The presence of non-criteria manifestations negatively affects the prognosis of seronegative antiphospholipid syndrome patients: a multicenter study.

BACKGROUND: Seronegative antiphospholipid syndrome (SN-APS) is often defined as the presence of APS criteria manifestations, negative antiphospholipid antibodies (aPL), and coexistence of APS non-criteria manifestations. Nevertheless, the impact of these non-criteria features is still unclear. On a different note, the relevance of one single aPL positive determination in patients with APS manifestations is another domain with limited evidence. We aim to compare the course of SN-APS and single-positive aPL (SP-aPL) patients with that of individuals with APS manifestations without non-criteria features/aPL positivity (controls). METHODS: Retrospective analysis of patients with thrombosis/obstetric morbidity assessed in two European hospitals between 2005 and 2020. Patients were divided into SN-APS, SP-aPL, and control groups. Clinical characteristics, comorbidities, and therapies were compared. RESULTS: A total of 82 patients were included in the SN-APS group, 88 in the SP-aPL group, and 185 in the control group. In Cox regression model, SN-APS displayed more thrombosis recurrence than controls (HR 3.8, 95% CI 2.2-6.5, p < 0.001) even when adjusting for the presence of hereditary thrombophilia, systemic lupus erythematosus, or contraceptive hormonal treatment. In SP-aPL, the difference in thrombosis recurrence did not reach statistical significance (p = 0.078). Indefinite anticoagulation (p < 0.001 and p = 0.008, respectively) and vitamin K antagonist (VKA) use (p < 0.001 in both cases) were more common in SN-APS/SP-aPL. CONCLUSION: SN-APS displayed more thrombosis recurrence, indefinite anticoagulation, and VKA use than controls without non-criteria manifestations. The presence of such features in patients with thrombosis and negative aPL may negatively impact their clinical course.

Causes of Hospitalization in Systemic Lupus Erythematosus: A Narrative Review.

Hospitalizations are frequent in Systemic Lupus Erythematosus (SLE) and carry a significant economic burden. The focus of this review was to summarize the information available on the main causes of SLE hospitalizations over recent decades. A literature review was conducted, using PubMed and Scopus, for articles related to SLE hospital admissions from 1981 onward. Active disease/ flare and infection were the leading causes of admission across the study period. More recently, other comorbidities gained relevance, such as cardio and cerebrovascular disease, pregnancy-related morbidity, adverse drug reactions, thromboembolic events, malignancy and renal, pulmonary and gastrointestinal disease. African and Southeast Asian studies seemed to display particularly high percentages of patients admitted with active disease/flare, while European and North American studies appeared to report more admissions due to comorbidities and accumulated disease/treatment damage. Some data support a temporal change of certain admission causes, but the limited number, heterogeneity and variance among studies weakens a consistent analysis. In conclusion, despite the developments in SLE management, causes of hospitalization have not prominently changed across recent decades.

Cervical Lymphadenopathy in a Nonagenarian Woman: What to Think?

Tuberculosis remains a worldwide public health problem. Cervical tuberculous lymphadenitis (TBL) or scrofula is the most common form of extrapulmonary tuberculosis, affecting the cervical lymph nodes. We report the case of a 93-year-old woman presenting with cervical adenopathies with 3 months duration. Fine needle aspiration (FNA) biopsy yielded a noncaseous granulomatous process, but was negative for Mycobacterium tuberculosis (MT). As the adenopathies had grown, an excisional biopsy was performed. An extensive study of infectious aetiologies was performed, including for MT, with a negative outcome. Owing to the persistence of cervical lymphadenitis with caseous granulomas, a diagnosis of TBL was strongly suspected and presumptive treatment was initiated. Afterwards, diagnostic confirmation was obtained by isolation of MT in the lymph node culture. The patient presented a favourable clinical outcome. This case highlights that a high index of suspicion is essential for the diagnosis of TBL, especially in the elderly, and emphasizes the importance of pursuing diagnostic confirmation, in which FNA and excisional biopsy plays a key role. LEARNING POINTS: The diagnosis of tuberculous lymphadenitis should be considered in all patients presenting with cervical adenopathies, especially in endemic areas, irrespective of the patient's age.A high index of suspicion is essential for the diagnosis due to the wide range of conditions that can mimic the disease.Fine needle aspiration biopsy is appropriate as an initial diagnostic approach, with excisional biopsy recommended when the former is not diagnostic.

Azathioprine-induced interstitial nephritis in an anti-neutrophil cytoplasmic antibody (ANCA) myeloperoxidase (MPO) vasculitis patient.

Azathioprine (AZA) is used in a wide array of autoimmune diseases, still corresponding to the mainstay maintenance therapy in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although generally well-tolerated, several side effects are recognized. We report the case of a 50-year-old Caucasian man with kidney-limited ANCA myeloperoxidase (MPO) vasculitis who presented with general malaise, fever, worsening renal function, and elevated inflammatory markers 2 weeks after the initiation of therapy with oral AZA. Although a disease relapse was suspected, renal biopsy revealed an eosinophilic infiltrate, suggestive of acute interstitial nephritis. After suspension of AZA, a sustained improvement of renal function and normalization of inflammatory markers was observed. A diagnosis of allergic interstitial nephritis secondary to AZA was established, corresponding to the first biopsy-proven case described in an ANCA MPO vasculitis patient. Although rare, renal toxicity of AZA must be present in the clinician's mind, avoiding the straightforward assumption of disease relapse in the case of worsening renal function.