Maternal and fetal outcomes of pregnancy in women with primary systemic vasculitis: A single-center cohort study of 20 patients and 30 pregnancies.

OBJECTIVES: To analyze pregnancy outcomes of patients with primary systemic vasculitis followed in a third-level referral center. METHODS: Retrospective cohort study of all pregnant women with systemic vasculitis followed between 2009 and 2022 at the High-Risk Pregnancy Clinic of the Department of Systemic Autoimmune Diseases of the Hospital Clínic, Barcelona. RESULTS: Twenty women with primary vasculitis were identified, with a total of 30 pregnancies. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (n = 7) and Behçet disease (n = 4) were the most frequent types of vasculitis. All women had the diagnosis of vasculitis before pregnancy, with a median time between disease diagnosis and pregnancy of 5.8 years (range: 2 months-29 years). Most were in remission at conception (76.7 %). During pregnancy, a vasculitis flare occurred in 4 (13.3 %) patients (one each with Takayasu arteritis, eosinophilic granulomatosis with polyangiitis [EGPA], IgA vasculitis [IgAV], and Behçet disease [BD]). Four (16.7 %) of the successful pregnancies had post-partum relapses (one each with EGPA, granulomatosis with polyangiitis, IgAV, and BD). Eighty percent of pregnancies resulted in live babies. In four cases (13.3 %), medical termination of pregnancy was decided, considering the mother or baby health risk. There were two spontaneous miscarriages, and no stillbirths or neonatal deaths. Preeclampsia was the most frequent maternal complication (25 %). Newborns were preterm in 24 % and low birthweight in 20 % of cases. No maternal deaths occurred. CONCLUSIONS: This cohort study shows that vasculitis relapses during pregnancy and post-partum, together with other pregnancy complications, occur in a considerable number of patients with systemic vasculitides, although a final good pregnancy outcome can be expected in most cases. These findings emphasize the convenience of managing these special situations in expert reference centers.

Susac Syndrome: Description of a Single-Centre Case Series.

This study describes the clinical characteristics, diagnostic results, treatment regimens, and clinical course of a cohort of patients with Susac syndrome (SS). It is a retrospective observational study of all patients with the diagnosis of SS evaluated at the Hospital Clinic (Barcelona, Spain) between March 2006 and November 2020. Nine patients were diagnosed with SS. The median time from the onset of the symptoms to diagnosis was five months (IQR 9.0), and the median follow-up time was 44 months (IQR 63.5). There was no clear predominance of sex, and mean age of symptoms onset was 36 years (range 19-59). Six patients (67%) presented with incomplete classical clinical triad, but this eventually developed in six patients during the disease course. Encephalopathy, focal neurological signs, visual disturbances, and hearing loss were the most frequent manifestations. Brain magnetic resonance imaging showed callosal lesions in all patients. Most were in remission within two years. Only four patients met the proposed criteria for definite SS. When SS is suspected, a detailed diagnostic workup should be performed and repeated over time to identify the clinical manifestations that will lead to a definite diagnosis.

Catastrophic antiphospholipid syndrome presented as ruptured papillary muscle during puerperium in a patient with systemic lupus erythematosus.

INTRODUCTION: Catastrophic antiphospholipid syndrome (CAPS) is a rare and serious phenomenon that requires prompt recognition and treatment. CASE PRESENTATION: The authors present the case of a puerperal woman with systemic lupus erythematosus (SLE) admitted to the emergency room with headache, blurred vision, thoracic pain, and purpuric lesions on both ears. Echocardiogram revealed global decrease in left ventricular function while cardiac and inflammatory markers were elevated. Three days after admission she developed cardiogenic shock due to rupture of mitral papillary muscle which required emergent cardiac surgery, with replacement of the mitral valve; treatment with anticoagulation, high-dose glucocorticoids, and intravenous immunoglobulins were initiated. Cardiac and brain MRI revealed signs of ischemic lesions in both organs. Histopathology analysis of the placenta and papillary muscle showed signs of ischemia secondary to microvascular thrombosis. Based on the clinical demonstration of thrombosis in three organs, and the presence of lupus anticoagulant antibodies, a diagnosis of probable CAPS was established. CONCLUSION: This case highlights the importance of a high level of suspicion of CAPS, particularly in patients with risk factors, and the value of immediate adequate treatment. Moreover, the rupture of a papillary muscle with histologically consistent signs of antiphospholipid syndrome expands the spectrum of involvement of this disease and should be considered as a rare but life-threatening possibility in patients with myocardial injury.

