Contribution of Imagery in the Diagnosis of Multisystemic Sarcoidosis in the Service Radiology Department of the Mother and Child Hospital “Le Luxembourg” in Bamako: A Case Report

The aim of this study was to report a case of multi-visceral sarcoidosis at the Mother-Child Hospital Center (CHME) “Le Luxembourg” in Bamako, Mali. Observation: This is a patient aged 62 at the time of consultation, a housewife, residing in the Banconi district, who was referred to us for thoracic-abdominopelvic imaging for chronic liver disease. After several diagnostic errors, the thoracic-abdominopelvic CT scan and liver MRI performed in our center showed, at the thoracoabdominal level, bilateral diffuse pulmonary micronodules and bilateral mediastinal-hilar lymphadenopathy;on the abdominal level, a dysmorphic liver with plaques of steatosis and a granular appearance of the liver parenchyma without periportal fibrosis. These imaging data combined with those from the liver nodule biopsy and biology confirmed the diagnosis of sarcoidosis type II. Treatment with corticosteroids gave satisfactory results and the patient recovered after 18 months. Clinical and CT monitoring 2 years from the start of the disease and 2 months from the end of treatment showed complete resolution of the lesions. Conclusion: The multi-visceral location of sarcoidosis is an entity whose diagnosis remains difficult;diagnostic and interventional imaging has an important place in its management.

Neurosarcoidosis: An Illustrative Case and Review of Perioperative Considerations

Sarcoidosis is a multisystem inflammatory granulomatous disease of unknown etiology. Neurosarcoidosis (NS) is a rare but potentially devastating manifestation of sarcoidosis, with a prevalence of approximately 5% in patients with systemic sarcoidosis. Due to the possible involvement of any part of the nervous system, a myriad of neurological manifestations can occur. Clinical features resulting from involvement of the hypothalmo-pituitary axis and cranial nerves, in particular, cranial nerve VII are the more common presentations of this condition. Medical therapy with corticosteroids is the mainstay of treatment and providing tissue for diagnosis remains the principal indication for surgery. Therapeutically, neurosurgery is indicated only for life-threatening complications. We describe the clinical case of a patient with fatally progressive NS who had multiple anesthetic exposures. This case highlights the perioperative considerations of NS and its anesthetic implications.

The Role of Gallium (Ga-67) Scintigraphy in the Diagnosis of Sarcoidosis

Purpose: To identify and interrogate the role of Gallium-67 scintigraphy in the diagnosis of Sarcoidosis. Methods: A retrospective study of 75 patients with suspected Sarcoidosis in a large teaching hospital with a specialist Sarcoidosis Centre. Results: Ga-67 scintigraphy in patients considered on clinical grounds to have Sarcoidosis is a useful and sensitive test, and furthermore, by relaxing the interpretation of gallium scintigraphy sensitivity may be increased (from 20% to 80%) for a relatively small drop in specificity. Conclusions: The sensitivity of diagnostic power may be increased with a change in diagnostic criteria at the expense of only a small drop in specificity. In the context of Neurosarcoidosis, our results support the theory that Ga-67 scintigraphy has value in identifying potential, asymptomatic biopsy sites outside of the CNS that may not be identified by other forms of imaging.

Exercise-induced torsades de pointes as an unusual presentation of cardiac sarcoidosis: A case report and review of literature

BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis cases,it is still underdiagnosed and is associated with a poor prognosis.Although the etiology of sarcoidosis remains unclear,an antigen triggered exaggerated immune response has been hypothesized.Early detection and prompt management of cardiac sarcoidosis remains pivotal.CASE SUMMARY A 60-year-old female,with pulmonary sarcoidosis in remission,presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion(New York Heart Association class II)that was relieved by rest.Submaximal exercise stress test showed multifocal ventricular extrasystoles,followed by a self-limiting torsades de pointes.Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis.The magnetic resonance imaging-derived left ventricular ejection fraction was 45%.Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles,all in favor of extensive cardiac involvement of sarcoidosis.A double-chamber implantable cardiac defibrillator was implanted,and methylprednisolone(12 mg/d)and methotrexate(12.5 mg/wk)treatment was initiated.Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.CONCLUSION Along an extensive review of the literature,this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis,in order to avoid potential complications and increase survival.

Cranial neuropathies in sarcoidosis

Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoidosis include peripheral and cranial neuropathies, myopathies, seizures, gait dysfunction, and cognitive decline. Because sarcoidosis has a predilection to involve the basilar meninges, cranial neuropathy is the most prevalent neurological deficit seen when the nervous system is involved. Sarcoidosis cranial neuropathy may occur at different stages of the disease and even as the initial clinical manifestation of central nervous system involvement. Attributing a cranial neuropathy to sarcoidosis can be challenging, particularly in the setting of normal imaging studies. In this review, cranial neuropathies in sarcoidosis are discussed in detail.

Gastrointestinal sarcoidosis associated with pneumatosis cystoides intestinalis

A 39-year-old male reported fevers,weight loss,watery loose stools,and decreased visual acuity in his right eye over the prior five years.He was pancytopenic,had an elevated American council on exercise level,total bilirubin,and alkaline phosphatase.Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes.Liver biopsy showed non caseating granulomas.The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone.The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum.He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis.This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis.The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis.Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.

