Age: 56.0
Sex: M
Vaccine: COVID19
Symptoms: Diarrhoea, HLA marker study, Haematology test, Haemoglobin, Haptoglobin
Clinical narrative: VEXAS syndrome; Limited range of motion at joint; Fever; Vaccination site tenderness; swelling at the injection site; Nausea; Vomiting; Diarrhea; This literature-non-study case was reported in a literature article and describes the occurrence of VEXAS SYNDROME (VEXAS syndrome), JOINT RANGE OF MOTION DECREASED (Limited range of motion at joint), DIARRHOEA (Diarrhea), PYREXIA (Fever), NAUSEA (Nausea), VOMITING (Vomiting), VACCINATION SITE PAIN (Vaccination site tenderness) and VACCINATION SITE SWELLING (swelling at the injection site) in a 56-year-old male patient who received mRNA-1273 (Moderna COVID-19 Vaccine) for COVID-19 vaccination.       The patient's past medical history included Pancytopenia (pancytopenia (persisting transfusion-dependency)), Splenomegaly and Blood transfusion (Patient was hospitalized and received multiple blood transfusions). Previously administered products included for Low blood pressure: hydrocortisone (Patient was also started on stress dose steroids for his low blood pressure with hydrocortisone and which was later tapered to prednisone.). Past adverse reactions to the above products included Low blood pressure with hydrocortisone. Concurrent medical conditions included Arthritis, Polychondritis, Splenic infarction (splenic infarctions on Eliquis) and Non-smoker (Patient denied any use of smoking of cigarettes). Concomitant products included APIXABAN (ELIQUIS) for Antiphospholipid syndrome, METHOTREXATE for Connective tissue disorder, PREDNISONE for Scleritis and Polychondritis.   On an unknown date, the patient received third dose of mRNA-1273 (Moderna COVID-19 Vaccine) (unknown route) 1 dosage form. On an unknown date, received second dose of mRNA-1273 (Moderna COVID-19 Vaccine) (unknown route) dosage was changed to 1 dosage form and first dose of mRNA-1273 (Moderna COVID-19 Vaccine) (unknown route) dosage was changed to 1 dosage form. On an unknown date, the patient experienced VEXAS SYNDROME (VEXAS syndrome) (seriousness criterion hospitalization), JOINT RANGE OF MOTION DECREASED (Limited range of motion at joint) (seriousness criterion hospitalization), DIARRHOEA (Diarrhea) (seriousness criterion hospitalization), PYREXIA (Fever) (seriousness criterion hospitalization), NAUSEA (Nausea) (seriousness criterion hospitalization), VOMITING (Vomiting) (seriousness criterion hospitalization), VACCINATION SITE PAIN (Vaccination site tenderness) (seriousness criterion hospitalization) and VACCINATION SITE SWELLING (swelling at the injection site) (seriousness criterion hospitalization). The patient was treated with SULFAMETHOXAZOLE, TRIMETHOPRIM (BACTRIM) for Adverse event, at an unspecified dose and frequency. At the time of the report, VEXAS SYNDROME (VEXAS syndrome), JOINT RANGE OF MOTION DECREASED (Limited range of motion at joint), VACCINATION SITE PAIN (Vaccination site tenderness) and VACCINATION SITE SWELLING (swelling at the injection site) was resolving and DIARRHOEA (Diarrhea), PYREXIA (Fever), NAUSEA (Nausea) and VOMITING (Vomiting) had resolved.      DIAGNOSTIC RESULTS (normal ranges are provided in parenthesis if available): On an unknown date, Angiotensin converting enzyme (9-67): 22 u/l (normal) 22 U/L. On an unknown date, Anti-cyclic citrullinated peptide antibody (0-30): 22 u (normal) 22 U. On an unknown date, Antineutrophil cytoplasmic antibody (Unknown-less than 1:20): negative (Negative) Negative. On an unknown date, Antinuclear antibody: non-reactive Non-reactive. On an unknown date, Antiphospholipid antibodies: negative (Negative) Lupus anticoagulant were negative. On an unknown date, Beta-2 glycoprotein antibody (Unknown-less than 20): 76.8 u/ml (High) 76.8  U/mL Autoimmune workup revealed elevated levels of B2GP1 (beta-2 glycoprotein 1) immunoglobulin (Ig) M antibody, which raised the concern for APLS (antiphospholipid syndrome) as the cause of the unprovoked splenic infracts. On an unknown date, Biopsy bone marrow: hypercellular bone marrow Bone marrow biopsy, which showed a hypercellular bone marrow with relative myeloid hyperplasia and megakaryocyte dysplasia (relative expansion of the gamma delta T-cell subpopulation associated with clonally expanded rearrangement of the T-cell receptor beta [TRB] locus) with vacuoles in myeloid and erythroid cells.. On an unknown date, Blood lactate dehydrogenase: unremarkable lactate dehydrogenase were unremarkable and not