Risk Factors and Outcomes for COVID-19 in Autoimmune Inflammatory Diseases during the SARS-CoV-2 Pandemic: A Comparative Study.

BACKGROUND: Patients with autoimmune disease (AID) and coronavirus disease 2019 (COVID-19) could have higher mortality due to the co-morbidity and the use of immunosuppressive therapy. OBJECTIVES: To analyze the risk factors and outcomes of patients with AID and COVID-19 versus a control group. METHODS: A prospective cohort study included patients with and without AID and COVID-19. Patients were paired by age and sex. Clinical, biochemical, immunological treatments, and outcomes (days of hospital stay, invasive mechanical ventilation [IMV], oxygen at discharge, and death) were collected. RESULTS: We included 226 COVID-19 patients: 113 with AID (51.15 ± 14.3 years) and 113 controls (53.45 ± 13.3 years). The most frequent AIDs were Rheumatoid arthritis (26.5%), systemic lupus erythematosus (21%), and systemic sclerosis (14%). AID patients had lower lactate dehydrogenas, C-reactive protein, fibrinogen, IMV (P = 0.027), and oxygen levels at discharge (P ≤ 0.0001) and lower death rates (P ≤ 0.0001). Oxygen saturation (SaO2) ≤ 88% at hospitalization provided risk for IMV (RR [relative risk] 3.83, 95% confidence interval [95%CI] 1.1-13.6, P = 0.038). Higher creatinine and LDH levels were associated with death in the AID group. SaO2 ≤ 88% and CO-RADS ≥ 4 were risk factors for in-hospital mortality (RR 4.90, 95%CI 1.8-13.0, P = 0.001 and RR 7.60, 95%CI 1.4-39.7, P = 0.016, respectively). Anticoagulant therapy was protective (RR 0.36, 95%CI 0.1-0.9, P = 0.041). CONCLUSIONS: Patients with AID had better outcomes with COVID-19 than controls. Anticoagulation was associated with a lower death in patients with AID.

Atypical Kawasaki Disease after COVID-19 Vaccination: A New Form of Adverse Event Following Immunization.

Kawasaki disease (KD) is a medium-vessel vasculitis that is typically presented during childhood; fewer than 100 cases of KD have been reported worldwide in adult patients who met the criteria according to the American College of Rheumatology. This study presents the case of an 18-year-old patient with no previous history of any disease, who presented atypical KD with liver and kidney dysfunction, with a good response to intravenous immunoglobulin therapy. The symptoms began 22 days after the application of the COVID-19 vaccine (nonreplicating viral vector Vaxzevria), and other conditions were ruled out. The term Adverse Events Following Immunization (AEFI)encompasses all the reactions that follow the application of any vaccine with no necessary causal relationship and can be due to the vaccine product, quality of the vaccine, immunization errors, or anxiety or just happen to be coincident events. These reactions should be reported so that clinicians can identify compatible cases and consider that the presentation of this disease, despite being atypical, can be manifested in adult patients. Likewise, case reports are an important basis for the pharmacovigilance of vaccines.

Kikuchi-Fujimoto Disease: Report of a Case with Progression to Lupus Nephritis.

BACKGROUND Kikuchi-Fujimoto disease (KFD) is an enigmatic disease, with a distinctive histopathology and a benign and self-limited course. It is more frequent in young Asian women. Autoimmune diseases are identified as one of its triggers; primarily SLE, which may precede, be concomitant with, or develop after the diagnosis of KFD. Patients with KFD should receive periodic follow-up for several years to detect possible evolution of SLE. The main feature of KFD is lymphadenopathy, and cervical lymph nodes are involved in 50% to 98% of cases. Other symptoms such as fever, fatigue, weight loss, and arthralgias are also reported. Differential diagnosis between KFD and SLE is a challenge. When KFD and SLE coexist, a lymph node biopsy may be diagnostic. Treatment should be symptomatic with analgesics and anti-inflammatories, with complete resolution in 3 to 4 months. Corticosteroids and immunosuppressive therapy are justified only in cases concomitant with SLE. CASE REPORT We report a case of KFD in a 28-year-old woman who was initially negative for anti-nuclear antibodies (ANA) and anti-double-stranded deoxyribonucleic acid antibodies (anti-dsDNA), but who became antibody-positive and presented with lupus nephritis 2 months later. CONCLUSIONS We present a case of a patient with KFD who developed SLE 2 months later; highlighting the importance of recognizing its association and its possible progression to monitor for future development of SLE and provide timely treatment to avoid complications. We also compared the clinical, laboratory, and histological similarities between the 2 entities.

Generalized chronic erythema nodosum: to consider Weber-Christian disease. / Eritema nudoso crónico de localización generalizada: a considerar enfermedad de Weber-Christian.

