Practice Matters Rituximab-associated hypogammaglobulinemia: Incidence, predictors and outcomes in patients with multi-system autoimmune disease. Roberts DM et al, J Autoimmun. 2014 Dec 31. pii: S0896-8411(14)00173-5. This study describes the incidence and severity of hypogammaglobulinemia in patients receiving rituximab for small vessel vasculitis and other multi-system autoimmune diseases. 288 patients who received rituximab were identified 243 were eligible for inclusion with median follow up of 42 months. 26% were IgG hypogammaglobulinemic at the time that rituximab was initiated and 56% had IgG hypogammaglobulinemia during follow-up ….. Pulmonary Fibrosis in Antineutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis: A Series of 49 Patients and Review of the Literature. Comarmond C et al, Medicine (Baltimore). 2014 Nov; 93(24):340-9. In this retrospective multicenter study, authors reported the main features and long-term outcomes of patients with PF associated with AAV, fulfilling the American College of Rheumatology criteria and/or Chapel Hill definitions. Forty-nine patients with PF associated with AAV were identified. Forty (81.6%) patients had MPA and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of Vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiologic pattern……. Comparison between classical polyarteritis nodosa and single organ vasculitis of medium-sized vessels: a retrospective study of 25 patients and review of the literature. Kint N et al, Acta Clin Belg. 2015 Jan Polyarteritis nodosa (PAN) is a rare form of systemic vasculitis, characterized by necrotizing inflammation of medium-sized vessels. In clinical practice, the distinction is made between a limited, mostly cutaneous, form and a generalized form. The Chapel Hill Consensus Conference of 2012 on the classification of the vasculitides classifies PAN as a medium vessel vasculitis, whereas the limited forms fall under the heading 'single-organ vasculitis' (SOV), with subdivions such as 'cutaneous arteritis' (formerly called cutaneous PAN) and 'others'. In this last category, forms of PAN limited to a single organ (e.g. testicle, gall bladder or appendix) should be categorized. This study compared demographics, clinical characteristics and prognosis between SOV and generalized PAN…… Blau syndrome: cross-sectional data from a multicentre study of clinical, radiological and functional outcomes. Rosé CD et al, Rheumatology (Oxford) 2014 Nov 20. Three-year, multicentre, observational study on articular, functional (HAQ, Childhood HAQ and VAS global and pain), ophthalmological, therapeutic and radiological data in BS patients. Baseline data on the first 31 recruited patients from 18 centers in 11 countries are presented. Of the 31 patients, 11 carried the p.R334W NOD2 mutation, 9 the p.R334Q and 11 various other NOD2 missense mutations…. IgG4-related disease. Kamisawa T et al, Lancet. 2014 Dec 3. pii: S0140-6736(14)60720-0. IgG4-related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. This multi-organ immune-mediated condition links many disorders previously regarded as isolated, single-organ diseases without any known underlying systemic condition. It was recognised as a unified entity only 10 years ago. Histopathology is the key to diagnosis. The three central pathology features of IgG4-related diseaseare lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis….. The role of the Th17 cytokines IL-17 and IL-22 in Rheumatoid Arthritis pathogenesis and developments in cytokine immunotherapy. Roeleveld DM et al, Cytokine. 2014 Nov 20. pii: S1043-4666(14)00550-X. Over the past few years, the importance of Interleukin (IL)-17 and T helper (Th) 17 cells in the pathology of Rheumatoid Arthritis (RA) has become apparent. RA is a systemic autoimmune disease that affects up to 1% of the population worldwide. By the production of effector cytokines like IL-17 and IL-22, the T helper 17 subset protects the host against bacterial and fungal infections, but it can also promote the development of various autoimmune diseases like RA. Hence, the Th17 pathway recently became a very interesting target in RA treatment….. The biology of IL-23 and IL-17 and their therapeutic targeting in rheumatic diseases. Sherlock JP et al, Curr Opin Rheumatol. 