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Widespread thrombosis and necrotizing arteritis of the liver in a 69-year-old woman with symptoms simulating those of temporal arteritis.

Autopsy findings of widespread thrombosis and necrotizing arteritis of the liver in a 69-year-old woman who had clinical symptoms suggesting those of temporal (giant cell) arteritis were presented. The lesion of the temporal artery was not of temporal arteritis but of thrombosis, part of widespread thrombosis, which occurred in the small arteries having hypertensive arteriolopathy. This case also had an arteritis of the liver resembling polyarteritis nodosa (PN). The pathologic processes demonstrated in this case suggested that the temporal arteritis-like symptoms may be brought about by occlusion of the temporal artery. Temporal arteritis is a nonfatal and self-limited disease and little information has been obtained from autopsy material. Accordingly, it is necessary to collect autopsy cases of temporal arteritis to investigate this disease through observations of whole bodies. Recently we examined a case which appeared clinically having symptoms of temporal arteritis, but the autopsy revealed that the symptoms were not brought about by temporal arteritis but by thrombosis of the temporal artery. It is the purpose of this paper to present this case and to discuss some problems concerning genesis of symptoms of temporal arteritis.

Aged

Analysis of granulomatous arteritis in MRL/Mp autoimmune disease mice bearing lymphoproliferative genes. The use of mouse genetics to dissociate the development of arteritis and glomerulonephritis.

MRL/Mp mice bearing the lymphoproliferation gene (lpr) spontaneously develop systemic granulomatous arteritis coincident with glomerulonephritis (GNP). Although the association of lpr-dependent lymphoproliferation in these mice seems to be a prerequisite for the development of granulomatous arteritis, the genetic basis is poorly understood. The first approach to this problem was to study the ability of another, nonallelic, lymphoproliferative gene, gld (generalized lymphoproliferative disease), inducing arteritis in MRL/Mp mice. The gld gene was placed on an MRL/Mp background by producing reciprocal (MRL/Mp-+/+ X C3H/Hej-gld/gld)F2 hybrid mice. Seventeen percent of these mice with lymphoproliferation had arteritis and GNP, suggesting that more than one lymphoproliferative gene could induce GNP and arteritis in an MRL/Mp background. Next, the effect of rearrangements in the genetic background of MRL/Mp-lpr/lpr mice by hybridization with non-autoimmune lpr-bearing mice was examined. This was done by making MRL/Mp-lpr/lpr X reciprocal (MRL/Mp-lpr/lpr X C57BL/6-lpr/lpr)F1 mice. Thirty-three percent of these mice developed arteritis, but one third of these did not get GNP, thus showing that susceptibility to arteritis was separate from GNP. The histopathologic features of the arteritis in both the F2 hybrids and the backcross mice were granulomatous and were identical to those seen in MRL/Mp-lpr/lpr mice. These findings suggested that it might be possible to dissociated two components (arteritis and GNP) of a severe autoimmune disease of MRL/Mp mice and to study their pathogenesis separately.

Animals

Giant-cell arteritis, temporal arteritis and polymyalgia rheumatica. A retrospective study of 63 patients.

The initial clinical symptoms, the course of the disease, and the effect of corticosteroid treatment have been analyzed in a retrospective study of 63 patients with temporal arteritis or polymyalgia rheumatica. The relationship between the physical examination of the temporal regions, the ophthalmological examination, and biopsy from the temporal artery with respect to the diagnostic value were examined. Histological examination of biopsy specimens from the temporal artery in 58 patients revealed arteritis in 46. Half of the patients had only local symptoms from the temporal regions; one fourth presented such symptoms as well as myalgias, and one fourth had myalgias only. Patients presenting local symptoms of temporal arteritis as well as of myalgias had always had myalgias as the initial symptom and developed local symptoms of temporal arteritis 1-24 months later. Permanent reduction of vision occurred in 20% of the patients. Symptoms of generalized arteritis were observed in several patients. The overlapping of the clinical symptoms, the positive biopsy findings in patients with polymyalgia rheumatica as the only local symptom and the identical reaction to corticosteroid treatment support the conception of temporal arteritis and polymyalgia rheumatica as two manifestations of the same disease. The physical and the ophthalmological examinations were of limited diagnostic value. Positive biopsy findings were seen in 25 patients with noraml palpatory findings, and in 46 patients without eye symptoms the ophthalmoscopic examination revealed no signs of arteritis. If the first biopsy from the temporal artery is negative, biopsy from the contralateral temporal artery should be performed. Correctly timed corticosteroid treatment in adequate doses can prevent reduction of vision in giant-cell arteritis. The treatment is a long-term therapy, its average duration in the present study being more than two years.