BACKGROUND: Weber-Christian disease is a recurrent, non-suppurative, febrile nodular panniculitis, more frequent in young women. It is characterized by recurrent outbreaks of subcutaneous nodules, distributed symmetrically. It has an idiopathic origin, or it is secondary to pancreatic disease, physicochemical agents or alpha-1 antitrypsin deficiency. It can affect any tissue with body fat. Histologically is a lobular panniculitis without vasculitis. CASE REPORT: 23-year-old woman. She was admitted with subcutaneous painful nodules in pelvic, gluteus and forearms, with erythematous and hyperpigmented plaques; ecchymosis in the right upper eyelid and bilateral hyposphagma; fever of 38 °C; diaphoresis and oral ulcers, not painful. The hemogram showed pancytopenia, elevation of acute phase reagents, amylase and normal lipase. Blood culture was negative. Antinuclear antibodies and complement were normal. Computed tomography of the abdomen showed disseminated hyperdensity in subcutaneous tissue. The biopsy showed lobular panniculitis without vasculitis compatible with WeberChristian panniculitis. The patient was treated with steroids and immunosuppressive therapy. CONCLUSIONS: Infectious etiology and other causes of erythematous nodules were ruled out through clinical evolution and complementary studies. The patient presented spontaneous involution in several weeks and sometimes the lesions evolved to sterile abscesses, in addition to systemic clinical manifestations. Corticosteroid therapy was deemed successful.

INTRODUCCIÓN: La enfermedad de Weber-Christian es una paniculitis nodular recidivante, no supurativa, febril, más frecuente en las mujeres jóvenes. Se caracteriza por brotes recurrentes de nódulos subcutáneos, de distribución simétrica. Tiene un origen idiopático o es secundaria a enfermedad pancreática, agentes fisicoquímicos o déficit de alfa-1-antitripsina. Puede afectar cualquier tejido del organismo que tenga grasa. Histológicamente presenta paniculitis lobulillar sin vasculitis. CASO CLÍNICO: Mujer de 23 años que ingresó por nódulos subcutáneos dolorosos con equimosis en miembros pélvicos, glúteos y antebrazos, con placas eritematosas e hiperpigmentadas; equimosis en párpado superior derecho e hiposfagma bilateral; fiebre de 38 °C; diaforesis y úlceras orales no dolorosas. El hemograma mostró pancitopenia, elevación de los reactantes de fase aguda, y amilasa y lipasa normales. Los cultivos fueron negativos. Los anticuerpos antinucleares y el complemento fueron normales. La tomografía del abdomen presentó hiperdensidad en tejido graso subcutáneo diseminada. La biopsia reveló paniculitis lobulillar sin vasculitis, compatible con paniculitis de Weber-Christian. Se trató con esteroide e inmunosupresor. CONCLUSIONES: Se descartaron la etiología infecciosa y otras causas de nódulo eritematoso por la evolución clínica y los estudios complementarios. La paciente presentó en varias semanas una involución espontánea; en ocasiones, las lesiones evolucionaron a abscesos estériles, además de manifestaciones clínicas sistémicas. El tratamiento corticoideo tuvo buen resultado.

Plasmablastic lymphoma: case report.

INTRODUCCIÓN: El linfoma plasmablástico es un linfoma no Hodgkin de alto grado. Se describe en pacientes con infección por el virus de la inmunodeficiencia humana (VIH), en receptores de trasplantes y en pacientes de edad avanzada. Se presenta en la cuarta década de la vida, siendo la cavidad oral el sitio más común de afección. Sus células neoplásicas expresan marcadores de células plasmáticas como CD138 y CD38. En su patogenia se ha implicado al virus de Epstein-Barr. La consideración más desafiante en el diagnóstico diferencial es el mieloma múltiple, ya que las características morfológicas e inmunofenotípicas de estas dos enfermedades son muy similares. El régimen EPOCH (etopósido, epirubicina, vincristina, ciclofosfamida y prednisona) asociado a tratamiento antirretroviral es la opción más eficaz, mejorando la supervivencia a 17 meses. CASO CLÍNICO: Varón de 35 años con VIH y síntomas de disfunción orgánica asociada a mieloma. Se realizó electroforesis de proteínas y aspirado de médula ósea, descartándose mieloma múltiple. Continuando con el abordaje diagnóstico y con la sospecha de neoplasia ósea, se realizó una biopsia ósea que reportó linfoma plasmablástico CD138+ y Ki-67 80%, Epstein-Barr positivo. El paciente recibió quimioterapia con EPOCH y tuvo una sobrevida de 6 meses. CONCLUSIONES: La presencia de disfunción orgánica relacionada con mieloma motivó la búsqueda de dicha patología y se determinó la presencia de linfoma plasmablástico. La correlación clínica, bioquímica, inmunohistoquímica y radiográfica es esencial para el correcto diagnóstico. BACKGROUND: Plasmablastic lymphoma is a high grade non-Hodgkin lymphoma. It is described in patients with HIV infection, post-transplant and advanced age. It appears in the fourth decade of life and the oral cavity is the most common site of affection. Its neoplastic cells express markers of plasma cells such as CD138, CD38. In its pathogenesis the Epstein-Barr virus has been implicated. The most challenging consideration in the differential diagnosis is with multiple myeloma, since the morphological and immunophenotypic characteristics of these two entities are very similar. Treatment with EPOCH (etoposide, epirubicin, vincristine, cyclophosphamide and prednisone) associated with antiretroviral treatment is the most effective option, improving survival to 17 months. CASE REPORT: Male of 35 years with HIV and symptoms of organic dysfunction associated with myeloma. Protein electrophoresis and bone marrow aspiration were performed, ruling out multiple myeloma. Continuing with the diagnostic approach and with suspicion of bone neoplasia, a bone biopsy was performed with report of plasmablastic lymphoma CD 138+ and Ki-67 80%, Epstein-Barr positive. He received chemotherapy with EPOCH, with a six-month survival. CONCLUSIONS: The presence of organic dysfunction related to myeloma motivated the search for said pathology and the presence of plasmablastic lymphoma was determined; clinical, biochemical, immunohistochemical, and radiographic correlation are essential for correct diagnosis.

