Macrophage activation syndrome triggered by active systemic lupus erythematosus : Successful treatment by interleukin-1 inhibition (anakinra).

This article presents a case of fulminant macrophage activation syndrome (MAS) as a rare complication of active systemic lupus erythematosus in a 33-year-old female patient. Initial presentation showed severe lupus disease exacerbation with renal involvement, hemolytic anemia, and neuropsychiatric changes. Early therapy focused on broad immunosuppression (high-dose corticosteroids and cyclophosphamide); however, disease remission could not be achieved. After an additional inflammatory focus and underlying malignancy were excluded, the triplet of pancytopenia, fever, and high ferritin levels indicated MAS, a bone marrow biopsy confirmed secondary hemophagocytic histiocytosis. Treatment with an interleukin­1 antagonist (anakinra) induced a fast, effective therapeutic success.

Diagnostic Gain from Surgical Biopsy for Interstitial Lung Disease - When is it Worth the Risk?

BACKGROUND: History, clinical presentation, lung function testing, radiographs including HRCT and nonsurgical biopsy techniques in most cases provide sufficient information for classification of interstitial lung disease (ILD). However, in a small percentage it is not possible to establish the diagnosis so that lung biopsy may be required. We analyzed under which circumstances a reduction of invasive procedures is reasonable. METHODS: Between January 1997 and December 2009 we examined 3399 specimens from 1299 patients with benign inflammatory and granulomatous diseases in whom ILD was clinically hypothesized. We compared the probability of disease according to Bayes before and after surgery which corresponds to the clinical diagnosis (a priori probability) and the final diagnosis (a posteriori probability). Additionally, procedures, operation related complications and the patients' smoking habits were documented. RESULTS: In 111 patients (8.5 %) surgical evaluation was performed (14 mediastinoscopies, 97 thoracotomies/VATS biopsies). All mediastinoscopies substantiated a epitheloid cell granulomatosis. In 30 % of all VATS procedures a prolonged air leak of more than 4 days was observed. One patient died and one had to get a new chest tube after removal. Changes of a priori/a posteriori probabilities was shown for non-smokers in Wegner's granulomatosis (0.6 vs. 2.2 %) and IPF (16.7 vs. 34.8 %), for smokers in Langerhans' cell histiocytosis (1.4 vs. 7.8 %) and IPF (16.7 vs. 33.3 %). In the majority of cases even a reduction of probability was seen. CONCLUSION: Considering complications and limited diagnostic gain, lung biopsies for diagnosis of ILD should be recommended only in selected patients.

[German guideline for diagnosis and management of idiopathic pulmonary fibrosis]. / S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose.

Idiopathic pulmonary fibrosis is a fatal lung disease with a variable and unpredictable natural history and limited treatment options. Since publication of the ATS-ERS statement on IPF in the year 2000 diagnostic standards have improved and a considerable number of randomized controlled treatment trials have been published necessitating a revision. In the years 2006 - 2010 an international panel of IPF experts produced an evidence-based guideline on diagnosis and treatment of IPF, which was published in 2011. In order to implement this evidence-based guideline into the German Health System a group of German IPF experts translated and commented the international guideline, also including new publications in the field. A consensus conference was held in Bochum on December 3rd 2011 under the protectorate of the "Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin (DGP)" and supervised by the "Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften" (AWMF). Most recommendations of the international guideline were found to be appropriate for the german situation. Based on recent clinical studies "weak negative" treatment recommendations for pirfenidone and anticoagulation were changed into "weak positive" for pirfenidone and "strong negative" for anticoagulation. Based on negative results from the PANTHER-trial the recommendation for the combination therapy of prednisone plus azathiorpine plus N-acetlycsteine was also changed into strong negative für patients with definite IPF. This document summarizes essential parts of the international IPF guideline and the comments and recommendations of the German IPF consensus conference.

[Non-specific interstitial pneumonia (NSIP)]. / Nicht spezifische interstitielle Pneumonie.

Non-specific interstitial pneumonia (NSIP) belongs to the group of idiopathic interstitial pneumonias (IIP). However, NSIP can also be found in several other diseases. For example, the NSIP pattern is most commonly found in interstitial lung disease due to connective tissue disease. In this review, the definition and classification, aetiology, pathogenesis and histology, clinical symptoms, serological markers, lung function parameters, radiographic signs, treatment, and prognosis of NSIP are presented. Idiopathic NSIP as a distinct form of NSIP will be discussed separately.

