ABSTRACT
Thrombocytopenia is one of the common haematological manifestations, occurring in 7% to 30% of systemic lupus erythematosus (SLE) patients. Immune thrombocytopenia (ITP) may occur in variable pathways as a result of cross-reacting antibodies or immune complexes that bind to platelet receptors, or infection of progenitor megakaryocytes, and decreased production of thrombopoietin (TPO). It was shown that the vast majority of SLE patients with thrombocytopenia had increased levels of anti-glycoprotein IIb/IIIa (anti-GPIIb/IIIa) or anti-thrombopoietin receptor (anti-TPOR). Eltrombopag is a thrombopoietin receptor agonist that binds to the transmembrane portion of the surface receptor and induces maturation of megakaryocytes and production of platelets. Herein, we report two SLE patients with severe thrombocytopenia who are also refractory to both intravenous immunoglobulin (IVIG), rituximab, and splenectomy. Ultimately, they successfully treated with eltrombopag without any complication. Eltrombopag worked well and their platelet counts increased above 200,000/mm3 only two weeks later.
ABSTRACT
Background. A large number of comparative studies have been conducted for ankylosing spondylitis (AS) and non-radiographic axial spondyloarthritis (nr-axSpA), including disease burden, treatment modalities and patient characteristics. The aim of this study was to compare physician related diagnostic delay time between patients with AS and nr-axSpA. Methods. In our retrospective study we included 266 patients with axSpA. Patients were classified into two subgroups, AS and nr-axSpA. The time from back pain onset until diagnosis of axSpA was defined as the diagnostic delay. The first specialist referred to and the first diagnosis for each patient was noted in detail. Patient characteristics, clinical manifestations and laboratory and imaging results at diagnosis were also compared between subgroups. Results. The diagnostic delay time was significantly longer for AS patients [6 ± 8.14 years vs 1.62 ± 2.54 years]. 40.9% of all patients were initially consulted by specialists in physical therapy and rehabilitation, followed by 29.7% consulted by a neurosurgeon and 19.9% by a rheumatologist. The most common initial diagnosis was fibromyalgia, 52.6% (140), followed by ankylosing spondylitis, 28.9% (77), and lumbar disc hernia, 12.7% (34). Conclusion. The vast majority of patients were initially evaluated by healthcare providers other than rheumatologists and mostly diagnosed with fibromyalgia. Efforts to increase awareness and to educate first healthcare providers may shorten the diagnostic delay time.