Adrenal Cortex Hormones

Giant cell arteritis of the female genital tract with temporal arteritis.

The clinical and pathological features of a patient with giant cell arteritis of the uterus and ovaries are described. A 61 year old woman had fever and weight loss over a period of eight months. A hysterectomy with bilateral salpingo-oophorectomy was performed for a large cystic ovarian mass. Histological examination showed a benign ovarian cyst and unexpected giant cell arteritis affecting numerous small to medium sized arteries in the ovaries and myometrium. The diagnosis of temporal arteritis was confirmed by a random temporal artery biopsy, despite the absence of symptoms of temporal arteritis. This observation is compared with previously reported cases and the relation between granulomatous arteritis of the genital tract and temporal arteritis is discussed. The main differential diagnosis in this localisation was represented by Wegener's granulomatosis and periarteritis nodosa.

Female

[Takayasu-Onishi arteritis. II. Relations of Takayasu-Onishi arteritis with other non-specific arteritides].

Takayasu-Onishi arteritis (T.O.) is similar to Hutchison-Horton arteritis (H.H.) on histological, clinical, laboratory, and pathogenetic grounds. Both probably depend on immunitary dysreactivity, their different clinical expression being attributable to differences in the district involved and the age of the subject. Both are preceded or accompanied by rheumatism. An interesting relation can be made out between temporal arteritis and "rheumatic polymyalgia" or, more aptly, "rhizomelic polymyalgia" (Ballabio, 1975). The latter (of rheumatic origin) may accompany arteritis - Hamrin, indeed, has suggested their unification in the description "arteritic polymyalgia". It is uncertain whether vasculopathy in the course of collagen disease, rheumatic arteritis, and polyarteritis nodosa can be identified with T.O., even though a common immunological basis can be made out. The difference between T.O. and thromboangiitis obliterans, on the other hand, is quite clear at the present time.

Aortic Arch Syndromes

Giant cell arteritis (temporal arteritis) affecting the breast: report of two cases and review of published reports.

The breast is an infrequently recognised site of primary giant cell arteritis. Two cases of giant cell arteritis affecting the breast are described and 11 previously described cases are reviewed. All cases presented with single or multiple breast masses, leading to a diagnosis before biopsy of suspected breast carcinoma. Symptoms similar to those of polymyalgia rheumatica occurred in about half of these patients, though the significance of these symptoms was only appreciated in retrospect. Clinical or pathological evidence of giant cell arteritis outside the breast (temporal artery, thyroid artery) was noted in a minority of cases. Seven patients received corticosteroid treatment, and all patients recovered without complications. Two patients had an adenocarcinoma of the breast contiguous with the giant cell arteritis. Giant cell arteritis affecting the breast may be underrecognised and should be considered in older women with polymyalgia rheumatica-like symptoms and tender breast mass(es).

Aged

[Liver changes in giant-cell arteritis: temporal arteritis and rheumatic polymyalgia (author's transl)].

Liver changes were demonstrated in six elderly patients with giant-cell arteritis (temporal arteritis, three with polymyalgia). Histologically there was fatty infiltration in four and pericentral congestion in five, star-cell nodules in one and non-specific hepatitis in one. Bromsulphalein test was abnormal in all, but rapidly became normal as the arteritis was successfully treated with corticoids. The pathogenesis of the liver changes is unclear. The authors' observations and published reports suggest that they are typical of giant-cell arteritis; it is of importance in the diagnosis of underlying disease.

Adrenal Cortex Hormones

Giant-cell arteritis of the uterus with associated temporal arteritis: a case report.

