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A 72-year-old woman presented to our emergency centre with acute horizontal diplopia. Neurological examination revealed an isolated abducens nerve paresis on the left. Hilar enlargement seen on the chest x-ray and an elevated serum ACE level led us to suspect sarcoidosis, but the patient declined further evaluation. In the following days, her visual acuity decreased steadily, and she developed cervicothoracic pain, left sided ptosis, weakness of the right arm, and general asthenia. When she was readmitted as an emergency case, neurological examination revealed decreased visual acuity, external ophthalmoplegia and ptosis on the left and a C8 radicular lesion on the right. Imaging studies showed multilocular lesions, e. g. in the left orbital space, spinal epidural manifestations and lymphoma nodular involvement, including retroperitoneally. Laboratory chemistry showed elevated serum levels of ACE, sIL2 receptor and an elevated CD4 / CD8 ratio while bronchoalveolar lavage indicated lymphocytic alveolitis. The biopsy performed under the left M. masseter with a presumptive diagnosis of sarcoidosis, showed a diffuse large-cell B-cell lymphoma. We initiated immuno-chemotherapy following the R-CHOP schema with a curative approach. The case shows the lack of specificity of clinical, imaging and laboratory findings and thus underlines the need for histology in the differential diagnosis of sarcoidosis., Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

Sarcoidosis is a rare multisystemic chronic inflammatory condition. Typically, there is a big discrepancy between the patient's subjectively perceived symptoms and the presence of clinical signs. Due to the high number of potential sites of onset (e. g. larynx, lungs, heart, liver, kidneys) the affected organs may show functional restrictions that are relevant for decisions related to anesthesia. Therefore, an anesthetists working in a clinical setting should be familiar with the special needs of this patient collective. This article describes the onset of sarcoidosis in a case of an unexpected difficult airway.

Amyloidosis is a rare disease characterized by extracellular deposition of fibrils. Among the most common forms of systemic amyloidosis with renal involvement are AL-amyloidosis based on plasma cell dyscrasia and AA-amyloidosis in chronic inflammatory diseases. Depending on the affected renal compartment, the clinical appearance of renal amyloidosis varies. The pattern of renal amyloid deposition can be glomerular, interstitial, tubular or even vascular. Renal amyloid deposits are detected by renal biopsy. Patients with glomerular deposits typically show severe nephrotic syndrome with volume overload. Patients with predominantly tubulo-interstitial or vascular deposits typically exhibit lower proteinuria and progressive renal impairment. Treatment strategies for renal amyloidosis are primarily based on the treatment of the underlying disease including chemotherapy or stem cell transplantation in AL-amyloidosis or treatment of chronic inflammatory diseases in AA-amyloidosis. Granulomatous interstitial nephritis is the most common renal lesion occurring in sarcoidosis. Therapy of granulomatous interstitial nephritis is mainly based on the use of glucocorticoids., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

Hypercalcemia is a highly prevalent complication of sarcoidosis. A medical history of a patient with sarcoidosis is shown as case report. Depending on the population studied about 2-63% of sarcoidosis patients show hypercalcemia. The major difference in the prevalence of hypercalcemia may be in part due to the undulating course of subacute sarcoidosis, so hypercalcemia may be missed when serum calcium is not frequently measured. Hypercalciuria appears to be twice as prevalent then hypercalcemia and should be looked for in every sarcoidosis patient. Hypercalcemia in sarcoidosis is due to the uncontrolled synthesis of 1,25-dihydroxyvitamin D3 by macrophages. 1,25-dihydroxyvitamin D3 leads to an increased absorption of calcium in the intestine and to an increased resorption of calcium in the bone. Immunoregulatory properties have been ascribed to 1,25-dihydroxyvitamin D3. It is an important inhibitor of interleukin-2 and of interferon-gamma-synthesis, two cytokines that are important in granuloma formation in sarcoidosis. It is thought that 1,25-dihydroxyvitamin D3 counterregulates uncontrolled granuloma formation. Treatment of hypercalcemia depends on the serum level of hypercalcemia and its persistence. Generally sarcoidotic patients should be advised to avoid sun exposition to reduce vitamin D3 synthesis in the skin, to omit fish oils that are rich of vitamin D and to produce more than two liters urine a day by adapting fluid intake. Although severe hypercalcemia seems to be rare, glucocorticosteroid treatment should be started if corrected total calcium level rises beyond 3 mmol/l. If hypercalcemia is symptomatic, treatment should be started even at lower levels. Glucocorticosteroids act by inhibition of the overly 1alpha-hydroxylase activity of macrophages. Alternatively, treatment with chloroquine or ketoconazole can be established. If isolated hypercalciuria without hypercalcemia is present with evidence for recurrent nephrolithiasis, patients can be treated with a thiazide diuretic.