2015 Jan; 27(1):71-5. IL-23 and related cytokines have been found to be up-regulated in rheumatoid arthritis, systemic lupus erythematosus andspondyloarthropathy, and preclinical models suggest that they play important pathological roles in these conditions. It is anticipated that agents which target the IL-23 pathway will have profound roles in modifying the natural history of these diseases and in preventing the structural damage which occurs secondary to such chronic inflammation…… Onset of psoriatic arthritis during ustekinumab treatment for psoriasis: a case series of seven patients. Jones BB et al, Br J Dermatol. 2014 Dec 30. Recently, ustekinumab, a monoclonal antibody that binds to p40, the shared subunit of interleukin (IL)-12 and IL-23, was approved for psoriasis and psoriatic arthritis.. Clinical trials have demonstrated that ustekinumab is a safe and efficacious therapy for psoriasis3 as well as PsA. Authors in this case series have discussed onset of PsA during ustekinumab….. Survival of adults with systemic sclerosis following lung transplantation: A nationwide cohort study. Bernstein EJ et al, Arthritis Rheumatol. 2015 Jan 7. doi: 10.1002/art.39021. The aims of this study were to determine whether adults with SSc have higher 1-year mortality rates after lung transplantation compared to those with interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) not due to SSc. This is a retrospective cohort study of 229 adults with SSc, 201 with PAH, and 3333 with ILD who underwent lung transplantation in the U.S. between May 4, 2005, and September 14, 2012. Authors found that adults with SSc undergoing lung transplantation in the U.S. had a multivariable-adjusted 48% relative increase in the 1-year mortality rate compared to those with non-SSc-related ILD….. Antinuclear antibody-negative systemic sclerosis. Salazar GA Semin Arthritis Rheum. 2014 Nov 28. pii: S0049-0172(14)00293-5. This study included 3249 patients, of whom 208 (6.4%) were ANA negative. The proportion of male patients was higher in the ANA-negative group. ANA-negative patients experienced less vasculopathic manifestations of SSc. The percent predicted diffusing capacity of carbon monoxide (DLCO) was higher in ANA-negative patients. Pulmonary arterial hypertension (PAH) per right heart catheterization was less common in the ANA-negative group . Furthermore, patients with negative ANA had a lower prevalence of telangiectasias and digital ulcers/pits. Although diffuse cutaneous involvement was more common, the modified Rodnan Skin Score (mRSS) was lower in the ANA-negative group. There was no difference in the frequency of pulmonary fibrosis or scleroderma renal crisis….. An Autoimmune Myositis-Overlap Syndrome Associated With Autoantibodies to Nuclear Pore Complexes: Description and Long-Term Follow-up of the Anti-Nup Syndrome. Senécal JL et al, Medicine (Baltimore). 2014 Nov; 93(24):383-94. We describe a novel myositis-overlap syndrome in 4 patients characterized by the presence of a unique immunologic marker, autoantibodies to nuclear pore complexes. The clinical phenotype was characterized by prominent myositis in association with erosive, anti-CCP, and rheumatoid factor-positive arthritis, trigeminal neuralgia, mild interstitial lung disease, Raynaud phenomenon, and weight loss. The myositis was typically chronic, relapsing, and refractory to corticosteroids alone, but remitted with the addition of a second immunomodulating drug. The prognosis was good with 100% long-term survival (mean follow-up 19.5 yr).By indirect immunofluorescence on HEp-2 cells, sera from all 4 patients displayed a high titer of antinuclear autoantibodies (ANA) with a distinct punctate peripheral (rim) fluorescent pattern of the nuclear envelope characteristic of nuclear pore complexes….. Kikuchi-fujimoto disease: retrospective study of 91 cases and review of the literature. Dumas G et al, Medicine (Baltimore). 2014 Nov;93(24):372-82. This retrospective, observational, multicenter study describes kikuchi-fujimoto disease (KFD) in France. We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers. Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis….