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Biomedical subjects

J T Lie

Publications and source records attributed to J T Lie.

At least 37 records · Page 2Linked to original sources
PubMed1997

Wegener's granulomatosis: histological documentation of common and uncommon manifestations in 216 patients.

BACKGROUND: Wegener's granulomatosis is the prototype of pulmonary angiitis and granulomatosis and a systemic vasculitic syndrome of unknown etiology. Wegener's granulomatosis can involve virtually any and often a multitude of organ-tissues. PATIENTS AND METHODS: This survey of 216 patients provides a histological documentation and pertinent literature review of both the common and uncommon, but with emphasis on the uncommon, manifestations of Wegener's granulomatosis. RESULTS: The common manifestations of the disease include the classic triad of upper airway, lung, and kidney, in 87%, 69%, and 48% of the patients, respectively. The less common manifestations involve the skin, central nervous system, eye and orbit, heart, breast, salivary gland, gastrointestinal tract, spleen, and male and female urogenital tracts; each of these accounts for less than 15% in all cases and below 5% for most of the patients. CONCLUSIONS: The manifestations of Wegener's granulomatosis in many of the uncommon anatomical sites of involvement may be distinctive or atypical and therefore, the histopathological diagnosis must be correlated with clinical and laboratory test findings.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1996

Primary antiphospholipid syndrome associated with mesenteric inflammatory veno-occlusive disease.

We describe a 24-year old Caucasian man with gangrene of small bowels and intestinal resection due to mesenteric inflammatory veno-occlusive disease (MIVOD) who later developed deep vein thrombosis in his left leg. He had no clinical evidence of an underlying symptomatic connective tissue disease or Behçet's disease. An IgG anticardiolipin antibody titre above 60 GPL unit/mL and thrombocytopenia confirmed the diagnosis of primary antiphospholipid syndrome (APS). This is the first known case of APS associated with MIVOD.

Acute Disease↗
PubMed1996

Vasculitis associated with infectious agents.

The HIV infection epidemic and, with it, the resurgence and emergence of old and new opportunistic microbes, have sparked off a renewed interest in vasculitis associated with infectious agents. Current investigations focus on identification of "newer" infectious agents, unusual clinical manifestations, and methodologic refinement in diagnosis and treatment. This article briefly reviews the background of infection-associated vasculitis and highlights some of the newer and unusual aspects of vasculitis known to be associated with nonviral and viral infections.

Bacterial Infections↗
PubMed1996

Vasculopathy of the antiphospholipid syndromes revisited: thrombosis is the culprit and vasculitis the consort.

Whether the vasculopathy in APS is thrombosis or vasculitis is more than a mere academic interest; the distinction is important not only for unravelling the pathogenesis of vascular injuries in APS but also for selecting the appropriate choice of drug treatment. A diagnosis of vasculitis would call for treatment with corticosteroids and cytotoxic agents which are not without serious side effects and drug toxicity. The same powerful but potentially dangerous drugs are clearly quite ineffectual in treating or preventing thrombosis associated with APS which has been known to respond in the lowly and inexpensive aspirin. The vasculopathy of APS remains almost exclusively thrombotic in nature according to our current state of knowledge, even if one were to accept capillaritis as a bona fide member in the family of vasculitides, the 'microangiitis'. Vasculitis secondary to an independent underlying disease, such as SLE, may coexist with APS in a patient. In the management of APS patients, the distinction between a true vasculitis coincidental with and one that is causally related to APS affects clinical decision making, and not just a matter of semantics or an academic curiosity. In vasculopathy of APS, thrombosis is the culprit and vasculitis, when present, is the consort. This is still true until newer and more convincing evidence emerges and proves to be contrary.

Antiphospholipid Syndrome↗
PubMed1996

Lymphoplasmacytic aortitis and acute aortic dissection. An uncommon association.

A 43-year-old white man with a history of cigarette smoking, hypertension, nephrolithiasis, and cervical degenerative arthritis was hospitalized for sudden-onset severe, substernal, and pleuritic chest pain with epigastric radiation. Despite evaluation, the cause remained unclear and the patient expired on hospital day 5. Autopsy revealed acute Stanford type A aortic dissection, hemopericardium, and hemothorax. Grossly, the aorta and its branches, including uninvolved medium-sized arteries, displayed extreme mural fragility. Microscopic examination showed a primary lymphoplasmacytic aortitis-periaortitis without giant cells. Rents within the tunica media, medial-adventitial inflammation, and elastic fiber disruption were limited to sites of gross aortic dissection. Muscular arteries showed patchy, chronic arteritis-periarteritis without giant cell infiltrate or aneurysm formation. This case documents an unusual association of primary lymphoplasmacytic aortitis and aortic dissection.

Adult↗
PubMed1996

Isolated pulmonary Takayasu arteritis: clinicopathologic characteristics.

