infectious sequela of COVID-19 has been increasingly reported [1]. Rare cases of autoimmune hemolytic anemia (AIHA) associated with COVID-19 are also emerging [2]. Our case describes a novel presentation of GBS and hemolysis in a patient with COVID-19. CASE PRESENTATION: A 62-year-old male with a past medical history of hypertension initially presented with 5 days of low-grade fever and cough. He denied dyspnea and was not hypoxic. Nasopharyngeal PCR confirmed COVID-19. He received bamlanivimab and etesevimab infusion and was discharged from the emergency room. He returned 6 days later with new-onset unsteady gait and weakness of the lower extremities. He had 1+ reflexes in the patellar and ankle bilaterally and 3+ in both triceps and biceps. This worsened into flaccid paralysis of bilateral lower extremities by admission day 2. Labs showed a drop in hemoglobin (Hgb) from 14.7 mg/dL to 10.9 mg/dL, haptoglobin 2) at time of discharge [1, 3]. AIHA has also been associated with COVID-19, with mixed warm and cold agglutin formation [2]. A negative CSF PCR panel with high protein suggests that novel antibodies may mediate myelin damage, which may also target other components such as fibrinogen in α/β chains. Immunoglobulin fragment alteration resulting in molecular mimicry may also occur following antigen-antibody complex formation directed against the virus [1]. CONCLUSIONS: GBS and AIHA are rare but important complications in patients with COVID-19. The spectrum of autoimmune phenomena associated with COVID-19 continues to expand. This case highlights the need for the differential to include autoimmune disease in patients with extrapulmonary manifestations of COVID-19. Further studies are needed to determine if causal relationships are present. REFERENCE #1: Sheikh AB, Chourasia PK, Javed N, et al. Association of Guillain-Barre syndrome with COVID-19 infection: An updated systematic review. 2021 Apr 18. J Neuroimmunol. 2021;355:577577. doi:10.1016/j.jneuroim.2021.577577 REFERENCE #2: Lazarian G, Quinquenel A, Bellal M, et al. Autoimmune haemolytic anaemia associated with COVID-19 infection. Br J Haematol. 2020;190(1):29-31. doi:10.1111/bjh.16794 REFERENCE #3: Hasan I, Saif-Ur-Rahman KM, Hayat S, et al. Guillain-Barre syndrome associated with SARS-CoV-2 infection: A systematic review and individual participant data meta-analysis. J Peripher Nerv Syst. 2020 Dec;25(4):335-343. doi: 10.1111/jns.12419. DISCLOSURES: No relevant relationships by Mateus Fernandes, source=Web Response No relevant relationships by Ifediba Nwachukwu, source=Web Response

Immune Phenomena in Myeloid Neoplasms: An "Egg or Chicken" Question.

Immune phenomena are increasingly reported in myeloid neoplasms, and include autoimmune cytopenias/diseases and immunodeficiency, either preceding or complicating acute myeloid leukemia, myelodysplastic syndromes (MDS), chronic myeloproliferative neoplasms, and bone marrow failure (BMF) syndromes. Autoimmunity and immunodeficiency are the two faces of a dysregulated immune tolerance and surveillance and may result, along with contributing environmental and genetic factors, in an increased incidence of both tumors and infections. The latter may fuel both autoimmunity and immune activation, triggering a vicious circle among infections, tumors and autoimmune phenomena. Additionally, alterations of the microbiota and of mesenchymal stem cells (MSCs) pinpoint to the importance of a permissive or hostile microenvironment for tumor growth. Finally, several therapies of myeloid neoplasms are aimed at increasing host immunity against the tumor, but at the price of increased autoimmune phenomena. In this review we will examine the epidemiological association of myeloid neoplasms with autoimmune diseases and immunodeficiencies, and the pivotal role of autoimmunity in the pathogenesis of MDS and BMF syndromes, including the paroxysmal nocturnal hemoglobinuria conundrum. Furthermore, we will briefly examine autoimmune complications following therapy of myeloid neoplasms, as well as the role of MSCs and microbiota in these settings.

Differences in autoimmunity factors based on the activity of thromboangiitis obliterans.

The treatment of Buerger's disease (BD) presents a medical problem as its etiology is still unclear. In this study, our objective was to evaluate the serum levels of autoimmune markers in patients with different clinical features of BD. In this study, 80 BD patients were categorized in three groups using a cross-sectional design: migratory thrombophlebitis, cold sensitivity, and skin discoloration (mild symptoms); chronic ulcers, claudication, and burning pain of the feet at night (moderate symptoms); pain at rest and spontaneous gangrene (severe symptoms). Enzyme-linked immunosorbent assay was performed to measure antibodies against immunoglobulin M rheumatoid factor (IgM RF), anti-nuclear antibodies (ANA), antibodies against cyclic citrullinated peptide (anti-CCP), antiphospholipid antibodies (APA), anti-cardiolipin antibodies (ACLA), anti-double stranded DNA (anti-dsDNA), and extractable nuclear antigen (ENA) profile. Patients with severe symptoms showed the lowest age (p=0.031), ESR (p<0.001), and highest prevalence of ischemia (p<0.001). In all the patients, the serum levels of ANA and IgM RF were higher than 1 U and 15 IU/mL, respectively. However, the progression of the disease from mild to moderate did not affect these markers significantly (p>0.05). Other markers were negative in patients with BD. The findings of this study indicate that BD may closely be correlated to transient autoimmune phenomena, despite the fact that further research is required to investigate how transient unspecific autoimmune reactions contribute to the BD pathogenesis.

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.&#x0D; 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. &#x0D; 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.&#x0D; 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. &#x0D; 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.

Acquired thrombotic thrombocytopenic purpura: A rare disease associated with BNT162b2 vaccine

BackgroundIn December 2020 the Israeli Health Ministry began a mass vaccination campaign with the BNT162b2 vaccine. This was an important step in overcoming the severe acute respiratory syndrome corona virus 2 (SARS‐CoV‐2) pandemic. Autoimmune phenomenon have been described after receiving vaccinations. Patients/MethodsHere we describe a case series of patients who developed acquired Thrombotic Thrombocytopenic Purpura, a rare autoimmune disease, within several days of receiving the BNT162b2 vaccine. ConclusionsA disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity should be evaluated in patients with history of aTTP before and after any vaccination, especially the SARS‐CoV‐2 vaccination, and immunosuppression treatment should be considered before vaccination in cases of low ADAMTS13 activity. Patients should be closely monitored after the vaccine for clinical situation and laboratory data. Post vaccination thrombocytopenia assessment should include immune thrombocytopenic purpura, vaccine‐induced immune thrombotic thrombocytopenia and acquired thrombotic thrombocytopenic purpura.