Some IMP Terms

·         A large proportion of Ad  and C afferents innervating viscera are completely insensitive in normal noninjured, noninflamed tissue. That is, they cannot be activated by known mechanical or thermal stimuli and are not spontaneously active. However, in the presence of inflammatory mediators, these afferents become sensitive to mechanical stimuli. Such afferents have been termed silent nociceptors, and their characteristic properties may explain how under pathologic conditions the relatively insensitive deep structures can become the source of severe and debilitating pain and tenderness. Low pH, prostaglandins, leukotrienes, and other inflammatory mediators such as bradykinin play a significant role in sensitization.

·         The aPTT has variable sensitivity to lupus anticoagulants, depending in part on the aPTT reagents used. An assay utilizing a sensitive reagent has been termed an LA-PTT. The dilute Russell Viper Venom test (dRVVT) and the tissue thromboplastin time (TTI) are modifications of standard tests with the phospholipid reagent decreased, thus increasing the sensitivity to antibodies that interfere with the phospholipid component. The tests, however, are not specific for lupus anticoagulants, as factor deficiencies or other inhibitors also result in prolongation.

·         Splenectomy in these diseases may be associated with significant tumor regression in bone marrow and other sites of disease. Similar regressions of systemic disease have been noted after splenic irradiation in some types of lymphoid tumors, especially chronic lymphocytic leukemia and prolymphocytic leukemia. This has been termed the abscopal effect.

·         In some patients, Raynaud's phenomenon and other typical features of SSc occur in the absence of detectable skin thickening. This syndrome has been termed SSc sine scleroderma.

·         In juvenile-onset spondyloarthritis, which begins between ages 7 and 16, most commonly in boys (60–80%), an asymmetric, predominantly lower-extremity oligoarthritis and enthesitis without extraarticular features is the typical mode of presentation. The prevalence of B27 in this condition, which has been termed the seronegative, enthesopathy, arthropathy (SEA) syndrome, is approximately 80%. Many, but not all, of these patients go on to develop AS in late adolescence or adulthood.

·         The clinical presentation is so variable that pheochromocytoma has been termed "the great masquerader"

·         Some individuals with phenotypic type 2 DM present with DKA but lack autoimmune markers and may be later treated with oral glucose-lowering agents rather than insulin (have been termed ketosis-prone type 2 DM)

·         some individuals (5–10%) with the phenotypic appearance of type 2 DM do not have absolute insulin deficiency but have autoimmune markers (ICA, GAD autoantibodies) suggestive of type 1 DM (termed latent autoimmune diabetes of the adult LADA). Such individuals are more likely to be <50 years of age, have a normal BMI, and have a personal or family history of other autoimmune disease. They are much more likely to require insulin treatment within 5 years.

·         Hypoparathyroidism can occur in association with a complex hereditary autoimmune syndrome involving failure of the adrenals, the ovaries, the immune system, and the parathyroids in association with recurrent mucocutaneous candidiasis, alopecia, vitiligo, and pernicious anemia. The responsible gene on chromosome 21q22.3 has been identified. The protein product, which resembles a transcription factor, has been termed the autoimmune regulator, or AIRE. A stop codon mutation occurs in many Finnish families with the disorder, commonly referred to as polyglandular autoimmune type 1 deficiency.