Giant-cell arteritis involving the uterus was identified incidentally upon hysterectomy and anterior colporrhaphy for uterine prolapse. Subsequently, the patient was found to have giant-cell temporal arteritis presenting with fever of unknown origin. Fourteen previous cases involving the female genital tract have been reported. There appears to be an association between constitutional symptoms of fever, weight loss, malaise, headache, and polymyalgia rheumatica in elderly women with uterine prolapse and giant-cell arteritis of the genital tract. The rare presentation of giant-cell arteritis in the female genital tract does not support invasive costly evaluation in asymptomatic patients. However, a thorough investigation for involvement of other sites, including appropriate treatment for generalized disease, should be undertaken.

Aged

[Takayasu arteritis associated with heart valve diseases (pulmonary and aortic) and arteritis (coronary and renal)].

The most severe arteritis due to Takayasu's disease are those related to renal and coronary arteries. The first one because it produces severe arterial hypertension and the second one because it puts the patient in high risk of suffering either myocardial ischemia or infarction. These situations worsen when this entity is associated to valvular heart lesions. The authors present the clinical cases of two female patients with Takayasu's disease. One of them in acute phase of the illness, where coronary arteritis, mild coarctation of the aorta, right pulmonary artery stenosis, and pulmonary valve stenosis were present. The second patient was seen during the remission phase of the disease with obstruction of the left subclavicular artery, renal arteritis, severe arterial hypertension and aortic valve insufficiency. The authors discuss the prognosis of patients with Takayasu's disease associated to valvular heart disease and its role in the etiology of pulmonary valvular stenosis. Finally, the authors point out the importance of recognizing the active and non active phases of the Takayasu's disease in relation of the adequate stage for surgical treatment of the lesions caused by this disease.

Adult

Giant cell arteritis (cranial arteritis, polymyalgia rheumatica).

Giant cell arteritis, which is probably due to disturbed immune mechanisms, has a spectrum of clinical symptoms in elderly people. In nearly all cases such general signs as loss of appetite, loss of weight and fever are present. The sedimentation rate is almost without exception about 100 mm in the first hour. The two most frequent and typical clinical syndromes are polymyalgia rheumatica and cranial arteritis. The polymyalgia rheumatica is characterized by periarticular pain which is mostly symmetrical and accentuated in the shoulder girdle. Increasingly severe temporal headache and ocular distrubances are found with cranial arteritis in more than 50% of cases. A combination of both diseases is frequent. Other arterial branches are rarely involved. The course of the disease is over a period of 1 1/2 to 2 years. Treatment with corticosteroids is indicated mainly because of the severe ocular complications with blindness. It should begin immediately, be intensive and last over a long period. Regular followup is necessary over several years in order to avoid relapses.

Age Factors

Arteritis of the aged (giant cell arteritis) and fever of unexplained origin.

Arteritis of the aged (giant cell arteritis) masquerades as a degenerative, infectious, neoplastic or even functional disorder in the elderly. In the absence of obliterative vascular changes, the diagnosis is often overlooked when too rigid diagnostic criteria are employed. Four elderly women presented with fever of unexplained origin as the initial manifestation of this disease. The temporal arteries were conspicuously normal in all four, and other traditional clinical clues, such as visual disturbances, headache or manifestations of polymyalgia rheumatica were likewise infrequent of entirely absent. Influenza immunization and uncomplicated rectal surgery preceded the onset of illness in two. Anemia and an increased erythrocyte sedimentation rate are important diagnostic features, particularly in the face of spontaneous clinical improvement accompanied by defervescence and disappearance of nonspecific liver dysfunction. Occult intestinal perforation complicated steroid therapy in one case. Significant and sometimes hectic fever may be a common pattern for this arteritis in its earliest stages, when palpably abnormal temporal arteries, obliterative vascular changes and other traditional diagnostic clues are more likely to be absent.

Aged

Monozygotic twins with temporal arteritis and ophthalmic arteritis.

Temporal arteritis - ophthalmic arteritis occurring in a pair of monozygotic twins, who were 72 and 77 years of age respectively at the onset of symptoms, is reported. It is pointed out that it is rare to see, as in the first case, an improvement in an already existing severely reduced vision, in relation to corticosteroid treatment. The importance of genetic factors in temporal arteritis is discussed; further it is suggested that a possible association of the disease with tissue type antigens be the object of further study.

Aged

Experimental candida-induced arteritis in mice. Relation to arteritis in the mucocutaneous lymph node syndrome.

