Budd-Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco.

Budd-Chiari syndrome (BCS) is considered a rare but serious complication of Behçet's disease (BD). This study was performed to define the prevalence, clinical and biological features, treatment, and clinical course of BSC associated with BD in a Moroccan population. We retrospectively analyzed the medical records of 1578 patients fulfilling the international diagnostic criteria for BD, including those with BSC. Eighteen male and 3 female patients, with a mean age of 36 ± 8.6 years. The inferior vena cava was involved in 81% (n = 17) of cases. All forms of BCS were noted: the chronic form in 52.4% (n = 11), the subacute form in 38% (n = 8), and the fulminant form (2 cases). Ascites was the main clinical sign and was present in 62% of patients (n = 13). Other venous thromboses (superior vena cava and lower limbs) were associated with BSC in 52.4% of patients (n = 11). Arterial involvement was noted in 28.6% (n = 6). Cardiac manifestations were present in 19% (n = 4) of the patients. All the patients received anticoagulants associated with corticosteroids. Immunosuppressants were used in 95% (n = 20). One patient received infliximab. Severe complications were noted in 38% (n = 8) of patients (digestive bleeding, confusion, infections and liver failure). Four patients have died during the study period. BCS in patients with BD is not uncommon and can be life threatening. It is frequently associated with other vascular manifestations that can be difficult to treat, particularly in the presence of pulmonary artery aneurysms. Prognosis improved with the use of immunosuppressants. Biologics can be promising in the early stages.

Mycophenolate Mofetil Use in Severe Myocarditis Complicating Systemic Lupus.

Cardiac involvement represents an increasingly frequent complication in systemic lupus, with pericarditis being the most classic cardiac manifestation. However, the most severe and fatal form seems to be myocarditis. We present the case of a patient with systemic lupus complicated by cardiogenic shock secondary to troponin-negative acute myopericarditis and successfully treated with mycophenolate mofetil and corticosteroid therapy. A 33-year-old woman with no past medical history presented with asthenia and inflammatory arthralgia. She was admitted in June 2021 for acute heart failure. Transthoracic cardiac ultrasound showed dilated cardiomyopathy with global hypokinesis (20-25% of ejection fraction) and right ventricular dysfunction without significant mitral and aortic valve disease. She had raised proBNP (pro-brain natriuretic peptide), low troponin, normochromic normocytic anemia at 10.4 g/dL, positive direct Coombs, lymphopenia at 460/mm3, serum creatinine at 23.9 mg/L, and proteinuria/creatininuria 2.48 g/g. Cardiac magnetic resonance imaging (CMR) suggested the diagnosis of myopericarditis. The etiological assessment did not identify an infectious, toxic, or medicinal cause. The clinical picture suggested the possibility of an autoimmune disease. The patient presented with lesions suggestive of cutaneous vasculitis, with oral ulcers with polyarthritis. The autoimmune workup showed anti-nuclear antibodies at 1:1,280, anti-native DNA antibodies at 210 IU/mL (normal < 10 IU/mL), and positive anti-SM Abs. The diagnosis of lupus myopericarditis complicated by cardiogenic shock was made, which was associated with acute renal impairment. The patient was initiated on heart failure medications along with corticosteroids and mycophenolate mofetil. On day 15, the left ventricular ejection fraction improved to 45-50%, with clinical improvement in signs of heart failure and general condition. The existence of myopericarditis without obvious etiology, especially when there are extra-cardiac signs such as skin and joint involvement, should lead us to look for systemic lupus in order to start etiological treatment in addition to cardiac medical treatment. Until now, there is no standard treatment for lupus myocarditis, but the use of mycophenolate mofetil seems to be a promising treatment.

Eosinophilic Fasciitis and Smoldering Multiple Myeloma: An Exceptional Association in Young Adults.

Eosinophilic fasciitis (EF) or Shulman's fasciitis is a rare condition characterized by subcutaneous edematous induration sparing the face and distal extremities and progressing to skin sclerosis. Its association with other pathologies, notably hemopathies, is described in the literature, but its association with smoldering multiple myeloma remains very rare, especially in a younger subject.