Still's disease: a rare condition, in a patient of unusual age.

INTRODUCCIÓN: Cuando inicia en el adulto, la enfermedad de Still es de mayor prevalencia en caucásicos y entre los 16 y 35 años. De etiología desconocida, se asocia a HLA-II, DR2, 4 y 7, y Bw35. El comienzo de los síntomas es agudo, con fiebre en agujas, asociada a exantema en las extremidades y el tronco, maculopapular, eritematoso y evanescente, pruriginoso, con fenómeno de Koebner. Presenta artralgias y artritis con un patrón poliarticular, simétrico y migratorio, mialgias y adenopatías. El 90% de los pacientes presentan anemia, leucocitosis con neutrofilia y trombocitosis asociada a la actividad, así como elevación de transaminasas y ferritina > 2000. El factor reumatoide y los anticuerpos antinucleares son negativos. El objetivo del presente trabajo es presentar un caso de enfermedad de Still que se sale del patrón habitual de manifestación. CASO CLÍNICO: Mujer de 56 años, inicia con exantema -maculopapular, eritematoso, pruriginoso, en zona periorbitaria -bilateral, tórax anterior, región glútea bilateral y zonas de extensión de codos y rodillas, que respetan el abdomen. Fiebre vespertina de 39 °C, con artralgias de codos, muñecas y rodillas, y mialgias, con faringe hiperémica. Después de descartar procesos infecciosos, neoplásicos y autoinmunitarios, y de acuerdo con los criterios de Yamaguchi y Fautrel, se diagnosticó enfermedad de Still. CONCLUSIONES: Este caso se presenta por la baja prevalencia de la enfermedad de Still y porque en el grupo etario de nuestra paciente no es habitual su presentación. Los -antecedentes familiares y el cuadro clínico sugestivo obligaron a descartar la presencia de otros procesos mórbidos, toda vez que el diagnóstico de enfermedad de Still es de exclusión. BACKGROUND: Adult-onset on Still's disease is common in Caucasians, between 16 and 35 years. Its cause is unknown, but it is associated with HLA-II, DR2, 4, 7 and Bw35. First symptoms are acute; fever in needles associated with exanthema in extremities and maculopapular trunk, erythematous and evanescent, pruritic with Koebner phenomenon. Patients present arthralgias and arthritis with a polyarticular, symmetrical and migratory pattern; myalgias and adenopathies. 90% of patients have anemia, leukocytosis with neutrophilia and thrombocytosis associated with the activity. Elevation of transaminases and ferritin greater than 2000. The rheumatoid factor and antinuclear antibodies are negative. The aim of this article is to present a case report of Still's Disease whose pattern of appearance is uncommon. CASE REPORT: A 56-year-old woman presented papular macular, erythematous, pruritic exanthema, in the bilateral peri-orbital area, anterior thorax, bilateral gluteal region, and elbow and knee extensions, while respecting abdomen. In addition, evening fever of 39 °C with arthralgias in elbows, wrists, and knees, myalgias, and hyperemic pharynx were manifested. According to the criteria of Yamaguchi and Fautrel, and after ruling out infectious, neoplastic, autoimmune processes, Still's disease was concluded. CONCLUSIONS: This case is presented due to the low prevalence of Still's disease and its presentation is not usual in the age group of our patient. The family history and very ­indicative clinical pictures forced us to rule out the presence of other morbid processes, while reinforcing the diagnosis of Still's disease, since it is by exclusion.