Sarcoidosis is defined as an inflammatory systemic disease; the characteristic morphological feature is the noncaseating granuloma. Typical finding in bronchoalveolar lavage (BAL) is a lymphocytic alveolitis with an increased CD4/CD8-quotient. A higher frequency of sarcoidosis in non-smokers (NS) than in smokers (S) has been reported. The influence of inhalative smoking on demographical data, lung function and results of BAL in prospectively selected patients with sarcoidosis was investigated. 111 NS (Sarcoidosis stage I/II+III 48/63) and 44 S (23/21) were included in the study. 16 patients without provable pulmonary disease (9 NS, 7 S) served as controls. Patients with sarcoidosis ware less often S than NS (28 vs. 72%, p = 0,0001) Controls 44 vs. 56%, p = 0.6 [chi(2)]). Sarcoidosis S were younger than NS (40.4 +/- 11.9 vs. 45.6 +/- 14.7 years, p = 0.009). There were no differences in the IVC (in % predicted). There was a negative effect of smoking on the course of the IVC (% predicted) with incremental age, not seen in the non smoking group (S vs. NS: r = -0.54, p = 0.001 vs. r = -0.13, p = 0.22). In BAL of Sarcoidosis S there was a lower concentration of albumin than of NS (in mg/dl), S vs. NS: 9.5 +/- 5.9 vs. 14.5 +/- 13.4, p = 0.012 and a trend to a less intensive lymphocytic alveolitis (in % of BAL-cells, S vs. NS: 29.2 +/- 21.1 vs. 34.1 +/- 18.6, p = 0.099). Influences of the smoking on the populations of T-lymphocytes could not be seen. (CD4/CD8-ratio S vs. NS 10.0 +/- 11.4 vs. 7. 2 +/- 7.1, p = 0, 25). In conclusion patients with sarcoidosis were more often NS than S. S were younger than NS. A protective effect of smoking on the course of lung function in sarcoidosis could be excluded. In the BAL S demonstrated a lower content of albumin and a trend to a less pronounced lymphocytosis and therefore a less pronounced alveolitis than NS. Influences of smoking on the distribution of the lymphocytic populations were not seen.

Reactive oxygen species (ROS) and cytokines like tumor necrosis factor-alpha (TNF-alpha) play a crucial role as inflammatory mediators in pulmonary sarcoidosis. We examined the antiinflammatory effect of pentoxifylline (POF) on alveolar macrophages (AM) of patients with sarcoidosis in vitro. We could demonstrate that POF (above 4.10(-4) M) inhibited the secretion of superoxide anion and TNF-alpha by AM in a dose-dependent manner via a prostaglandin synthesis-dependent mechanism that was independent of the glucocorticoid receptor. POF is an interesting immunomodulating substance that should be further evaluated in clinical trials.

A 24 year old patient with epigastric pain, polyuria, polydipsia and hypercalcemia was admitted to the hospital. Besides the frequent causes of hypercalcemia such as primary hyperparathyroidism and malignancy-related hypercalcemia we had to consider sarcoidosis because of massive splenomegaly. The interstitial lung disease shown on x-ray films of the chest, the epithelioid granulomas in lung tissue and the increased ACE confirmed the diagnosis of sarcoidosis. Hypercalcemia is found in less than 5% of all cases with sarcoidosis. After treatment with steroids, diphosphonates and diuretics all symptoms and the hypercalcemia improved.

In a retrospective study of 152 patients with histologically proven sarcoidosis (stage I: 76 patients; stage II: 70; stage III: 6) clinical pointers towards respiratory involvement were found in 45%, morphological evidence in the form of epithelioid cell granulomatosis of the mucosa in 20%, and histological findings in the form of nonspecific inflammatory and degenerative mucosal changes in 90%. In 30% of patients there were indications of respiratory tract obstruction in the lung function tests. Circumscribed central granulomatous lesions with stenoses of a segment or lobar bronchus and resulting atelectasis were rare, seen in only three. Generalized central respiratory tract obstruction, diagnosed by an increase in airway resistance, was present in 8-17% and was stage-dependent. It correlated in a statistically significant manner with the occurrence of epithelioid-cell granulomas. Peripheral airway obstruction with involvement of the small airways, of which there was functional evidence, can be caused by peribronchial formation of granulomas, as typically seen in transbronchial lung biopsies. But these findings were not of any clinical significance nor required changes in the therapeutic regimen. Functionally precipitated airway obstruction caused by bronchospasm was not demonstrated in the inhalation carbachol provocation test. On the other hand, a subgroup of sarcoidosis patients with raised serum IgE levels had a significantly lower stimulation threshold of the respiratory tract to carbachol.

