Clin Exp Rheumatol
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To investigate the clinical characteristics of SSc patients with DUs in China. ⋯ The occurrence of DUs in Chinese SSc patients is frequent. It is possible that SSc patients with DUs were influenced by the disease earlier in life, which should be detected early for effective intervention.
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Case Reports
Stiff skin syndrome and myeloma successfully treated with autologous haematopoietic stem cell transplantation (HSCT).
Stiff skin syndrome (SSS) is a rare scleroderma-like syndrome characterised by stone hard skin, joint limitation and progressive restriction of chest that may lead to death. We describe the efficacy of haematopoietic autologous stem cell transplantation (HSCT) in a case of SSS secondary to a smouldering myeloma (SM), with severe joint disability, lung interstitial disease and oesophageal dysfunction. The patient was evaluated at 1, 12 and 18 months after HSCT, clinically (joint motility, HAQ and NYHA for dyspnoea) and instrumentally (DLCO, chest HRCT, oesophagus x-ray). After 18 months since HSCT, we observed a high improvement, contemporaneously to SM remission, of HAQ, joint motility, lung (at DLCO and HRCT) and oesophageal abnormalities.
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The risk of activation of latent tuberculosis infection (LTBI) is increased in patients treated with anti-TNF-α drugs. Tuberculin skin test (TST) and Quantiferon-TB Gold test (QFT) are used to detect LTBI before and during anti-TNF-α treatment. We describe here a relation of these tests at various timepoints and also longitudinal QFT data. ⋯ Poor correlation between the QFT and TST renders the TST non-specific for LTBI. QFT is more specific to detect LTBI and conversion to a positive result may predict active TB. An increase in IFN-γ production in response to mycobacterial antigens is seen when the TST is performed before the QFT. Mitogen-induced IFN-γ production increases after initiation of anti-TNF-α therapy.
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The aim of this study was to evaluate the aetiology of 'unexplained' cytopenias in patients with autoimmune disorders, as well as to identify parameters that should alert clinicians to the need for bone marrow examination. ⋯ Clonal haematologic disorders and toxicity are frequent findings in patients with autoimmunity referred for haematologic consultation, owing to otherwise unexplained cytopenias. Patients with high serum iron, high MCV and presence of serum monoclonal band should undergo bone marrow examination to exclude haematologic malignancy or bone marrow toxicity.
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Medically 'unexplained' chronic disorders remain a challenge for clinicians because the patients with these syndromes have a wide range of symptoms, including pain, impaired concentration, sleep disturbances, fatigue and mood disorders, as well as functional problems and difficulties in carrying out the activities of daily living. Such disorders are the result of a complex physiological interaction of central and peripheral nervous signaling that leads to a highly individual symptom complex, although some of them seem to be related to one another, especially in terms of the mechanism of chronicity and pain amplification, and the co-occurrence of fatigue, sleep alterations, mood disturbances and cognitive impairment. This review will discuss the recent literature concerning the most common dysfunctional disorders: fibromyalgia syndrome, myalgic encephalomyelitis/chronic fatigue syndrome, and irritable bowel syndrome.