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Biomedical subjects

P Vaith

Publications and source records attributed to P Vaith.

At least 19 recordsLinked to original sources

[Blindness in both eyes due to late diagnosis of giant cell arteritis].

BACKGROUND: Giant cell arteritis (GCA) is often diagnosed very late, variable "facets" of the disease exist render the diagnosis more difficult. METHODS/PATIENTS: Follow-up observations of five very old patients are reported in whom diagnosis was made too late, resulting in blindness of both eyes. RESULTS: Five patients (age 76-84 years, 4 women, one man) with GCA became blind in both eyes because diagnosis had been delayed (two patients) or onset of therapy was too late (three patients). In two patients who also had arterial hypertension, the symptom "headache" had been misleading. Symptoms of accompanying general diseases masked the real diagnosis, particularly in the second patient who had renal insufficiency, coronary artery disease, and unilateral obstruction of the internal carotid artery. Symptoms that failed to lead to the correct diagnosis were: muscle or chewing pain (three patients), circumscribed numbness around the mouth (second patient), and persistent headache despite normalization of blood pressure. Normal findings from cranial CTAs (two patients) led to the wrong reassurance of the patient. Swelling of the optic disk (two patients) was misdiagnosed by ophthalmologists, as was a retinal branch arterial occlusion (first patient). Three patients, afraid of possible side effects caused by glucocorticoids, took ineffective alternative medications. Poor vigilance led to blindness of the fifth patient with long-standing polymyalgia rheumatica. CONCLUSIONS: Targeted examinations at the onset of symptoms are necessary. GCA-symptoms were mis-constructed by additional diseases that disguised the correct diagnosis. The danger of bilateral blindness is particularly great in patients of great age.

Aged↗

Assessment of the cranial involvement pattern of giant cell arteritis with 3T magnetic resonance imaging.

OBJECTIVE: To noninvasively determine the involvement pattern of the cranial arteries in giant cell arteritis (GCA), with high-resolution magnetic resonance imaging (MRI). METHODS: The superficial cranial arteries of 21 patients with suspected GCA were examined using a 3T high-field MRI scanner. Postcontrast T1-weighted spin-echo images were acquired with submillimeter spatial resolution, to assess mural thickness and lumen diameter of the major cranial arteries on both sides of the head. In all cases, MRI results were compared with findings of clinical examination and laboratory tests. In addition, temporal artery biopsy specimens from 10 patients were examined by histology. RESULTS: MRI sharply revealed all of the major superficial cranial arteries, allowing for an evaluation of their lumen and vessel wall. Nine of the 21 patients were diagnosed as having GCA according to the criteria of the American College of Rheumatology. In all of these patients with clinically diagnosed GCA, multiple cranial arteries showed signs of inflammation on MRI. In 1 patient, the occipital arteries were inflamed, while the temporal arteries were spared. CONCLUSION: Postcontrast high-resolution MRI visualizes the major cranial arteries on both sides of the head within a single examination. The cranial involvement pattern in GCA can be assessed precisely and noninvasively. In the majority of GCA patients, several cranial arteries were affected simultaneously, with a predominance of involvement of the frontal branch of the superficial temporal artery. Inflammation of the occipital arteries, with sparing of the temporal arteries, was also encountered.

Aged↗

Immunosuppressive treatment of chronic periaortitis: a retrospective study of 20 patients with chronic periaortitis and a review of the literature.

BACKGROUND: Retroperitoneal fibrosis (RPF) and inflammatory aneurysm of the abdominal aorta (IAAA) are regarded as two manifestations of the same disease, termed "chronic periaortitis". OBJECTIVE: To determine the optimal therapeutic and diagnostic approaches to IAAA. METHODS: The outcome of medical immunosuppressive and surgical treatment of 20 patients was examined. Measurements of the C reactive protein (CRP) were compared with contrast enhanced imaging studies in the follow up of the patients. RESULTS: The diameter of the periaortic mantle and its contrast enhancement improved in 13/15 (87%) patients given immunosuppressive treatment for a period of more than 6 months. Strong contrast enhancement was associated with a substantial rise in CRP, but no correlation between the CRP value and thickness of the fibrotic mass was found, even at intraindividual follow up. CONCLUSIONS: Immunosuppressive treatment should be included in the first line treatment of patients with RPF and should be maintained long term. Imaging studies are better than CRP measurements in the evaluation of response to treatment.

Adult↗

[Acute arthritis].

In the case of inflammatory disease of the joints, the first diagnostic step is to determine whether the patient has monoarthritis, oligoarthritis or polyarthritis. After excluding a septic or a crystal-related process by joint puncture and synovial fluid analysis, further laboratory investigations including immunological and bacteriological tests can contribute to the diagnosis, and imaging procedures may also be useful. Primary therapy can be provided with NSAIDs or easy-on-the-stomach coxibs. Where indicated, antibiotics or, if warranted by the diagnosis and in the presence of persistent effusion, corticosteroids.

Acute Disease↗

Infectious CNS disease as a differential diagnosis in systemic rheumatic diseases: three case reports and a review of the literature.