Serum carboxypeptidases N (E.C.3.4.17.3) were determined spectrophotometrically in 39 patients with untreated pulmonary sarcoidosis (radiographic stages I-II). Carboxypeptidases N were, due to variable substrate affinity, subdivided into N1 (CN1) and N2 (CN2). The control group consisted of 52 healthy persons. In addition, haemodynamics, chemical pathology, and lung function tests were evaluated. Among controls both CN1 and CN2 were slightly increased in females when compared to males. Patients with pulmonary sarcoidosis had significantly higher CN1 and CN2 activities (P less than 0.005) than the control group. Neither among healthy probands nor among patients with sarcoidosis were there positive correlations between haemodynamics, chemical pathology and lung function data on the one hand and CN1 and CN2 on the other. However, a linear correlation between CN1 and CN2 could be established in both groups (P less than 0.001).

One hundred and sixty-four patients with pulmonary fibrosis were examined by CT and by conventional radiological methods. Sixty patients had asbestosis, thirty-nine silicosis, forty sarcoidosis and twenty-five had idiopathic pulmonary fibrosis. CT is superior to conventional radiography in evaluating interstitial pulmonary changes, particularly of the pleura and the lung parenchyma. Areas of fibrosis and of emphysema can be demonstrated without any overlap. In sixty-nine patients there were some findings which could only be demonstrated by CT. In asbestosis, silicosis and sarcoidosis the CT classification of the lung parenchyma which we have suggested produces significantly better correlation with vital capacity than can be achieved from conventional chest films, according to the guide-lines of the I.L.O.

Sarcoidosis without radiologically demonstrable intrathoracic changes is described, in which there was involvement of the retroperitoneal lymph nodes and of the spleen, together with ascites. Contrary to descriptions in the literature of the lymphographic findings in sarcoidosis, there is evidence of lymphatic obstruction and at operation fluid was found in the peritoneal cavity. The relationship between the lymphatic obstruction which was associated with low-grade peritoneal involvement and the increased amount of peritoneal fluid is discussed.

The possible effects of sarcoidosis of the heart on the conduction system and their treatment are discussed. In the underlying case this rare cardiac manifestation of sarcoidosis resulted in a total AV-block. Rehabilitation of the young patient was facilitated by implantation of an atrial-triggered pacemaker-system which is the best possible way of therapy.

Since prolactin (HPRL) occurs as a separate pituitary hormone in man the paper will discuss results about the mechanisms involved regulation of HPRL secretion. A number of factors which lead to an increase in HPRL secretion are named. The clinical relevance of HPRL inhibiting substances as L-DOPA and 2-Br-alpha-ergocryptine is mentioned. The influence of HPRL in pregnancy and lactation period is described also the regulative effect of HPRL on hormone-dependent breast tumors.

Unlabelled: In longterm rational controls of clinically significant disseminated sarcoidosis of the lungs 1. standardized X-ray-graphs record the change-over of the granulomatous sarcoidosis stage to a fibrotic one, discrete X-ray-alterations put up to optimistic interpretation, 2. standardized function parameters verify the frequently resting decrease in pulmonary function. In controls over 3-5 years the curve of forced exspiration already signalizes dangerous delayed effects of sarcoidosis (deficient load-arterialization?) in spite of an apparently roentgenological improvement. This statement becomes decisive for individual and industrial-medical prognoses. Herefore 3 examples: A picture of structure and function of disseminated sarcoidoses of the lungs before and after a treatment lasting for years (corticosteroids) with standardized controls., Conclusion: A standard X-ray-graph is to be evaluated only in combination with standard function parameters. Pay attention to the signal restriction in --2 sigma.

Pulmonary and bronchial artery angiographic pictures were evaluated in 14 patients with pulmonary disseminations. Diffuse fibrosing alveolitis was present in 4 patients, in 4 other patients secondary pulmonary fibrosis and in 6 patients pulmonary sarcoidosis. Most changes of pulmonary vascularisation were found in the lobular and terminal branches of the pulmonary artery. They consisted in their narrowing and even occlusion. Different degrees of the reduction of pulmonary perfusion could be observed. The most morbid alterations were in fibrosing alveolitis. They were less pronounced in cases of secondary fibrosis, showing a localized character, and in sarcoidosis in which the upper pulmonary parts were involved. Bronchial artery vascularisation was showing hypervascularisation of various degrees. Increased and dilated vessels strongly winding and even reticularly arranged could be regularly found. These alterations were very pronounced in patients with sarcoidosis. After transgressing of the pathological process to the state of fibrosis the vessels were showing a striped course presenting a greater number of broncho-pulmonary anastomoses.