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The authors present a case report of a 59-year-old female suffering from hyperlipidemia who developed chronic vitamin A intoxication syndrome after ingestion of 30,000 IU retinol/daily over a period of six years. The patient's main complaints included severe headaches, morning nausea, myalgias and disability around the hip, knee, and ankle joints. Radiologically, hyperostosis of the acetabular circumference and the spine was demonstrated. Because of rapidly increasing pain, total hip replacement was performed. Histology of cross sections from the femoral head revealed destructive osteoarthritis. Since no other causative reason was found, retinol may not only be responsible for hyperostotic bone and soft tissue formations but may perhaps also account for rapid progressing of degenerative joint disease. Despite the cessation of vitamin A intake the clinical symptoms persisted due to hyperlipidemia. The enlarged number of chylomicrons and the higher fraction of very low density lipoproteins may represent a second retinyl ester pool in case of overloaded fat storing Ito-cells in the liver. Therefore, rheumatological treatment reducing risk factors such as hyperlipidemia is mandatory.

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A 51-year old patient with congenital basal cell naevus syndrome was treated with etretinate (50-100 mg/daily) over a period of 5 years (cumulative dose 110 g). Clinically he developed a stiff back with pronounced disability of both hips. Radiologically, axial involvement of the spine with severe diffuse idiopathic skeletal hyperostosis especially of the lumbar spine, and ossifications of both hips were documented. Rheumatological implications of long-term therapy with etretinate are discussed.

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Corticosteroids and immunosuppressive agents are standard treatment for polymyositis (PM) and dermatomyositis (DM) respectively. Recent reports have emphasized a potentially successful regimen with intravenous immune gammaglobulins (IVIG). The short term success of this treatment in a personally observed case is described. IVIG treatment resulted in normalization of the serum concentrations of the muscle enzymes after continued inflammatory activity under treatment with azathioprine, cyclophosphamide and methotrexate in combination with corticosteroids. The improvement of PM by IVIG was further documented by an increase in muscle strength of up to 367% of the initial value and a regression of the myositic changes in the muscles of the thighs as evidenced by magnetic resonance imaging (MRI). The therapeutic response was paralleled by reversal of peripheral lymphopenia. Experience with IVIG treatment in PM/DM is reviewed and the potential role of this regimen in the management of PM/DM is discussed.

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Chondroitin sulfate was administered orally to six healthy volunteers, six patients with rheumatoid arthritis and six patients with osteoarthritis. Blood was collected at intervals before and after treatment and the glycosaminoglycan concentration was analyzed in serum using a sensitive assay based on the metachromatic reaction with 1,9-dimethylmethylene blue. The glycosaminoglycan concentration in serum before and after ingestion of chondroitin sulfate was statistically unchanged in all of the subjects studied. We suggest that chondroprotection by orally administered chondroitin sulfate is a biologically and pharmacologically unfounded theory. Any possible benefit to osteoarthritic patients after ingestion of chondroitin sulfate should be sought at the gastrointestinal rather than at the plasmatic or articular cartilage level.

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In an open three-center pilot study, 17 patients suffering from chronic persistent pain syndrome, due to osteoarthritis of the hip and knee or spondylarthrosis, were treated orally with 1800-2400 mg Ibuprofen per day for 3 weeks. The chronic pain syndrome and joint status were assessed by the physician at the beginning, and after 7, 14 and 21 days. Self-assessments were made by the patients six times daily during the full study period by means of battery-driven electronic diaries (E.D.). The data entered by the patients were stored in the E.D. and after online transfer, processed and evaluated in a PC using appropriate software programs. E.D. were returned by 16 of 17 patients for evaluation. The technical functioning of the instrument was perfect; its use was accepted by patients and physicians. The closely-meshed real-time recording of pain course and other subjective data, such as adverse events or medication, etc., enables the physician to calculate more exactly and reliably improvement rates, as well as to carry out prognostic trend analyses and individual benefit-risk-ratio estimates. By comparing different kinds of data, each entered at the same time, plausibility checks are possible. The procedure presented here is considered to be a new valuable tool for reviewing subjective data from clinical drug trials.

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Arthrosis is a collective term the elucidation of which is of decisive importance for the clinical practitioner. The main definition (pathologic/anatomic, radiologic and clinical arthrosis) as well as the pertaining sub-definitions are presented and explained. Both condition definitions latent (dormant) and manifest (active and/or decompensated arthrosis) are clinically relevant. While the cause of primary arthrosis is--presently--still mostly unknown, causes of secondary arthrosis can be defined. Secondary arthrosis is the consequence of a pre-arthrosis. This definition nowadays comprises all kinds of joint lesions (not only of mechanical or traumatic kind) causing an irreversible lesion of the joint organs. Pains of manifest arthrosis are either due to activation (reactive secondary synovitis) or to decompensation (periarthropathy and painful muscular irritations as well as consecutive functional disorders). The distinction is decisive for applying therapeutic means. The clinically subjective and objective symptoms of arthrosis and considerations regarding differential diagnosis are described in detail.

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Heat induced radial segmentation of leukocyte nuclei is an in vitro phenomenon accompanying inflammatory diseases. We studied 62 patients with suspected polymyalgia rheumatica (PMR) to determine, whether heat induced radial segmentation can help in discriminating PMR from other conditions. At the initial presentation patients with PMR had more radial segmentation formation than patients with other inflammatory conditions. Prednisone induced a rapid and marked decrease in radial segmentation formation in patients with PMR. This latter finding was much less marked in patients with other inflammatory conditions. We conclude that heat induced radial segmentation at the initial presentation and during prednisone treatment can help in discriminating PMR from other inflammatory conditions.