Usefulness of exhaled nitric oxide to guide risk stratification for bronchiolitis obliterans syndrome after lung transplantation.

The aim of this study was to assess fractional exhaled nitric oxide (FeNO) for the early diagnosis of bronchiolitis obliterans syndrome (BOS) after lung transplantation (LTX). 611 FeNO measurements in 166 consecutive patients were classified depending on BOS stage at the time of assessment and course during minimum follow-up of 3 months: (1) stable non-BOS, (2) unstable non-BOS, (3) stable BOS and (4) unstable BOS. Unstable course was defined as new onset of BOS≥1 or progression of BOS. FeNO before unstable course was significantly increased in comparison to their stable counterparts (non-BOS: 28.9 ± 1.2 ppb, n = 40 vs. 16.4 ± 0.8 ppb, n = 131 and BOS: 32.5 ± 1.3 ppb, n = 35 vs. 15.3 ± 0.8 ppb, n = 26; p = 0.01 each). Average time from FeNO reading to onset of deterioration was 117 ± 9 days in non-BOS and 136 ± 9 days in BOS patients. The positive and negative predictive value of FeNO >20 ppb for BOS was 69.0% and 96.9%, respectively. Serial measurements demonstrated significantly lower mean individual variation in stable recipients as compared to stable patients switching to unstable course (3.2 ± 0.3 ppb vs. 12.7 ± 1.4 ppb, p = 0.02). In particular, the excellent negative predictive value of persistently low FeNO readings for future BOS make FeNO assessments a useful tool for continuous risk stratification after LTX.

[Histomorphology of interstitial lung disease and pulmonary vasculitis]. / Histomorphologie interstitieller Lungengerüstveränderungen und pulmonaler Vaskulitiden.

Rheumatic diseases or collagen vascular diseases represent a heterogeneous group of immunologically mediated inflammatory disorders. The respiratory system is often affected,the causes being manifold: infection, medication toxicity and specific manifestations of immunological processes due to the underlying disease. The lung can be involved in all its components. Due to their extremely broad differential diagnosis, pulmonary vasculitic syndromes still constitute a major challenge for the pathologist. Pulmonary involvement is frequent in primary systemic vasculitis (PSV) associated with anti-neutrophil-cytoplasmic antibodies (ANCA); other PSV only rarely affect the lungs. Histomorphologically, small vessel vasculitis with neutrophil alveolitis and diffuse alveolar hemorrhage, as well as extravascular intraparenchymal or peribronchial granulomas, can point to PSV. A single biopsy is often insufficient to identify all diagnostic criteria. Therefore, the selection of suitable biopsy material and correlation with clinical, serological and radiological parameters is indispensable. Almost all forms of interstitial lung disease may be present in collagen vascular disease; however, several parallel morphological types, rather than one in isolation, are frequently found.

[Pleural metastases of a typical bronchial carcinoid 7 years after lobectomy]. / Pleurales Rezidiv eines typischen pulmonalen Karzinoids 7 Jahre nach Operation.

BACKGROUND: Bronchial carcinoids are a rare differential diagnosis of solitary pulmonary nodes. Because of their typical manifestation in the major bronchi, carcinoid tumours are visible regularly via bronchoscopy where they show a typical picture. In lymph node-negative disease a favourable outcome can be expected. Typically metastases develop in the lung, liver, brain, bone and adrenal glands. CASE REPORT: Seven years after lobectomy of a bronchial carcinoid, a slow-growing thickening of the pleura parietalis was noted in a 54-year-old male patient. No clinical signs of neuroendocrine activity were seen. The histological diagnosis of pleural metastases was established via trans-thoracic punctation. Pleural metastases of bronchial carcinoids are extremely rare. Only two other cases have been reported so far. Palliative cytotoxic chemotherapy was started. CONCLUSIONS: The postoperative prognosis of bronchial carcinoids in lymph node-negative disease is excellent. Metastatic disease--as in the rare case of pleural metastases shown here--remains a therapeutic dilemma. Extensively evaluated concepts for adjuvant or palliative settings do not exist. Further research is needed.

Lung injury following thoracic aortic occlusion: comparison of sevoflurane and propofol anaesthesia.