BACKGROUND: Immunosuppressive treatment of rheumatic diseases may be associated with several opportunistic infections of the brain. The differentiation between primary central nervous system (CNS) involvement and CNS infection may be difficult, leading to delayed diagnosis. OBJECTIVE: To differentiate between CNS involvement and CNS infection in systemic rheumatic diseases. METHODS AND RESULTS: Three patients with either longstanding or suspected systemic rheumatic diseases (systemic lupus erythematodes, Wegener's granulomatosis, and cerebral vasculitis) who presented with various neuropsychiatric symptoms are described. All three patients were pretreated with different immunosuppressive drugs (leflunomide, methotrexate, cyclophosphamide) in combination with corticosteroids. Magnetic resonance imaging of the brain was suggestive of infectious disease, which was confirmed by cerebrospinal fluid analysis or stereotactic brain biopsy (progressive multifocal leucoencephalopathy (PML) in two and nocardiosis in one patient). DISCUSSION: More than 20 cases of PML or cerebral nocardiosis in patients receiving corticosteroids and cytotoxic drugs for rheumatic disease have been reported. The clinical aspects of opportunistic CNS infections and the role of brain imaging, cerebrospinal fluid analysis and stereotactic brain biopsy in the differential diagnosis are reviewed.

Adult↗

In polymyalgia rheumatica serum prolactin is positively correlated with the number of typical symptoms but not with typical inflammatory markers.

OBJECTIVES: Hyperprolactinaemia has been associated with the active phase of human systemic lupus erythematosus and rheumatoid arthritis. In the present study, we investigated the role of prolactin (PRL) in relation to the number of typical symptoms and serum markers of systemic inflammation in patients with polymyalgia rheumatica (PMR). METHODS: One hundred and two PMR patients presented with typical symptoms such as adynamia, bilateral muscular pain in shoulders, upper arms or neck, bilateral muscular pain in the pelvic girdle, headache, morning stiffness, arthralgia, symptoms of depression, fever, initial weight loss (>4 kg/month), and transient visual symptoms. If one of the mentioned symptoms was present, the corresponding item was scored with one point (maximum unweighted item points=10). PRL, interleukin-2 (IL-2), IL-6, IL-1 receptor antagonist (IL-1ra), tumour necrosis factor (TNF), soluble IL-2 receptor (sIL-2R), and soluble vascular cell adhesion molecule (sVCAM) were measured by enzyme-linked immunosorbent assay in patients and 31 age-matched healthy controls. RESULTS: Fifteen PMR patients with elevated PRL had a higher number of symptoms as compared with patients with normal levels (P=0.003). PRL was correlated with the number of symptoms (all PMR patients: r(rank)=+0.380, P<0.001) and duration of morning stiffness (all PMR patients: r(rank)=+0.335, P=0.002) irrespective of prior corticosteroid treatment. However, PRL did not correlate with markers of systemic inflammation such as erythrocyte sedimentation rate, C-reactive protein, serum IL-1ra, IL-2, sIL-2R, IL-6, TNF, and sVCAM. CONCLUSION: The number of symptoms in PMR patients was positively correlated with PRL, but PRL was not correlated with serum markers of inflammation. This indicates that PRL is not a pro-inflammatory stimulus in patients with PMR. The inter-relationship between PRL and symptoms or duration of morning stiffness may be more a sign of central nervous system involvement, as it can be observed in people with depressed mood or under psychological stress.

Aged↗

Occurrence of C-reactive protein in cryoglobulins.

A previous case report described the formation of a complex between a monoclonal IgA with cryolabile properties and C-reactive protein (CRP). Our study provides the first evidence for the frequent occurrence of CRP in cryoglobulins (Cg) of all three types according to Brouet's classification. We performed a systematic immunochemical analysis of cryoglobulins from 18 patients by Western blotting and in 15 of 18 cryoprecipitates a single band (23 KD), immunoreactive with anti-CRP antibody, was demonstrable irrespective of the clonal composition of the cryoglobulins. This band was detectable in 4/5 of type I, in 6/8 of type II, and in 5/5 of type III cryoprecipitates, classified according to Brouet et al. In addition, the complement proteins C1q and C3 were present in nearly all CRP-containing cryoglobulins, presumably reflecting previous activation of the classical complement pathway at least. All three CRP-negative cryoprecipitates were derived from sera with low cryoglobulin content (1-2 g/l). Longitudinal investigation of 23 cryoprecipitates from seven patients confirmed that successful detection of CRP by Western blotting depends on the protein concentration of the cryoglobulins. Since complexed CRP was previously shown to be an effective activator of complement, via C1q binding, CRP may modulate pathophysiologic effects mediated by cryoglobulins in vivo.

Adult↗

Inflammation and advanced glycation end products in uremia: simple coexistence, potentiation or causal relationship?