suggestive of hemolytic anemia. On an unknown date, C-reactive protein (0.00-7.30): 309.80 mg/l (High) 309.80 mg/L C-reactive protein were elevated. On an unknown date, C1 esterase inhibitor test (21-39): 20 mg/dl 20 mg/dL. On an unknown date, Cardiolipin antibody (Unknown-less than 20): negative (Negative) anticardiolipin antibodies were negative and 19 gpl-u/ml 19 GPL-U/mL. On an unknown date, Computerised tomogram: mediastinal lymphadenopathy Patient was evaluated by the pulmonology service because of mediastinal lymphadenopathy noticed on a computed tomography (CT) scan.. On an unknown date, Computerised tomogram abdomen: worsening splenomegaly CT of his abdomen showed worsening splenomegaly with splenic infarcts. On an unknown date, Coombs direct test: unremarkable The hemolytic panel, including direct and indirect antiglobulin testing were unremarkable. On an unknown date, Coombs indirect test: unremarkable The hemolytic panel, including direct and indirect antiglobulin testing were unremarkable. On an unknown date, HLA marker study: negative (Negative) Negative. On an unknown date, Haematology test: normocytic normochromic red blood cells Hematological workup performed before admission for pancytopenia was consistent for a blood smear with normocytic normochromic red blood cells (RBCs), teardrop cells, and rare schistocytes.. On an unknown date, Haemoglobin (14.0-18.0): 11.8 g/dl (Low) 11.8 g/dL. On an unknown date, Haptoglobin: unremarkable The hemolytic panel, including were unremarkable. On an unknown date, Immune complex assay (Unknown-less than 25.2): less than 1.2 mcg eq/ml (normal) less than 1.2 mcg Eq/mL. On an unknown date, Inflammatory marker test: elevated (High) Inflammatory marker were elevated. On an unknown date, Magnetic resonance imaging heart: no evidence of gadolinium cardiac magnetic resonance imaging showed no evidence of gadolinium enhancement within the myocardium to suggest infiltrative processes such as sarcoidosis. On an unknown date, Mean cell volume (75-100): 112.9 fl (High) 112.9 fL. On an unknown date, Platelet count (150-450): 47×1000/µl (Low) 47×1000/µL. On an unknown date, Platelet morphology: normal (normal) normal. On an unknown date, Procalcitonin (0.05-0.5): 155.87 ng/ml (High) 155.87 ng/mL Pan-cultures did not yield any pathogen despite an elevated procalcitonin, making distributive-anaphylactic shock the most likely cause of hypotension. On an unknown date, Red blood cell sedimentation rate (0-15): 41 mm/h (High) 41 mm/h. On an unknown date, Rheumatoid factor (0-30): 22 iu/ml (normal) 22 IU/mL. On an unknown date, Treponema test (0.0-0.8): 0.2ai (normal) 0.2AI. On an unknown date, Ultrasound scan: deep venous thrombosis ultrasound of his extremities ruled out any deep venous thrombosis. On an unknown date, White blood cell analysis: evidence of premature forms white blood cell analysis did not show any evidence of premature forms. On an unknown date, White blood cell count (4-11): 11.3× 1000/µl (High) 11.3 × 1000/µL.     For mRNA-1273 (Moderna COVID-19 Vaccine) (Unknown), the reporter considered VEXAS SYNDROME (VEXAS syndrome), JOINT RANGE OF MOTION DECREASED (Limited range of motion at joint), DIARRHOEA (Diarrhea), PYREXIA (Fever), NAUSEA (Nausea), VOMITING (Vomiting), VACCINATION SITE PAIN (Vaccination site tenderness) and VACCINATION SITE SWELLING (swelling at the injection site) to be related.    Company comment: This literature non-study case concerns a 56 year old male patient with relevant medical history of  arthritis, polychondritis, pancytopenia (persisting transfusion-dependency), splenomegaly, splenic infarctions on Eliquis, orbital inflammatory syndrome of unclear etiology and nonischemic heart failure with reduced ejection fraction (ejection fraction of 35 percent), who experienced the unexpected serious (hospitalization) events of VEXAS syndrome (latency unknown), Joint range of motion decreased, Diarrhoea, Nausea, Vomiting, Vaccination site pain and Vaccination site swelling after receiving the third dose of mRNA-1273 vaccine in the COVID-19 vaccination series. He presented to the emergency department (ED) for fever, nausea, vomiting, and diarrhea of one-day duration. He received the Moderna COVID-19 booster, after which he developed significant tenderness along with swelling at the injection site. He stated that he had similar symptoms after his second COVID-19 vaccination six months prior to presentation. He had recently traveled, where he was hospitalized and received multiple blood