BACKGROUND: Halogenated anaesthetics have been shown to reduce ischaemia-reperfusion injuries in various organs due to pre- and post-conditioning mechanisms. We compared volatile and total intravenous anaesthesia with regard to their effect on remote pulmonary injury after thoracic aortic occlusion and reperfusion. METHODS: Eighteen pigs were randomized after sternotomy and laparotomy (fentanyl-midazolam anaesthesia) to receive either sevoflurane or propofol in an investigator-blinded fashion. Ninety minutes of thoracic aortic occlusion was induced by a balloon catheter. During reperfusion, a goal-directed resuscitation protocol was performed. After 120 min of reperfusion, the anaesthetic regimen was changed to fentanyl-midazolam again for another 180 min. The oxygenation index and intra-pulmonary shunt fractions were calculated. After 5 h of reperfusion, a bronchoalveolar lavage was performed. The total protein content and lactate dehydrogenase activity were measured in epithelial lining fluid (ELF). Alveolar macrophage oxidative burst was analysed. The wet to dry ratio was calculated and tissue injury was graded using a semi-quantitative score. Ten animals (n=5 for each anaesthetic) without aortic occlusion served as time controls. RESULTS: The oxygenation index decreased and the intra-pulmonary shunt fraction increased significantly in both occlusion groups. There were no significant differences between sevoflurane and propofol with respect to the oxygenation index, ELF composition, morphologic lung damage, wet to dry ratio and alveolar macrophage burst activity. Differences were, however, seen in terms of systemic haemodynamic stability, where catecholamine requirements were less pronounced with sevoflurane. CONCLUSION: We conclude that the severity of remote lung injury was not different between sevoflurane and propofol anaesthesia in this porcine model of severe lower-body ischaemia and reperfusion injury.

Effects of sevoflurane and propofol on ischaemia-reperfusion injury after thoracic-aortic occlusion in pigs.

BACKGROUND: Thoraco-abdominal-aneurysm surgery predicts high mortality. Propofol and sevoflurane are commonly used anaesthetics for this procedure. Halogenated anaesthetics induce organ protection similar to ischaemic preconditioning. We investigated which anaesthetic regimen would lead to a better protection against ischaemia-reperfusion injury induced by temporary thoracic-aortic occlusion. METHODS: Following initial fentanyl-midazolam anaesthesia for surgical preparation, 18 pigs were randomly assigned to two groups: group one received propofol (n=9) and group two sevoflurane (n=9) before, during, and after lower body ischaemia in an investigator blinded fashion. Ten animals without aortic occlusion served as time controls (propofol, n=5; sevoflurane, n=5). For induction of ischaemia, the thoracic aorta was occluded by a balloon-catheter for 90 min. After 120 min of reperfusion, the study anaesthetics were discontinued and fentanyl-midazolam re-established for an additional 180 min. Goal-directed therapy was performed during reperfusion. Fluid and catecholamine requirements were assessed. Serum samples and intestinal tissue specimens were obtained. RESULTS: Severe declamping shock occurred in both study groups. While norepinephrine requirements in the sevoflurane group were significantly reduced during reperfusion (P<0.05), allowing cessation of catecholamine support in 4/9 animals, all 9/9 animals were still catecholamine dependent at the end of the experiment in the propofol group. Serum activities of lactate dehydrogenase, aspartate transaminase, and alanine aminotransferase were lower with sevoflurane (P<0.05). Small intestine tissue specimens did not differ histologically. CONCLUSIONS: Use of sevoflurane compared with propofol attenuated the haemodynamic sequelae of reperfusion injury in our model. Release of serum markers of cellular injury was also attenuated.

Transgenic animals in experimental xenotransplantation models: orthotopic heart transplantation in the pig-to-baboon model.

Pig organs are at risk for hyperacute and acute vascular rejection mediated by anti-pig antibodies, mainly binding to the Galalpha(1,3)Gal epitope. Acute cellular rejection is characterized by progressive infiltration of mononuclear cells. There is an ongoing search for immunosuppressive regimens that provide adequate protection against all patterns of xenograft rejection, but have no severe impact on the condition of xenograft recipients. Herein orthotopic heart transplantations were performed from hDAF or hCD46 piglets to nonsplenectomized baboons. Basic immunosuppression consisted of tacrolimus, sirolimus, GAS914, steroids, and ATG. Group 1 received basic immunosuppression. Group 2 was additionally treated with rituximab and group 3 with half-dose cyclophosphamide. Group 4 received cyclophosphamide and an anti-HLA-DR antibody. Three baboons received GAS914 and TPC. Monitoring included the regular assessment of anti-porcine antibodies, blood counts, therapeutic drug monitoring, and graft histology. Two grafts failed due to technical mistakes. In group 1, baboons died after 1 and 9 days. In group 2, maximum survival was 30 hours. In group 3, baboons lived 20 hours, 25 days, and 14 days. Group 4 survival times were 9.5 hours, 5.5 hours, 4 days, 34 hours, and 3 days. An increase of non-Galalpha(1,3)Gal antibodies was observed. Depositions of immunoglobulins and complement revealed a humoral rejection process. No cellular infiltration could be observed. In conclusion, suppressing cellular rejection with half-dose cyclophosphamide together with tacrolimus and sirolimus produced longer graft survival with a good general condition. Prevention of acute xenograft rejection further needs inhibition of non-Galalpha(1,3)Gal cytotoxicity by sufficient depression of B-cell activation.