Involvement of the pulmonary arteries is common in systemic Takayasu arteritis and, in nearly all of the reported cases, the diagnosis has been based solely on clinical and angiographic evaluations of the patients. Isolated pulmonary Takayasu arteritis occurs rarely and can be diagnosed only after histologic examination of the appropriate tissue specimens; five such patients are described herein. The patients were two men and three women. Their age at diagnosis ranged from 25 to 66 years. The initial clinical diagnosis was thromboembolism in two patients, and primary pulmonary hypertension, pulmonary granulomatosis, or pulmonary tumor or sarcoidosis in the remaining three patients, respectively. All five patients underwent surgery for lung resection (three patients) or for reconstruction/bypass of the obstructed pulmonary arteries (two patients). Three types of vascular lesions were observed in the surgical specimens: the classic large-vessel granulomatous giant cell arteritis, a peculiar type of organized thrombus with prominent recanalization and neoangiogenesis, and plexogenic arteriopathy. Thus, the histopathologic findings of pulmonary Takayasu arteritis is distinctive and differs in many aspects from that of systemic Takayasu arteritis. Moreover, the vascular lesions of pulmonary Takayasu arteritis are distinguishable from those of pulmonary hypertension, sarcoidosis, and other types of pulmonary angiitis and granulomatosis.

Adult↗
PubMed1996

Primary cutaneous granulomatous phlebitis with visceral involvement: unmasking of a masked villain.

Primary cutaneous granulomatous phlebitis (PCGP) is distinctly unusual. The entity was first described in 1954 and, to date, the only four known reported cases of PCGP occurred in two men and two women, all under 40 years of age. The arm and/or leg veins were affected in three patients and mesenteric veins in one; all were diagnosed by means of excisional biopsy specimens. The first three patients presented with a febrile illness, and two of them had elevated erythrocyte sedimentation rates but little else indicative of a systemic disease. Three of the four patients received no immunosuppressive drug treatment. The fourth patient presented with a segmental infarction of the ileum that required a bowel resection. We now describe four new cases of PCGP, in women aged 26, 62, 76, and 38 years, one black, one Hispanic, and two white. The diagnosis of PCGP was made by means of biopsy specimens in two patients, at autopsy in one, and from a below-knee amputation specimen in one. Despite the obvious limited global experience of this rare form of phlebitis, there is ground for uneasiness that PCGP may not be as innocuous a curiosity in surgical pathology as was thought at first sight but a more sinister, little-known villain among the vasculitides that is only now beginning to show its true color.

Adult↗
PubMed1996

Nonsteroidal antiinflammatory drug induced hypersensitivity vasculitis clinically mimicking temporal arteritis.

Not all arteritides of the temporal arteries are giant cell temporal arteritis (Horton's disease). We describe an unusual case of etodolac (Lodine) nonsteroidal antiinflammatory drug (NSAID) induced hypersensitivity vasculitis of the temporal artery clinically simulating giant cell temporal arteritis. NSAID are common drugs and their gastrointestinal and renal side effects are well known. Ours is the first known documentation of NSAID induced hypersensitivity vasculitis isolated to the involvement of a temporal artery.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1996

Female genital tract giant cell arteritis associated with occult temporal arteritis.

Giant cell arteritis (GCA) of the female genital tract has been described as an incidental finding, but associated temporal arteritis (TA) has been rarely reported. We describe a case of female genital tract GCA associated with occult giant cell TA, which in the absence of cranial symptoms was confirmed by a random temporal artery biopsy. The patient remains asymptomatic at 12 month followup after treatment with prednisolone and azathioprine.

Aged↗
PubMed1995

Aortic and extracranial large vessel giant cell arteritis: a review of 72 cases with histopathologic documentation.

Giant cell arteritis (GCA) is closely identified with the temporal arteritis-polymyalgia rheumatica syndrome of the elderly. It is also a systemic disease that can cripple and kill. Up to 15% of patients with temporal arteritis may have angiographic evidence of extracranial GCA, and aortic insufficiency, ruptured aortic aneurysm, aortic dissection, stroke, or myocardial infarction may be the initial manifestation of systemic GCA. A review of 72 cases of aortic and extracranial GCA, all with histopathologic verification of the disease, revealed that 25% of patients with aortic and extracranial large-vessel GCA had asymptomatic temporal arteritis; the ascending aorta and aortic arch were most frequently involved (39%), followed by the subclavian and axillary arteries (26%), and the femoropopliteal arteries (18%). Nine patients (12.5%) underwent an upper or lower limb amputation. Of the 18 patients whose death was directly attributable to extracranial GCA the causes were ruptured aortic aneurysm (6), aortic dissection (6), stroke (3), and myocardial infarction (3). The findings of these 72 cases caution against attributing all aortic and large-vessel arterial disease in the elderly to atherosclerosis and emphasize that timely surgical intervention may be necessary for life-saving and limb-salvage in patients with aortic and extracranial GCA.

Aged↗
PubMed1995

Clinicopathologic correlations of the antiphospholipid syndrome.

