Rheumatoid Arthritis: Delsante G

Fietta P, Delsante G, Quaini F. · Dipartimento Medico Polispecialistico, S.D. di Medicina Interna e Reumatologia, Azienda Ospedaliero-Universitaria di Parma, Italy. · Clin Exp Rheumatol. · Pubmed #19327244 No free full text.

Abstract: Autoimmune connective tissue diseases (ACTDs) constitute a heterogeneous group of chronic immune-mediated inflammatory disorders, primarily affecting connective tissues and usually characterized by multisystem involvement with variable and frequently overlapping clinical manifestations. Abnormal immune regulation patterns and persistent inflammation are ACTD hallmarks. In such a context, autoimmunity/inflammation-associated cellular and molecular networks drive a complex of reactions that may involve hemopoietic tissue and peripheral blood cells. Hematologic abnormalities affecting one or more cellular lineages are frequent manifestations of ACTDs, and may represent an important prognostic factor, reflecting the rate of activation of autoimmune/inflammatory processes. Moreover, an increased frequency of hematologic malignancies, mainly lymphoproliferative disorders, has been observed in ACTDs, such as Sjögren's syndrome, systemic lupus erythematosus, rheumatoid arthritis, and polymyositis/dermatomyositis. A proliferative drive likely constitutes the link between chronic immune activation/dysregulation and malignant transformation, creating an increased risk for genetic aberrations that may lead to uncontrolled clonal proliferation. Revealing the nature of lymphomagenesis in relation to autoimmunity/inflammation will allow the identification of subjects at risk in order to select the appropriate diagnostic and therapeutic options. In this paper, the main hematologic manifestations of adulthood ACTDs are reviewed and discussed.

Manganelli P, Salaffi F, Carotti M, Delsante G, Mozzani F. · II Divisione Medica e Reumatologia, Azienda Ospedaliera, Parma. · Minerva Med. · Pubmed #10432956 No free full text.

Abstract: In rheumatic diseases (RD) pulmonary hypertension (PH) may result by either direct damage of the pulmonary arteries (isolated PH) or pulmonary interstitial fibrosis and other causes. PH is an important cause of morbidity and mortality in systemic sclerosis in which it is more frequently isolated in the limited cutaneous variant and secondary to interstitial fibrosis in the diffuse type. In isolated PH the main histopathological finding is an occlusive arteriopathy. The role of recurrent vasospasm ("lung Raynaud's phenomenon") is still being debated. In systemic lupus erythematosus, although uncommon, PH is being increasingly reported and may recognize multiple etiological factors including vasoconstriction, vasculitis, in-situ pulmonary thrombosis or chronic recurrent thromboembolism. PH may be a severe and often fatal complication of mixed connective tissue disease and dermato/polymyositis. PH may also be diagnosed in patients with rheumatoid arthritis, primary Sjögren's syndrome and primary antiphospholipid syndrome. Doppler echocardiography is the technique of choice for the evaluation of PH because it is nonivasive and allows serial determinations of the arterial pulmonary pressure. The therapy of PH associated with RD includes corticosteroids, immunosuppressive drugs, calcium-antagonists, ACE-inhibitors, anticoagulants, O2, prostacyclin or its stable analogue, iloprost. Carefully selected patients may benefit from single lung or heart-lung transplantation.