By Robert G. Lahita, George Tsokos, Jill P. Buyon, Takao Koike
Systemic lupus erythematosus (SLE), more often than not referred to as lupus, is a protracted autoimmune ailment that could contain each organ procedure of the human physique. In lupus, the body's immune method, which often services to guard opposed to international invaders, turns into hyperactive, forming antibodies that assault general tissues and organs, together with the surface, joints, kidneys, mind, middle, lungs, and blood. Lupus is characterised by means of classes of affliction, referred to as flares, and classes of wellbeing, or remission. Lupus is the prototypic autoimmune ailment and Systemic Lupus Erythematosus examines the complete box of autoimmunity via taking a look at lupus. there's no different publication within the box which conscientiously covers clinical learn, scientific findings, organ process involvement and therapy among its covers. The 5th variation maintains to function an important translational reference for experts within the prognosis and administration of sufferers with SLE, and as a key a device for the dimension of medical task for pharmaceutical improvement and simple learn of the illness and a reference paintings for medical institution libraries. * hugely illustrated and in complete colour all through* simple technology part expanded, allowing the reader to target the latest concepts in molecular medication and its results on disorder expression and remedy* scientific features and new drugs are coated in nice aspect supplying an invaluable connection with execs drawn to lupus
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Additional info for Systemic Lupus Erythematosus, Fifth Edition
Homozygous C2 deficiency is associated with SLE (a recent comprehensive review of this deficiency and rheumatologic manifestation in the Swedish population) . Of interest, the female predominance in C2 deficiency approaches that seen in lupus in general. Also, the clinical picture may be suggestive of the deficient state. In several series these patients tended to have early childhood onset of a milder disease process with prominent photosensitive dermatologic manifestations, speckled ANAs (which on further testing demonstrate that the antibody specificity is common for the Ro (SSA) antigen), and a family history of SLE.
BASIS OF DISEASE PATHOGENESIS 24 2. 4 Schematic diagram of the structural features of human C4. Pro-C4 is the intracellular, single-chain precursor of C4. Following a series of post-translational modifications that include sulfation, glycosylation, and two proteolytic cleavages, a three-chain protein is secreted (C4s). The major form of C4 in plasma, however, is C4p. This form differs from C4s by removal of a 22-amino-acid peptide from the carboxylterminus of the a-chain. The shaded area at the NH2-terminus of the a-chain represents the C4a fragment.
Each C4 gene consists of 41 exons and 40 introns. The thioester is encoded by exon 24, the C4A and C4B isotypic residues are encoded by exon 26, and the Rodgers 1 and Chido 1 blood group antigens are encoded by exon 28. 6 kb in size and contains the endogenous retrovirus HERV-K(C4) in its ninth intron. 36 kb in size, consists of sequences similar to gag, pol and env, is flanked by two long terminal repeats (LTRs), and is organized in opposite orientation with respect to the transcription of human C4.