BACKGROUNDSystemic sclerosis is a connective tissue disease, characterized by fibrosis of several organs and tissues, with signs and symptoms varying depending on the affected structure. In the course of the disease, clinical decompensations can occur when there is exposure to some factors, such as: infections, surgical procedures or exposure to medications. CASE REPORTMale, 26 years old, diagnosed with systemic sclerosis since 2011, with fibrous interstitial pneumopathy as one of its manifestations. In order to prevent progression of lung disease, he has already received several therapeutic modalities, without satisfactory response. September 2019 echocardiogram showed 100 mmHg systolic pulmonary artery pressure, showing a severe picture of pulmonary hypertension. In April 2020, home oxygen therapy was indicated; however, at the end of that month, the patient evolved with worsening of lower limb edema, increased abdominal volume and worsening of baseline dyspnea. On physical examination, in addition to the edema already reported, he had tachydyspnea, rales on the lung bases bilaterally, moderate ascites, digital ulcers and fixed Raynaud's phenomenon. In view of the clinic compatible with decompensated cor pulmonale, hospital admission was indicated. The patient evolved with significant clinical worsening, being referred to the intensive care unit (ICU). The ICU admission exams showed positive RT-PCR for coronavirus disease 2019 (COVID-19), although there were no associated flu-like symptoms. With progressive worsening of the condition, he died after approximately 1 week of hospital admission.

No abstract

No abstract

BACKGROUNDAcquired hemophilia (AH) is a rare bleeding disorder, caused by antibodies against a clotting factor, usually antifactor VIII. In some cases, antifactor VIII can occur in patients with no relevant comorbidities, but in 40-50% it can be associated with postpartum period, cancer or autoimmune diseases, such as Sjögren's syndrome (SS). The treatment is based on hemostatic therapy during bleeding and immunosuppressive therapies to eradicate the inhibitor, usually with corticosteroids in combination with cytotoxic agents, such as cyclophosphamide. We bring here a case report of type A AH as a manifestation of SS, with intolerance to treatment with oral cyclophosphamide but response to rituximab. CASE REPORTFemale, 57 years old, in follow-up with rheumatology due to SS, using intravenous cyclophosphamide due to vasculitis, with multiple mononeuritis and skin lesions (purpura and livedo). In 2020, after hemorrhagic manifestations, such as bleeding from the gastrointestinal tract and muscle hematoma after peripheral venipuncture, laboratory tests revealed changes in the coagulogram, with increased activated partial thromboplastin time. Due to this change, the dosage of coagulation factor VIII was continued, which shown to be reduced, with the presence of factor VIII inhibitor, and type A AH was diagnosed. Due to severe bleeding, an infusion of activated prothrombin complex was indicated and, due to hemophilia, immunosuppression with oral cyclophosphamide was initiated. However, about 15 days after starting cyclophosphamide, the patient evolved with bicytopenia (hemoglobin of 5.4 and lymphopenia of 670), a condition compatible with myelotoxicity by the drug and, therefore, opted to discontinue the medication, with subsequent recovery of hematimetric levels. She evolved with fever, lymphadenopathy and relapse of hemophilia. As a patient developed hemophilia during the use of intravenous cyclophosphamide and had important side effects with the use of oral cyclophosphamide, we opted for treatment with rituximab, in the regimen of two doses of 1 g each, spaced for 15 days. After the first course of rituximab, the patient presented significant clinical and laboratory improvement, with no new skin lesions and AH control. The patient remains on rituximab, with no relapses of the disease since starting treatment, about a year ago. CONCLUSIONAfter failure of other immunosuppressive agents, rituximab is a drug that can be considered in the treatment of severe and refractory SS. In this case, we have a satisfactory effect of its use for this serious and rare manifestation of SS.

No abstract