Das ,,periodische Fieber": Vom Ätiocholanolon zum Immunglobulin-D - Langzeitbeobachtung einer Patientin mit adultem Morbus Still

Abstract

Recent reports on "Hyperimmunoglobulinemia D and Periodic Fever Syndrome (HIDS)" prompted a comparative study of the syndromes linked by the cardinal symptom "periodic fever": familial Mediterranean fever, juvenile Still's disease (systemic j.c.A.= juvenilie chronic arthritis), or Wissler's disease, adult Still's syndrome and HID syndrome, respectively. On looking back 30 years to 1966 one is reminded of the severe recurring attacks of fever that were attributed to the pyrogenic sexual hormone metabolite etiocholanolone as "etiocholanolone fever". The "rheumatic etiocholanolone fever syndrome" reported on at that time (4 cases) was in all probability equal to "Still's syndrome in the adult" that was established later. This syndrome is described and exemplified by reporting on a 30-year course in a female patient whose adult Still's syndrome had begun during late adolescence as etiocholanolone fever and then became chronic polycyclically with "erythema multiforme rheumatoides" and symmetric joint deformations (constructive inflammation of the hip joint, or coxarthritis, and carpal ankylosis). Differential diagnosis of these symptomatologically related disease patterns is analysed. They have a "triad" of signs and symptoms in common: attacks of fever, acute-phase reactions including leucocytosis, and arthritides. It is suggested to re-examine in appropriate cases the septiform periodic fever via a technically improved and endocrinological tested etiocholanolone determination in respect of its pathogenesis and to correlate it with the type of fever that is actually present. This might help to differentiate the diagnosis between Still's syndrome on the one hand and HIDS on the other. The same may be expected from the pathohistology of the skin eruptions. The differential criterion which is of cardinal importance for the HID syndrome, namely, elevated IgD (immunoglobulin D), may possibly be the epiphenomenon of an immunopathology related to Still's syndrome. However, HIDS often involves the lymphatic nodes and the gastrointestinal tract while avoiding the remaining visceral manifestations such as pleuritis, carditis and hepatopathy. The clinical symptomatology of HIDS consisting of periodic fever, exanthema, symmetric oligoarthritis, lymphadenopathy and gastrointestinal abdominal crises makes it possible to discriminate between HIDS and familial Mediterranean fever. In fact, in this genetically conditioned disease the pathological condition of the skin an lymph nodes is absent. The "benign chronicity" of HIDS without permanent damage to the joints is an essential mark of differentiation against the chronic form of juvenile and adult Still's syndrome, especially if x-ray morphology is taken into consideration. Hence, it is recommended to distinguish between the more frequent chronic course of Still's syndrome on the one hand and the subacute form on the other, which is self-limiting and is probably identical with Wissler's disease. This yields appropriately different criteria for prognosis and treatment. HIDS does not seem to offer differentiation of the course in respect of Still's syndrome.