Since the original description and definition of the antiphospholipid syndrome (APS), a number of distinct clinical manifestations related to it have appeared in the literature. These include vascular obstruction of both veins and arteries, thrombus formation on the endocardium and its consequences, as well as a group of other conditions where vascular obstructive mechanisms are either incompletely understood or unproven, eg, chorea, avascular necrosis, and pulmonary hypertension. Single vessel (large/medium) involvement or multiple vascular occlusions may cause a wide variety of presentations. Any combination of vascular occlusive events may occur in the same individual, and the time interval between them also varies considerably from weeks to months or even years. Rapid chronological occlusive events occurring over days to weeks have been termed the "catastrophic" APS. Most of these complications may be ascribed to the hypercoagulable state of which antiphospholipid antibodies appear either to be "markers" or intimately connected with the highly complex coagulation mechanisms resulting in thrombotic occlusions.

Antiphospholipid Syndrome↗
PubMed1995

Fatal infantile polyarteritis nodosa with predominant central nervous system involvement.

BACKGROUND: Infantile polyarteritis nodosa usually presents in children under 2 years of age as a multiorgan system disease with signs of congestive heart failure or renal failure. This disease and Kawasaki disease may share certain clinical and pathological features. CASE DESCRIPTION: We describe a child who first presented at 8 months of age with a febrile illness followed by a delay in motor and language development and a mild right hemiparesis. Five years later he died after developing oculomotor dysfunction, hypertension, and intracranial hemorrhage. Autopsy revealed focal segmental necrotizing vasculitis of cerebral arteries, without involvement of coronary or renal vessels. CONCLUSIONS: Although this child was evaluated on several occasions during this time period, the diagnosis was not made antemortem. The predominant central nervous system features, both clinical and pathological, together with the prolonged course are the two unique features of this child's disease that need to be emphasized.

Brain↗
PubMed1995

Pulmonary tuberous sclerosis.

We describe the clinical presentation, pulmonary function tests, chest radiograph, and computed tomography findings, response to hormonal treatment, and duration of survival of nine patients with pulmonary involvement in tuberous sclerosis complex with follow-up over an average of 17 years (range, 1 to 35 years) since diagnosis. All patients were female, and the average age at onset of symptoms was 16 years (range, 3 months to 39 years); pulmonary symptoms did not develop until an average age of 33 years (range, 22 to 46 years). There was an average delay of 8 years before the correct diagnosis was made. The most common presenting clinical features were seizures, pneumothorax, bleeding into a renal angiomyolipoma, dyspnea, and typical skin changes. Pulmonary function tests commonly demonstrated obstruction to airflow and reduced single-breath diffusing capacity. Chest radiograph and computed tomography characteristically demonstrated diffuse interstitial infiltrates with cystic changes. Two asymptomatic patients with mild pulmonary involvement have remained in stable condition without hormonal therapy. The remaining seven patients had moderate to severe airflow obstruction; of these, five underwent hormonal therapy. Three patients had a clinical response to treatment. Two patients who did not receive hormonal treatment died of progressive respiratory failure. Most patients with pulmonary involvement in tuberous sclerosis have a slowly declining clinical course. Although the available data are limited, they suggest that a trial of hormonal therapy is recommended both for symptomatic patients and for those with declining pulmonary function. Tuberous sclerosis complex should be suspected in all patients with the diagnosis of lymphangioleiomyomatosis.

Adult↗
PubMed1995

Fatal hemoptysis in Ehlers-Danlos syndrome. Old malady with a new curse.

We describe the case of a 27-year-old man with Ehlers-Danlos syndrome, type IV. The patient had recurrent and eventually fatal pulmonary hemorrhage. Type IV Ehlers-Danlos syndrome is a rare disorder of type III collagen synthesis. It is characterized by an unusual facies, thin, translucent skin with venous vascular pattern, and hypermobility of the small joints. The cause of death is usually due to rupture of a viscus or a major arterial hemorrhage and, in women, rupture of the gravid uterus. Fatal lung hemorrhage in Ehlers-Danlos syndrome, to our knowledge, has not been previously described.

Adult↗
PubMed1995

The use of clinical characteristics to predict the results of temporal artery biopsy among patients with suspected giant cell arteritis.

OBJECTIVE: To develop a mathematical model which predicts temporal artery biopsy results. METHODS: We collected clinical and laboratory data as well as biopsy results among a consecutive cohort of all individuals who underwent temporal artery biopsy at Mayo Medical Center between January 1, 1988 and December 31, 1991. All biopsies were independently reviewed by one pathologist. Logistic regression was used to identify a set of variables which best predicted the biopsy results. This model was then used to identify patients who were highly likely (> or = 95% predictive value) to have either a negative or a positive biopsy. A receiver operating characteristic (ROC) curve was generated using the best fit model. RESULTS: Of the 525 people in the study, there were 187 men and 338 women. The logistic regression model and the ROC curve generated from this model were of modest value in predicting biopsy results from prebiopsy clinical characteristics. However, this model identified 60 (11%) individuals who had a > or = 95% probability of having a negative biopsy. None of these individuals had any symptoms of claudication, only 5 of 60 (8%) had temporal artery abnormalities on examination, 45 (75%) had synovitis (suggesting an alternate diagnosis), and their median erythrocyte sedimentation rate was only 31 mm/h (Westergren). CONCLUSIONS: In individuals with these findings, we recommend a careful search for other diagnoses before temporal artery biopsy.

Aged↗