Vascular ectasia of the whole intestine as a cause of recurrent gastrointestinal bleeding after high-dose chemotherapy.

We present the first case in the literature of vascular ectasia of the whole intestine as a cause of recurrent and profuse gastrointestinal bleeding in a patient with relapsing Hodgkin's disease. The 17-year-old patient experienced early relapse of his Hodgkin's disease after first-line chemotherapy. Salvage chemotherapy was followed by high-dose chemotherapy and autologous stem cell transplantation. Complete remission was achieved after another relapse by means of a second transplant. The patient presented with profuse gastrointestinal bleeding 5 months later, however. Gastric antral vascular ectasia following hematopoietic stem cell transplantation was diagnosed by endoscopy, with histological confirmation. Similar lesions were found in the duodenum, the ileum, and throughout the entire colon. In conclusion, vascular ectasia of the whole intestine should be considered as cause of acute gastrointestinal bleeding after stem cell transplantation. Physicians should be aware of this complication because its onset is typically delayed. Importantly, this disease is not limited to patients who have undergone allogeneic transplantation, but can also occur after autologous transplantation.

Fluorescent microspheres reveal different regional blood flow in hyperacutely rejected nontransgenic and hDAF pig hearts.

Classic features of hyperacute rejection show differential severity in the inner compared to the outer myocardium. In the present study, regional blood flow (RBF) measured by fluorescent microspheres served as a marker of the extent of hyperacute rejection. Using a working heart model, hearts of nontransgenic and hDAF transgenic pigs were perfused with human blood. Additionally, hDAF transgenic pig hearts were perfused with human blood containing GAS914 or the GPIIb/IIIa inhibitor tirofiban. Injections of fluorescent microspheres into the donor heart were performed in situ and during perfusion. Reference arterial blood samples were collected from the inferior aorta and the afterload line. Perfusion was terminated before hyperacutely rejected hearts failed to pump against the afterload column. RBF was determined in tissue samples of standardized areas of the left atrium and ventricle. Each specimen was divided into subepicardial and subendocardial tissue samples. Fluorescence intensity was measured using an automated luminescence spectrometer. At the end of perfusion with human blood, hyperacutely rejected nontransgenic pig hearts showed a higher RBF in the subendocardium. In hDAF-transgenic pig hearts perfused with unmodified human blood the subendocardial/subepicardial blood flow ratio changed in favor of the subepicardium. This ratio was not further improved by GAS914. In contrast, tirofiban was able to assimilate subepicardial and subendocardial blood flow. In conclusion, RBF of hyperacutely rejected pig hearts was inhomogeneous. Inhibition of complement activation improved the reduced subepicardial RBF, but depletion of antibodies had no positive effect. The ability of tirofiban to further increase subepicardial RBF affirms thrombosis of subepicardial veins as the defining characteristic of hyperacute rejection.

Outcome following lung resections for pT1 non-small cell lung cancer.

AIMS: To analyse the outcome of patients with pT1 NSCLC treated at our institution by antero-lateral thoracotomy, anatomical lung resections and mediastinal lymph node dissection between 1980 and 2001. METHODS: Follow-up data were obtained retrospectively from 1980 to 1990 and prospectively after 1990. Survival was analysed using the Kaplan-Meier method. RESULTS: Histopathological examinations revealed mediastinal lymph node infiltration in 27.6% (pN1 17.8% and pN2 9.8%). pN2 was classified in 14.1% of adenocarcinomas compared to 6.2% of squamous cell carcinomas. Median overall survival of patients with pT1 carcinomas was 89+16 months (median+standard error). Histopathological N-classification indicates differential prognostic and therapeutic implications in pT1 adeno- and squamous cell carcinomas. CONCLUSIONS: Complete lymph node dissection is required for all patients with T1 NSCLC treated by either open surgery or VATS resection. Histopathological N-classification indicates differential prognostic and therapeutic implications in pT1 adeno- and squamous cell carcinomas.

