Quiz Ref ID Systemic sclerosis is a heterogeneous autoimmune connective tissue disease characterized by vasculopathy, autoantibody formation, low-grade inflammation, and fibrosis in skin and internal organs, with varying geographical prevalence (50-300 per million persons per year) and incidence (2.3-22.8 per million persons per year).^1,2 Previous studies have shown that systemic sclerosis is amenable to treatment with autologous hematopoietic stem cell transplantation (HSCT).^3-9 Improvement of skin involvement and functional ability was consistently observed, although some studies showed that HSCT can also ameliorate vasculopathy, improve skin and lung involvement, and correct immune abnormalities.^10-13 The benefits of HSCT must be weighed against the risk of serious toxicities due to organ involvement in systemic sclerosis.¹⁴ It is still unclear whether HSCT prolongs survival in systemic sclerosis. We therefore conducted a randomized clinical trial called ASTIS (Autologous Stem Cell Transplantation International Scleroderma) to compare safety and efficacy of HSCT vs 12 successive monthly intravenous pulses of cyclophosphamide.

We studied the clinical features and autoantibody profile in 21 patients with antisynthetase syndrome (AS) comparing to 48 patients with classical polymyositis and dermatomyositis without AS. At presentation, the AS group showed more frequently the presence of interstitial lung disease (ILD), arthritis/arthralgia, mechanic's hand and anti-Ro/SSA antibodies. Patients without AS had more frequent proximal weakness and cutaneous erythematosus rash. Interestingly, the AS patients with associated anti-Ro/SS-A antibodies seem to be predisposed to the development of a more severe ILD, expressed as HRCT total score ≥ 7. During a follow up of about 3 years (range 6–110 months), the presence of anti-Jo-1 antibody alone or in association with anti-Ro/SSA did not influence survival or a more severe prognosis of ILD.

Objective. IL-1β plays a key role in the pathogenesis of Schnitzler's syndrome (SS). We have investigated inflammasome activity in peripheral blood mononuclear cells (PBMCs) from a patient affected by a variant type of SS. Methods. PBMCs were purified by Ficoll and examined for ability to secrete IL-1β and -18, expression and function of the P2X₇ receptor and expression of apoptosis-associated speck-like protein containing a caspase recruitment domaine (ASC) and NOD-like receptor protein 3 (NLRP3) before and after the therapy with steroid. Furthermore, extracellular adenosine 5′-triphosphate (ATP) blood levels were determined by luciferase assay. Expression of inflammasome components was measured by real time PCR and western blotting. Results. PBMCs of patient with SS showed a high, spontaneous and lipopolysaccharide-stimulated, IL-1β release but low response to stimulation with the P2X₇ agonist benzoyl ATP. P2X₇ expression was several fold increased, whereas ASC expression was dramatically decreased compared with PBMCs from healthy controls. NLRP3 expression was unchanged. Prednisone treatment induced remission of clinical symptoms and normalized IL-1β secretion and P2X₇ and ASC expression. Conclusion. These findings reveal the presence of an overall derangement of the inflammasome and IL-1β processing and release in SS.

Background: Multicentric reticulohistiocytosis (MRH) is a rare systemic disease, presenting with typical skin abnormalities and erosive polyarthritis, which is often associated with malignancy. Case report: A case of MRH arthropathy, in which the typical nodular skin manifestation of the disease was absent, is described in a patient with a past history of breast cancer and no evidence of recurrent or new malignancy. Results: Careful clinical and roentgenological evaluation disclosed important clues to differentiate this condition from other more common distal interphalangeal arthritides—namely, osteoarthritis and its “erosive” variant, rheumatoid arthritis, psoriatic arthritis, tophaceous gout, dialysis related hand arthropathy, and from the rarer fibroblastic rheumatism, all of which can be mimicked by MRH. Histopathology showed the characteristic histiocytic and multinucleated giant cell infiltrate with ground glass cytoplasm, and immunohistochemical analysis showed markers evocative of a monocyte/macrophage origin of MRH.