Clin Exp Rheumatol
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A number of patients with Behçet's disease (BD) associated with myelodysplastic syndrome (MDS) with or without trisomy 8 have been reported. A high frequency of gastrointestinal (GI) involvement was reported in such patients. The aim of this systematic literature review was to delineate whether GI involvement is an inherent feature of BD associated with MDS, whether these patients do actually have BD rather than GI symptoms related to MDS, and whether the presence of trisomy 8 plays a role in the disease expression of BD associated with MDS. ⋯ GI involvement seems to be an inherent feature of BD associated with MDS regardless of geographic differences. Despite the increased frequency of GI involvement in these patients, MDS does not seem to modify the clinical expression of gastrointestinal involvement. Presence of trisomy 8 seems to modify the disease expression with an increased frequency of fever.
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A number of patients with Behçet's disease (BD) associated with myelodysplastic syndrome (MDS) with or without trisomy 8 have been reported. A high frequency of gastrointestinal (GI) involvement was reported in such patients. The aim of this systematic literature review was to delineate whether GI involvement is an inherent feature of BD associated with MDS, whether these patients do actually have BD rather than GI symptoms related to MDS, and whether the presence of trisomy 8 plays a role in the disease expression of BD associated with MDS. ⋯ GI involvement seems to be an inherent feature of BD associated with MDS regardless of geographic differences. Despite the increased frequency of GI involvement in these patients, MDS does not seem to modify the clinical expression of gastrointestinal involvement. Presence of trisomy 8 seems to modify the disease expression with an increased frequency of fever.
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Review Case Reports
Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature.
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) that is characterised by new-onset malignant hypertension and progressive acute renal failure, often with associated microangiopathic haemolytic anaemia and thrombocytopenia. SRC was at one time almost uniformly fatal, with death often occurring within a few weeks. ⋯ In this report, we present a case in which a patient developed SRC prior to being diagnosed with scleroderma. Additionally, we review the pathogenesis, presenting signs and symptoms, management and prognosis of SRC.
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Systemic lupus erythematosus is a multiorgan autoimmune disease with a highly variable clinical course, typically involving women in childbearing age. At present, many aspects of its pathogenesis still remain unclear. ⋯ Therefore, multiple aspects of SLE still remain challenging and it continues to be the object of both clinical and translational clinical research. Herewith, we provide a critical digest of the recent literature on this topic.
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Non-infectious aortitis often presents with non-specific symptoms leading to inappropriate diagnostic delay. We intend to describe the clinical spectrum and outcome of patients with aortitis diagnosed at a single centre. ⋯ Aortitis is not an uncommon condition. The diagnosis is often delayed. Atypical PMR features, unexplained low back or limb pain, constitutional symptoms along with increased acute phase reactants should be considered 'red flags' to suspect the presence of aortitis.