The high-grade endometrial sarcoma: a rare entity.

INTRODUCTION: With an estimated incidence of one to two per one million women, the endometrial stromal sarcoma (ESS) is a rare disease. It is subclassified into a high-grade and a prognostically better low-grade type. Evidence-based data for a standardized therapy is lacking. CASE REPORT: A 32-year-old obese nulligravida presented with persistent vaginal bleeding after the operation of an acute adnextorsion at another hospital. The repeat gynecological ultrasound examination showed a 5.3x5.3x3.6 cm vascularized, partially inhomogeneous mass in the uterus. A fractioned curettage yielded a differential diagnosis of malignant muellerian mixed tumor or a non-differentiated endometrial sarcoma. For completion of the operative treatment, laparotomy with hysterectomy, adnexectomy, and pelvine lymphonodectomy were performed. The final histological report described a 7 cm non-differentiated endometrial sarcoma with infiltration of the left ovary and 25 tumor-free lymph nodes. DISCUSSION: Standard therapy for resectable sarcoma is abdominal hysterectomy and bilateral adnexectomy. So far, there is little data from studies reporting radio- or chemotherapy treatment of small patient numbers in an adjuvant setting. CONCLUSION: The ESS is a very rare disease of the uterus. Due to missing clinical data, it remains a multidisciplinary therapeutic challenge requiring individual decisions. To receive more information on this rare disease, treatment should be performed according to international protocols.

Human herpesvirus 6 in bronchalveolar lavage fluid after lung transplantation: a risk factor for bronchiolitis obliterans syndrome?

Bronchiolitis obliterans syndrome (BOS) is the limiting factor to long-term survival after lung transplantation. Previous studies suggested respiratory viral tract infections are associated with the development of BOS. To identify the impact of virus detection in bronchoalveolar lavage (BAL) fluid, we analyzed BAL samples from 87 consecutive lung transplant recipients for human herpesvirus (HHV)-6, Epstein-Barr virus, Herpes simplex virus 1/2, Cytomegalovirus, respiratory syncytical virus and adenovirus by PCR. Acute rejection, BOS and death were recorded for a mean follow-up time of 3.27 +/- 0.47 years. Results of PCR analysis and other potential risk factors were entered into a Cox regression analysis of BOS predictors and death. Only acute rejection was a distinct risk factor for BOS of all stages, death and death from BOS. HHV-6 was detected in 20 patients. Univariate and multivariate analysis revealed that HHV-6 was associated with an increased risk to develop BOS > orb = stage 1 and death, separate from the risk attributable to acute rejection. Identification of HHV-6 DNA in BAL fluid is a potential risk factor for BOS. Our results warrant further studies to elucidate a possible causal link between HHV-6 and BOS.

Combining the hDAF transgene with the GP IIb/IIIa inhibitor tirofiban improves heart performance and reduces myocardial damage following hyperacute rejection in an ex vivo perfusion model.

Xenograft rejection is associated with vascular injury resulting at least in part from platelet activation, and rejected xenografts invariably demonstrate intravascular thrombosis. Assuming that complement activation is a major determinant of humoral immune reactions bringing about platelet-endothelial cell interactions, we tested the effects of the specific platelet glycoprotein IIb/IIIa inhibitor tirofiban in combination with the human decay accelerating factor (hDAF) transgene on hyperacute rejection of pig hearts. Four groups were studied in a working heart-perfusion model. Pig hearts transgenic for hDAF and nontransgenic pig hearts were perfused with human blood containing tirofiban or with unmodified human blood. Cardiac output, stroke work index, and creatine phosphokinases were measured for the evaluation of the extent of myocardial damage. Consumption of complement components was determined. Endothelial deposition of fibrin and intravascular thrombosis were evaluated. Tirofiban improved cardiac output and stroke work index of nontransgenic pig hearts and was able to further increase hemodynamic function of hDAF transgenic pig hearts. Low levels of creatine phosphokinases also revealed a cardioprotective effect of tirofiban. However, a further extension of the survival of hDAF transgenic pig hearts could not be achieved, although tirofiban prolonged beating time of nontransgenic pig hearts. Tirofiban was able to reduce the consumption of complement components independently of hDAF. Intravascular evidence of fibrin and thrombosis tended to be particularly reduced by the combination of tirofiban and hDAF. Thus, the application of tirofiban together with hDAF improves the performance of pig hearts by reducing myocardial damage and intravascular thrombosis.

