No abstract

BACKGROUNDSystemic lupus erythematosus (SLE) is an inflammatory, chronic and multisystemic disease that can cause cardiac involvement in the form of pericarditis, myocarditis and endocarditis. Endocarditis in SLE is characterized by valvular involvement, sometimes with sterile (Libman-Sacks) or infected vegetations. Infections in patients with SLE are responsible for significant mortality, and constitute a clinical challenge regarding the differentiation between disease activity and an infectious process. CASE REPORTA 34-year-old female patient with SLE, in outpatient follow-up, using prednisone and hydroxychloroquine. Referred to hospital due to fever; headache in the left frontal orbital region, vomiting, diplopia and asthenia. On examination, she had bradyphrenia, strabismus, nystagmus, reduced left side strength and sensitivity. There was tachypnea and systolic murmur in the mitral area. Initially, the hypothesis of bacterial meningitis was considered and ceftriaxone was started. Laboratory tests showing anemia, lymphopenia, anti-DNA 1/160, without consumption of complement, requested evaluation by the rheumatological team to assess the possibility of SLE activity. Relevant complementary tests: Transesophageal echocardiogram with an image of vegetation measuring 1.1 × 0.7 cm adhered to the anterior leaflet of the mitral valve, with significant mitral and tricuspid regurgitation, and mild left atrium enlargement. Brain magnetic resonance imaging described signal alteration in the left medial aspect of the bulbopontine region without restriction to water diffusion, and flair hypersignal foci in the supratentorial white matter, suggestive alterations with septic embolization. Blood cultures with two positive samples with Staphylococcus aureus, sensitive to oxacillin, which started to be used instead of ceftriaxone. Follow-up transesophageal echocardiogram with vegetation reduction to 0.7 × 0.6 cm. Patient evolved with stabilization of neurological manifestations, interruption of fever and progressive improvement of infectious markers such as leukogram and CRP, with subsequent valve replacement approach after antimicrobial treatment and control of SLE activity. CONCLUSIONWe report a case of staphylococcal endocarditis in the mitral valve with septic embolization to the brain, which could mimic or be confused with lupus disease activity in the central nervous system, leading to a misguided approach with intensification of immunosuppressive therapy. Differentiating disease activity versus infection in lupus patients remains a diagnostic challenge.

No abstract

BACKGROUNDMacrophage activation syndrome (MAS) is a severe and potentially fatal hyperinflammatory condition characterized by excessive macrophage and T cell activation resulting in multiple organ dysfunction. MAS is considered a secondary or acquired form of hemophagocytic lymphohistiocytosis (HLH) and is usually associated with infection, malignancy, or in the context of rheumatologic disorders, such as systemic lupus erythematosus (SLE). In SLE, MAS can mimic underlying disease activity because both entities share some common features, such as fever, lymphadenopathy, splenomegaly, and blood cytopenias. This overlap in clinical presentations can make it difficult to recognize MAS and delay the definition of the most appropriate therapeutic approach. CASE REPORTA 16-year-old female patient, started in February 2022 with fatigue, edema in the lower limbs with ascending progression, evolving to anasarca, hypertension and oliguria, referred for hospitalization. On admission exams she had anemia, lymphopenia, fine speckled nuclear ANA pattern (1/160), consumption of complements and positive autoantibodies (anti-RO, anti-RNP and anti-SM), protein/creatinine ratio at nephrotic level. Pulse therapy with methylprednisolone and cyclophosphamide was started, and renal biopsy showed class III lupus nephritis. Patient evolved with persistent fever, without improvement after antibiotic therapy, splenomegaly, pancytopenia with worsening of the general condition, need for transfer to intensive care unit, intubation and initiation of renal replacement therapy. Laboratory review showed elevated ferritin, lactic dehydrogenase, fibrinogen, prolonged activated partial thromboplastin time and hypertriglyceridemia. A myelogram was performed, which confirmed the main diagnostic hypothesis, MAS, due to the presence of hemophagocytosis. Treatment with dexamethasone 10 mg/m² of body surface and intravenous human immunoglobulin 1 g/kg was started. One week after the start of treatment, the patient showed clinical and laboratory improvement, and monthly cyclophosphamide was maintained and dexamethasone was gradually reduced. CONCLUSIONMacrophage activation syndrome should be included in the differential diagnosis of SLE patients with persistent fever and pancytopenia. The mortality rate of MAS is high and therefore prompt initiation of treatment is of utmost importance.

BACKGROUNDThrombocytopenia in systemic lupus erythematosus (SLE) is associated with prognosis and association with other manifestations that denote greater disease severity. Its management may require several therapeutic strategies. CASE REPORTYoung female patient diagnosed with systemic lupus erythematosus at 13 years of age. Initial presentation with hypocomplementemia and thrombocytopenia plus mild splenomegaly. Refractory to oral corticotherapy, in use of azathioprine and hydroxychloroquine. Recent episode of severe hemorrhagic acute abdomen due to ruptured ovarian cyst leading to urgent laparotomy. Maintaining values persistently below 20,000 platelets with spontaneous mucosal bleeding and need for blood transfusion. Pulse therapy with methylprednisolone plus cyclophosphamide was opted as rescue therapy. Despite clinical stability, the clinical and laboratory findings did not change after close surveillance. Rituximab was chosen as a second line of therapy, but serial follow-up of platelet counts showed no improvement after 3 weeks on an optimized dose of immunobiological. Myelogram with preserved cellularity, increased megakaryocytic series, moderate dyserythropoiesis with asynchronism of maturation, thus revealing a process of PTI-like peripheral destruction. Referred for laparotomy splenectomy, with gradual and lasting sequential platelet count response in the weeks following surgery. The patient has returned to normal hematimetric levels, with good recovery, and remains in low disease activity. CONCLUSIONThrombocytopenia in SLE is associated with prognosis and association with other manifestations that denote greater disease severity. The first line of therapy remains corticosteroid therapy, which is sufficient for most patients in whom this manifestation is mild to moderate. For refractory cases, immunosuppressants are the choice. Addressing secondary causes and differential diagnoses is part of the assessment as well as the therapeutic strategy. Splenectomy remains one of the later options, given the complexity and morbidity of the procedure. But, when well indicated, it can be the rescue of refractoriness situations.