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Periarthropathia humeroscapularis (PHS) is a malfunction of the shoulder girdle due primarily to diseases of the periarticular tissue and the joint capsule. Clinically a primary and a secondary syndrome are defined. Spondylogenous cervical spine conditions play an important role as causative factors in secondary PHS, among them cervical disc herniation. Among 141 patients operated on for cervical disc herniation 14 presented with a PHS-syndrome prior to surgery, and 31 patients developed the syndrome postoperatively. The patients belong to a fairly young population and showed no high incidence of a particularly severe cervical spondylosis.

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In addition to the habitual skin manifestations, the following forms of organic involvement were found (with decreasing frequency) in 33 patients with progressive systemic sclerosis treated from 1972-1986 at the Clinic of Rheumatology, University Hospital of Zürich: blood vessels 29, lungs 18, digestive system 11, joints 11, bones 8, lacrimal and salivary glands 7, musculature 3, heart 3, kidneys 2, cranial nerves 1 and peripheral neuropathy 1.-A 65-year-old patient with classic progressive systemic sclerosis and visceral involvement of the esophagus and the lungs showed, as a principal sign, unilateral neuropathy of the trigeminal nerve involving all three branches as well as absent corneal reflex. Electromyographic examination afforded evidence of a sensory neuropathy of the trigeminal nerve. Chemical laboratory tests revealed a typical high titre of antinuclear antibodies. In addition to autoimmunological causes, proliferation of fibrous structures of the perineurium and vascular lesions are discussed in connection with these complications.-The subsequent history of 37 patients with localized scleroderma and 4 patients suffering from progressive systemic sclerosis, treated at the Clinic of Dermatology, University Hospital of Zürich from 1980-1985 provided no evidence of a cranial nerve involvement. The fact that during the period 1975 to 1985 only one patient treated at the Clinic of Neurology of Zürich University Hospital showed signs of disturbances of pupil motility confirms the very rare involvement of the nervous system in progressive systemic sclerosis.

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To determine the clinical significance of the Raji cell radioimmunoassay as a laboratory marker of activity, four patients with systemic lupus erythematosus (SLE) were followed for a period of 30 to 90 months. These sequential analyses did not show a correlation between circulating immune complexes, determined by Raji cell radioimmunoassay, and signs and symptoms in these lupus patients over a period of several years. We believe that the Raji cell test offers no advantage over other well known immunologic parameters used in SLE (antibodies to native DNA, complement factors C3 and C4 or CH50). In the management of SLE patients, laboratory data should not serve as a key by which to adjust treatment with prednisone or immunosuppressive drugs; clinical data are of outstanding importance in this respect.

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During the years 1984-1986 we saw 22 patients with cryoglobulinemia, 8 of whom had rheumatoid arthritis, 1 psoriasis arthritis, 2 Sjögren syndrome, 1 progressive systemic sclerosis, 5 different arthralgias, 1 soft tissue rheumatism, 2 vasculitis and arthralgias and 2 purpura-arthralgia-nephritis syndrome. The measurement of cryoglobulins is done only in special cases of diagnostic importance. The prognostic outlook in patients with nephritis is bad. A monoclonal cryoglobulinemia is always suspicious for a lymphoproliferative syndrome.

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Polyneuropathy due to vasculitis in connection with rheumatoid arthritis is a rare complication of the disease. Special investigations can help us to distinguish between a peripheral sensory-neuropathy with a good prognosis, the neuropathy of the autonomous nervous system and the severe form of mononeuritis multiplex. The bad prognosis of this form is due to the diffuse vasculitis of internal organs. We describe 5 patients with rheumatoid arthritis and different forms of vasculitis. The differential diagnosis, therapy and prognosis are described in detail.

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Osteoarthritis (OA) is one of the most common diseases requiring treatment. A clear clinical and pathophysiological understanding of the disease is required before treating a patient with OA. Drug therapy of patients with OA is mainly divided into two groups: a group receiving symptomatic therapy and a group receiving drugs intended to modify or improve the disease. Symptomatic therapy includes pure analgesics as well as non-steroidal antirheumatic drugs and muscle relaxants. Their use depends on the activity as well as on the form of clinical manifestation (activated/decompensated disease). If there are signs of severe activation, intra-articular steroids, orgotein or even synoviorthesis with yttrium are administered as local therapy. All of these therapies are used in the sense of trouble shooters, as they cannot alter the course of the disease in the long run. The disease-modifying substances have well been investigated in vitro and in animal models. Furthermore, some clinical trials have shown evidence for the usefulness of these substances in the therapy of OA.

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Immunohistochemical studies were performed on the temporal artery of 34 patients with clinically established polymyalgia rheumatica (PR) or temporal arteritis, 6 patients with vasculitis, and 25 patients with various diseases. The combined immunofluorescence and peroxidase-anti-Peroxidase Methode zeigte Immunoglobulin- und C3-Ablagerunin histologically affected and to some degree also in unaffected arteries of patients with PR and in all patients with temporal arteritis. The deposits were found both inter- and intracellularly, and contained IgA and to a lesser extend IgG, IgM, and C3. Linear deposits of leukocyte elastase were found along the fragmented internal lamina, and decaying polymorphonuclear (PMN) leukocytes surrounded by elastase-containing inclusions were found in the neighborhood of zones rich in elastic material. These findings suggest that immune complex deposition is a prominent feature of temporal arteritis and that the PMN elastase is probably involved in the destruction of elastic fibers. The combined immunohistochemical investigation appears to increase the diagnostic value of temporal artery biopsy.

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