ANP preconditioning does not increase protection against experimental pancreatitis, observed after general anesthesia and jugular vein catheterization.

It has been widely shown that preconditioning, inducing heat shock proteins, can protect against experimentally induced pancreatitis. Solid evidence indicates that HSP70 plays a central role in this context, possibly by inhibition of premature intracellular trypsinogen activation. Current preconditioning protocols such as whole body hyperthermia are, however, quite strenuous and clinically not applicable. There is little data on other means to induce pancreatic HSPs such as pharmacologic pretreatment.However, in models of ischemic liver reperfusion injury, it has been demonstrated that atrial natriuretic peptide (ANP) can be used for such pharmacologic preconditioning. Evidence indicates that ANP exerts its protective effects via increased cGMP levels, activation of heat shock transcription factor (HSF) and, increased protein levels of HSP70. Pancreatic acinar cells express ANP receptors and respond to ANP treatment with increased cGMP levels. We have, therefore, investigated whether intravenous ANP pretreatment could be used to protect the pancreas against experimental pancreatitis. When given 20 minutes prior to pancreatitis induction, ANP pretreatment had no effect on cerulein-induced pancreatitis. In contrast, 24 hours after preconditioning, induction of HSP70 protein expression and protection against experimental pancreatitis were found. However, controls treated with NaCl without ANP showed a similar response. This indicates that stress caused by general anesthesia and jugular vein catheterization can be sufficient for preconditioning while ANP, in contrast to models of ischemic liver reperfusion injury, does not confer additional protection.

[Pathology of lung transplantation and molecular aspects of pathogenesis]. / Pathologie der Lungentransplantation mit molekularen Aspekten der Pathogenese.

Lung transplantation has become an established therapy for patients with a variety of end stage pulmonary diseases during the last twenty years. Many complications can affect the lung after transplantation, e.g. preservation related injury, mechanical complications of surgery, acute or chronic (obliterative bronchiolitis) rejection, infection, drug toxicity, recurrent disease, de novo disease, lymphoproliferative disorder. The regulation of tissue inflammation and repair mechanisms involving components of the immune systems depends on a number of cell-cell interactions. Today, our understanding of this pathogenetic and functional network is still at the beginning. The numbers of molecules and factors involved are still increasing. Technical progress has introduced a lot of new molecular techniques (e.g. PCR, cDNA Microarray etc.) for analysis of transplant reaction. Nevertheless assessment of lung allografts is usually by transbronchial biopsy and less commonly by thoracoscopic or open lung biopsy. A minimum of five fragments of alveolated lung parenchyma is required. Biopsies are examined by routine haematoxylin and eosin stain, connective tissue or elastic stains and further special stains, immunohistochemistry and molecular techniques will be performed if required. In examining biopsies from lung allografts three critical points to assess are: a. the presence or absence of acute rejection or b. chronic rejection (obliterative bronchiolitis), and c. the identification of infection. Interpretation of the histology requires knowledge of the clinical and microbiological results. New molecular techniques could help to elucidate the pathogenesis of transplant reaction and provide new insights to facilitate the clinical diagnostics of allograft pathology.

[Rare cause of a chest pain]. / Seltene Ursache eines Thoraxschmerzes.

HISTORY AND ADMISSION FINDINGS: A 42-year-old woman was admitted because of a newly perceived pain localized in the ventral part of the fifth left rib. The physical examination was normal except for a palpable nodular thyroid and pain on palpation of this rib. The patient history contained a serious car accident and severe cigarette abuse. DIAGNOSTIC PROCEDURES: The chest x-ray in "bone technique" showed an osteolysis in the fifth left rib, which was confirmed by computer tomography and bone scan. The high resolution CT scan of the lung revealed a discrete interstitial reticular pattern with minor cystic alterations without lymph node enlargement. DIAGNOSIS, THERAPY AND CLINICAL COURSE: Transbronchial biopsy, bronchoalveolar lavage and a malignancy screening did not lead to a diagnosis. Therefore, the patient was submitted to partial rib resection and open lung biopsy. Histological examination revealed a Langerhans cell histiocytosis. The initial therapeutic approach was a strict smoking cessation. CONCLUSION: The differential diagnosis of a lytic bone lesion in a heavy smoker should include Langerhans cell histiocytosis. On smoking cessation a remission of the disease may be achieved.