ABSTRACT
Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by multiple thromboses affecting mainly small vessels in a short period of time in patients with antiphospholipid antibodies. A high suspicion index is mandatory in order to initiate rapidly aggressive immunomodulatory therapy to avoid a very poor prognosis. Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome, with a worse outcome when the catastrophic features occur. We report the case of a 64-year-old woman with a clinical debut of SLE who presented concomitantly with CAPS with several thrombosis affecting the kidney, spleen and bilateral limbs with blue toe syndrome in both legs. Furthermore, she presented with aortitis, with a malaise and myalgias and general syndrome (asthenia, hyporexia and mild weight loss). Fortunately, she had a good response to multi-target combination therapy (anticoagulants, corticosteroids, hydroxychloroquine, intravenous immunoglobulins, plasma exchange and rituximab). Here, we discuss the association between aortitis and CAPS secondary to SLE, and review the literature regarding similar conditions.
Subject(s)
Antiphospholipid Syndrome/complications , Aortitis/complications , Lupus Erythematosus, Systemic/complications , Thrombosis/etiology , Adrenal Cortex Hormones/therapeutic use , Antibodies, Antiphospholipid/blood , Anticoagulants/therapeutic use , Antiphospholipid Syndrome/therapy , Aortitis/therapy , Catastrophic Illness , Combined Modality Therapy , Female , Humans , Hydroxychloroquine/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Middle Aged , Plasma Exchange , Rituximab/therapeutic useABSTRACT
Castleman disease (CD) represents a heterogeneous group of lymphoproliferative disorders that share well-defined histopathological features. An observational study of patients with CD was conducted. A total of 53 patients had CD: 20 had the unicentric form (UCD) and 33 the multicentric (MCD) variant; 10 of the latter cases were infected with human herpesvirus-8 (HHV-8) and 23 were idiopathic (iMCD). Median age differed between UCD and iMCD (30 vs. 49 years, p = .004). Males were completely predominant in HHV-8-associated MCD (100%), and females were more frequent in UCD (75 vs. 48%, p = .06). Relapses were more frequent in iMCD (57 vs. 10% UCD, p = .002), and mortality was significantly higher in iMCD and the HHV-8-associated form with respect to UCD. We conclude that UCD is a benign disorder of younger ages and female predominance, while iMCD represents a different entity with more disease relapses and higher mortality.