An extract of Candida albicans isolated from a patient with typical mucocutaneous lymph node syndrome (MCLS) can produce coronary arteritis in a mouse when injected intraperitoneally. An unusual feature of this arteritis is that it is granulomatous, shows no fibrinoid change and is confined to the coronary arteries. These characteristics are quite similar to those found in patients with MCLS.

Animals

A concept of diffuse actinic arteritis. The role of actinic damage to elastin in 'age change' and arteritis of the temporal artery and in polymyalgia rheumatica.

Actinic damage (actinic elastosis) affecting the internal elastic lamina appears to be the prime cause of 'age change' and arteritis of the temporal artery. Resorption and removal of altered elastin (elastolysis) is an integral part of the pathology of actinic damage. Actinic irradiation is probably responsible for the destruction and disappearance of a vast number of arterioles in elastotic skin. The intimate connection between temporal arteritis and polymyalgia rheumatica prompts the belief that the vascular and other internal malign components of the temporal arteritis/polymyalgia rheumatica syndrome might likewise be due, albeit indirectly, to the same actinic cause. Actinic elastotic damage at the body surface could have this effect by provoking a state of systemic elastolysis. Although ultraviolet (uv) light is often regarded as the sole cause of actinic elastosis, penetrating infrared (heat) irradiation may deserve a large or even a dominant share of the blame.

Adult

[Multi-infarct dementia in giant cell arteritis (temporal arteritis)].

Dementia is an infrequent and little known manifestation of giant cell arteritis or temporal arteritis (GCA-TA). The cases of 2 women with histologically proven GCA-TA are presented in which, together with the most classical symptoms, they presented a brusque mental deterioration on initiation of the disease. One patient developed a severe irreversible dementia which coincided with a rapid reduction in the doses of corticoids; while the other patient demonstrated improvement in cognitive function with steroid treatment and control of the disease. Magnetic resonance studies revealed multiple areas of infarction in both hemispheres in the two patients. The importance of treating multi-infarct dementia is underlined in the context of GCA-TA with high doses of corticoids. It is also emphasized that GCA-TA should be considered in the evaluation of older patients with mental alterations.

Aged

Giant cell arteritis-a systemic spectrum including temporal arteritis and polymyalgia rheumatica.

Polymyalgia rheumatica and giant cell arteritis are closely associated disorders that may pose serious threats to the elderly. While the etiological factors remain unknown, these clinical entities may represent different expressions of a common disorder. Recent evidence suggests that autoimmunological involvement of the internal elastic lamina of the large and medium-sized arteries might play an importent role in pathogenesis. Genetic and environmental factors may also contribute. Polymyalgia rheumatica and giant cell arteritis have subtle and protean manifestations and formes frustes occur, contributing to delays in diagnosis unless the clinician holds a high index of suspicion while treating the elderly. The sequelae, including generalized disability, blindness, myocardial and cerebrovascular catastrophies can probably be avoided by timely and prudent use of corticosteroids. These are not uncommon disorders are among the most amenable to treatment of the rheumatological diseases. Family physicians must be diligent in their efforts to diagnose these disorders since early generalized and focal signs and symptoms frequently precede the catastrophic events.

Adrenal Cortex Hormones

A population-based case-control study of temporal arteritis: evidence for an association between temporal arteritis and degenerative vascular disease?

The first population-based incident case-control study of temporal arteritis (TA) in the US was conducted using the unique data resources of the Rochester Epidemiology Project. During the period 1950-1985, 88 newly diagnosed cases of biopsy-proven TA were identified among residents of Olmsted County, Minnesota. Cases were each matched to four Olmsted County community controls on age, sex and duration of community medical record. Odds ratios (OR) were calculated for marital status, education, Quetelet index, pregnancy, age at menopause, thyroid disease, diabetes, smoking, hypertension, angina, myocardial infarction, peripheral vascular disease, and stroke. Multivariable conditional logistic regression analysis identified statistically significant adjusted OR for smoking (2.3, 95% CI = 1.3-4.1). Elevated ORs which were not statistically significant were noted for angina, myocardial infarction, and peripheral vascular disease. These data suggest that TA and arteriosclerosis may share a common causal pathway. Alternatively, histopathological misclassification of temporal artery biopsies may have resulted in the observed association. Due to the limited power of this population-based study, multicentre collaboration should be encouraged to more precisely define the epidemiology of TA.

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