Pleural Effusion Secondary to Obstructive Uropathy: A Case of Urinothorax.

Urinothorax is a rare type of pleural effusion and usually the result of genitourinary tract disease. An accurate and early diagnosis is crucial as resolution of the underlying pathology is the mainstay of treatment. We report the case of a 69-year-old man who was admitted to the Internal Medicine ward due to obstructive acute kidney injury of unknown origin. The patient was submitted to urinary catheterization and to right percutaneous nephrostomy. Two weeks after admission he developed a large left pleural effusion; a left urinoma was also visible on computed tomography. After thoracentesis, pleural fluid analysis demonstrated a paucicellular transudate with pH <7.40 and pleural fluid/serum creatinine ratio >1.0. The diagnosis of urinothorax was made and further study allowed the diagnosis of prostate cancer as the aetiology of the obstruction. When bilateral percutaneous nephrostomy was performed, resolution of the urinothorax and normalization of renal function occurred. LEARNING POINTS: Urinothorax is a rare and possibly under-recognized cause of transudative pleural effusion; over the last five decades, fewer than 60 cases have been reported in the English literature worldwide.Diagnosis of urinothorax depends upon a high index of clinical suspicion and on pleural fluid analysis, but imaging can also support the diagnosis, for example by showing the presence of a urinoma.Clinicians should recognize urinothorax early in its clinical course as favourable outcomes are often achieved after correction of the underlying genitourinary tract pathology.

Urologic and male genital manifestations of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatous vasculitis, which predominantly affects small-sized blood vessels. Major organ involvement includes the upper/lower respiratory tract and kidneys. In contrast, genitourinary disease is rare in GPA patients, reported in <1% of cases in large cohorts. Manifestations at this level include prostatitis, destructive urethritis, genital ulcers, orchitis and renal masses. Also, high-dose cyclophosphamide, one of the main immunosuppressive drugs used for GPA treatment, is associated with bladder toxicity, i.e., hemorrhagic cystitis and cancer. Here, we review the main urogenital symptoms associated with this ANCA-associated vasculitis. In addition, cyclophosphamide-induced urologic complications are detailed.

Development and validation of a risk calculator to differentiate flares from infections in systemic lupus erythematosus patients with fever.

OBJECTIVE: To develop and validate a predictive risk calculator algorithm that assesses the probability of flare versus infection in febrile patients with systemic lupus erythematosus (SLE). METHODS: We evaluated SLE patients admitted because of fever in the Department of Autoimmune Diseases of our Hospital between January 2000 and February 2013. Included patients were those with final diagnosis of infection, SLE flare or both. Data on clinical manifestations, treatment and laboratory results were collected. Variables considered clinically relevant were used to build up all possible logistic regression models to differentiate flares from infections. Best predictive variables for SLE relapses based on their higher area under the receiver operating characteristic (ROC) curve (AUC) were selected to be included in the calculator. The algorithm was developed in a random sample of 60% the cohort and validated in the remaining 40%. RESULTS: One hundred and thirty SLE patients presented 210 episodes of fever. Fever was attributed to SLE activity and to infection in 45% and 48% of the cases, respectively. Three independent variables for prediction of flares were consistently selected by multivariate analysis: days of fever, anti-dsDNA antibody titres and C-reactive protein levels. Combination of these variables resulted in an algorithm with calculated AUC of 0.92 (95% CI: 0.87 to 0.97). The AUC for the validation cohort was of 0.79 (95% CI: 0.68 to 0.91). CONCLUSION: The proposed flare risk predictive calculator could be a useful diagnostic tool for differentiation between flares and infections in febrile SLE patients.