The causes for the high frequency of cardiovascular disease in dialysis patients are multifactorial in origin. Disturbances in the carbohydrate and lipid metabolism, the balance between oxidants and antioxidants and the immuno-inflammatory system are thought to play a role. Chronic uremia is characterized by the accumulation of advanced glycation end products (AGEs) and advanced oxidation products (AOPP) as well as activation of the acute phase response. High serum levels of these products and acute phase reactants such as C-reactive protein (CRP), fibrinogen and serum amyloid A can be found. CRP has been shown to predict cardiovascular and overall mortality in hemodialysis patients. Whether CRP is involved causally in atherosclerosis or merely represents a marker of disease is as yet unknown. Since CRP has been detected in colocalization with modified apolipoproteins or complement components in atherosclerotic lesions, a pathophysiological role seems very likely. AGEs as well have been detected in aortas of hemodialysis patients. Incubation of endothelial cells with AGEs induced expression of adhesion molecules with consecutive attraction of monocytes to the vessel wall. Thus far, clinical studies investigating the predictive effects of AGEs on cardiovascular mortality in hemodialysis patients are lacking. There is considerable debate about what factors turn on the acute phase response in this population. Proinflammatory effects of AGEs mediated through one receptor for AGEs, RAGE, have been described. We hypothesize that there may be a link between increased hepatic CRP production and the accumulation of AGEs in uremia. AGEs may stimulate CRP production in hepatocytes either directly or indirectly via interaction with monocytes.

Acute-Phase Reaction↗

[Ophthalmoscopic findings in 3 patients with panarteritis nodosa and review of the literature].

BACKGROUND: Ocular involvement in panarteritis nodosa (PAN) has been reported to occur in 10 to 20% of patients. In 3 patients with acute visual disturbance we point out unusual findings. PATIENTS: Case 1. A 40-year-old man initially presented with papilledema together with partial optic atrophy in both eyes, later polyneuropathy, gangrene of the toes and myalgic pains developed. Caliber changes in the small arteries in the liver were seen angiographically and recognized as signs of PAN. Under treatment with cyclophosphamide und prednisone no relapse occurred during a follow-up of 2 years. Case 2. In a 67-year-old man who suffered from arterial hypertension and coronary heart disease, central retinal artery occlusion occurred, at first in the left and then later in the right eye. The clinically suspected diagnosis of PAN (arterial hypertension, myalgia, polyneuropathy) was confirmed by a muscle biopsy. During a follow-up of 4 years--including treatment with prednisone and cyclophosphamide--no relapse occurred. Case 3. A 16-year-old adolescent with throbbing headaches and a thickened right temporal artery reported visual disturbances. These were due to an inflammation of choroidal vessels of the right eye appearing as an initial sign of PAN. Histology revealed a necrotising arteritis of the temporal artery. He presented with signs of Raynaud's disease, cachexia and arterial hypertension. Multiple vasculitic changes were detected by aorto-arteriography. Five months after the visual deterioration an anterior spinal artery syndrome with quadriplegia developed. After a follow-up of 2 years and treatment with prednisone und cyclophosphamide, he still had paralysis of both legs. The visual acuity was 1.0 in each eye. CONCLUSION: PAN should be considered in differential diagnosis in patients with acute inflammatory signs of the optic nerve head, the choroid and/or the retina together with general signs of the disease. If the disease is suspected, a muscle biopsy is indicated.

Adolescent↗

The adrenal steroid status in relation to inflammatory cytokines (interleukin-6 and tumour necrosis factor) in polymyalgia rheumatica.

OBJECTIVES: To determine the correlation between inflammatory cytokines and adrenal hormones in patients with polymyalgia rheumatica (PMR) and to compare the ratio of serum cortisol and androstenedione (ASD) or dehydroepiandrosterone sulphate (DHEAS) in normal subjects with PMR patients. METHODS: In 102 patients with PMR (32 beginning and 70 chronic disease) and 31 age-matched and sex-matched healthy subjects, ASD, cortisol, DHEAS, interleukin-6 (IL-6), and tumour necrosis factor (TNF) were measured by immunometric assays. RESULTS: Serum levels of IL-6 were elevated in patients with PMR as compared with normal subjects (10.0 +/- 1.6 vs 2.1 +/- 0.1 pg/ml, P = 0.01), which was not found for TNF. In PMR patients, serum levels of IL-6 were positively correlated with serum levels of ASD (P < 0.001), cortisol (P < 0.001), and DHEAS (P = 0. 038) irrespective of corticosteroid treatment. Serum levels of cortisol in relation to IL-6 were significantly lower in patients with chronic disease and long-standing corticosteroid administration as compared with patients with recent onset of the disease and without corticosteroid therapy (P < 0.01). CONCLUSIONS: In PMR, as expected, there was an increase in IL-6 serum levels that was associated with elevated serum levels of ASD, DHEAS, and cortisol which was more marked in patients with recent-onset disease and without corticosteroids. However, serum levels of cortisol in patients with and without corticosteroids were lower than expected by considering the inflammatory status (increased IL-6). This may indicate a change in the hypothalamic-pituitary-adrenal (HPA) axis responsiveness to inflammatory stimuli such as IL-6 during chronic disease. Furthermore, there seems to be a shift of biosynthesis to cortisol in relation to DHEAS or ASD in chronic disease.

Adrenal Glands↗