transfusions. His investigations included: Angiotensin converting enzyme- 22 U/L; Anti-cyclic citrullinated peptide antibody-22 U; Antineutrophil cytoplasmic antibody-Negative; Antinuclear antibody;;Non-reactive; Antiphospholipid antibodies-negative; Lupus anticoagulant- negative; Beta-2 glycoprotein antibody-76.8 U/mL; Autoimmune workup revealed elevated levels of B2GP1 (beta-2 glycoprotein 1), immunoglobulin (IgM)-raised; Biopsy bone marrow-hypercellular bone marrow with relative myeloid hyperplasia and megakaryocyte dysplasia (relative expansion of the gamma delta T-cell subpopulation associated with clonally expanded rearrangement of the T-cell receptor beta [TRB] locus) with vacuoles in myeloid and erythroid cells; Blood lactate dehydrogenase;;unremarkable; C1 esterase inhibitor test-20 mg/dL; Cardiolipin antibody-19 GPL-U/mL; Computerised tom   Most recent FOLLOW-UP information incorporated above includes: On 05-May-2022: Follow up received by safety 06-May-2022 has Email with Agency received from team and contains significant information. Literature citation, author information, medical history, lab test, product information, event details updated.; Sender's Comments: This literature non-study case concerns a 56 year old male patient with relevant medical history of  arthritis, polychondritis, pancytopenia (persisting transfusion-dependency), splenomegaly, splenic infarctions on Eliquis, orbital inflammatory syndrome of unclear etiology and nonischemic heart failure with reduced ejection fraction (ejection fraction of 35 percent), who experienced the unexpected serious (hospitalization) events of VEXAS syndrome (latency unknown), Joint range of motion decreased, Diarrhoea, Nausea, Vomiting, Vaccination site pain and Vaccination site swelling after receiving the third dose of mRNA-1273 vaccine in the COVID-19 vaccination series. He presented to the emergency department (ED) for fever, nausea, vomiting, and diarrhea of one-day duration. He received the Moderna COVID-19 booster, after which he developed significant tenderness along with swelling at the injection site. He stated that he had similar symptoms after his second COVID-19 vaccination six months prior to presentation. He had recently traveled, where he was hospitalized and received multiple blood transfusions. His investigations included: Angiotensin converting enzyme- 22 U/L; Anti-cyclic citrullinated peptide antibody-22 U; Antineutrophil cytoplasmic antibody-Negative; Antinuclear antibody;;Non-reactive; Antiphospholipid antibodies-negative; Lupus anticoagulant- negative; Beta-2 glycoprotein antibody-76.8 U/mL; Autoimmune workup revealed elevated levels of B2GP1 (beta-2 glycoprotein 1), immunoglobulin (IgM)-raised; Biopsy bone marrow-hypercellular bone marrow with relative myeloid hyperplasia and megakaryocyte dysplasia (relative expansion of the gamma delta T-cell subpopulation associated with clonally expanded rearrangement of the T-cell receptor beta [TRB] locus) with vacuoles in myeloid and erythroid cells; Blood lactate dehydrogenase;;unremarkable; C1 esterase inhibitor test-20 mg/dL; Cardiolipin antibody-19 GPL-U/mL; Computerised tomogram-mediastinal lymphadenopathy; Computerised tomogram abdomen-worsening splenomegaly with splenic infarcts; The hemolytic panel, including direct and indirect antiglobulin testing were unremarkable; C-reactive protein- 309.80 mg/L; Hematological workup performed before admission for pancytopenia showed normocytic normochromic red blood cells (RBCs), teardrop cells, and rare schistocytes ; Haemoglobin-11.8 g/dL; Haptoglobin- unremarkable; HLA marker study- Negative; Immune complex assay-less than 1.2 mcg Eq/mL; Inflammatory marker -elevated; Magnetic resonance imaging heart- showed no evidence of gadolinium enhancement within the myocardium to suggest infiltrative processes such as sarcoidosis; Pan-cultures did not yield any pathogen despite an elevated procalcitonin; Red blood cell sedimentation rate-41 mm/hr; Rheumatoid factor-22 IU/mL; Treponema test-0.2AI; Ultrasound scan of his extremities ruled out any deep venous thrombosis; White blood cell analysis- did not show any evidence of premature forms; White blood cell count-11.3× 1000/µL. He failed to respond to disease-modifying antirheumatic drugs. He was treated with high dose steroids , anticoagulants and antibiotics. The events pyrexia, nausea, vomiting, and diarrhea had resolved and the other events were resolving. The benefit-risk relationship of mRNA-1273 is not affected by this report.