Subcutaneous sarcoidosis of the upper and lower extremities: A case report and review of the literature

BACKGROUND Sarcoidosis is a granulomatous disease of unknown etiology that most often impacts the lungs.Cutaneous manifestations of sarcoidosis are seen among 9%- 37% of patients.Subcutaneous sarcoidosis is a rare presentation of cutaneous sarcoidosis with estimates of frequency ranging from 1.4%-16%.To date,very few articles and case reports have been written about this subject.In this paper,we describe a case of subcutaneous sarcoidosis and perform a review of the literature to determine if there are commonalities among patients who present with subcutaneous sarcoidosis.CASE SUMMARY A 38-year-old female,with a past medical history of arthritis and recurrent nephrolithiasis,presents with an 8-mo history of 4 firm,asymptomatic,skincolored nodules on her left and right upper extremities and neck.Needle biopsy and post-excisional pathology report both revealed well-formed,dense,noncaseating granulomas localized to the subcutaneous tissue.Chest computed tomography revealed mild mediastinal lymphadenopathy.A diagnosis of subcutaneous sarcoidosis was made,and the lesions were surgically removed.CONCLUSION Commonalities among patients presenting with subcutaneous sarcoidosis include: middle-aged female,lesions localizing to the upper or lower limbs,lymphadenopathy or pulmonary infiltration on chest imaging,elevated serum angiotensin-converting enzyme.

Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.

Ultrasound imaging of abdominal sarcoidosis: State of the art

Since it has been recognized that sarcoidosis(SA) is not an exclusive disorder of the lungs but can also affect other organs such as the liver and spleen, efforts have been made to define specific imaging criteria for the diagnosis of the single organ involvement, and the concept has been reinforced that the exclusion of alternative causes is important to achieve the correct diagnosis. Ultrasound(US)is a useful tool to evaluate patients with suspected abdominal SA, such as of the liver, spleen, kidney, pancreas and other organs, showing findings such as organomegaly, focal lesions and lymphadenopathy. While the diagnosis of abdominal SA is more predictable in the case of involvement of other organs(e.g.,lungs), the problem is more complex in the case of isolated abdominal SA. The recent use of contrast-enhanced ultrasound and endoscopic ultrasound elastography has provided additional information about the enhancement patterns and tissue rigidity in abdominal SA. Here we critically review the role of US in abdominal SA, reporting typical findings and limitations of current evidence and by discussing future perspectives of study.

Sarcoidosis imitating breast cancer metastasis:a case report and literature review

Sarcoidosis is a benign systematic granulomatous disorder of unknown etiology and is associated with various malignancies.However, granulomatous and metastatic lymph node lesions are difficult to distinguish even when using precise and modern diagnostic methods, such as positron emission tomography. Thus, histological verification is the only method that can be used to accurately describe the nature of this disease. In this article, we report a case of non-luminal HER-2/neu-positive breast cancer in a patient without history of sarcoidosis and suspected to have metastatic disease.

Ductopenia related liver sarcoidosis

Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis.Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry.The occurrence of jaundice in sarcoidosis is rare;extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic.Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver-related death.We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded.The patient follow up was 8 years.Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis.A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.

Gastric sarcoidosis mimicking irritable bowel syndrome-Cause not association?

Sarcoidosis is a systemic disease of unknown aetiology that may affect any organ in the body. The gastrointestinal tract however is only rarely affected outside the liver. Symptoms may be non-specific. Irritable bowel syndrome （IBS） is a common diagnosis. The recognition of IBS is aided by the use of the Rome Ⅱ criteria-in the absence of organic disease. We describe the first case of a patient with gastric sarcoidosis who presented with IBS symptoms but subsequently responded to immunosuppressive therapy.

Efficacy of additional treatment with azathioprine in a patient with prednisolone-dependent gastric sarcoidosis

Gastric sarcoidosis with noncaseating granuloma is rare. Although corticosteroid produces a dramatic clinical response, it is unknown whether azathioprine show efficacy in prednisolone-dependent cases. Here, we report a case of gastric sarcoidosis in a 25-year-old man with severe epigastlargia. Gastroendoscopy revealed multiple map-like ulcerations. Histological examination showed multiple noncaseating granulomatous lesions in gastric mucosa, which were incompatible with diagnoses of Crohn's disease or tuberculosis. He was started on prednisolone at 30 mg/d, and his symptoms improved within 7-d. The prednisolone was gradually tapered by 5 mg every 2-wk, but oral azathioprine at 50 mg was added after symptoms recurred at tapered dose of 10 mg. Endoscopy 4-wk later showed healing ulcers, and, lymphocytic infiltration was absent. The efficacy of additional azathioprine in gastric sarcoidosis is not well defined. Here, we report a case of prednisolone-dependent gastric sarcoidosis that improved after additional azathioprine, and also review the literature concerning the treatment, especially for prednisolone-dependent cases.

Experiences and benefits of positron emitted tomography-computed tomography (PET-CT) combined with video-assisted thoracoscopic surgery (VATS) in the diagnosis of Stage 1 sarcoidosis

Background： The purpose of this study was to describe our experiences and analyze the benefits of video-assisted thoracoscopic surgery （VATS） combined with positron emitted tomography （PET）-computed tomography （CT） in the diagnosis of patients with early （Stage 1） sarcoidosis. Methods： From 1995 to 2006, seven patients （two males, five females）, with ages ranging from 26 to 58 years, were impressed with Stage 1 sarcoidosis （mediastinal or hilar lymph nodes involvements without lung involvement） by histological examination of intrathoracic lymph nodes （LNs） and/or lung parenchyma taken＇from VATS biopsy. Three of them received PET or PET-CT evaluation. VATS was approached from the right and left side in one and six patients, respectively, according to the locations of their lesions. Results： All the VATS biopsied LNs or lung specimens were adequate for establishing diagnosis. Mediastinal LNs were taken from Groups 3, 4 in four, Group 7 in two, and Groups 5, 6 in one of them. Hilar LNs biopsies were performed in four cases. Lung biopsy was performed in all but two cases. All of them were expressed pathologically or radiologically as Stage 1 sarcoidosis. PET-CT revealed high emission signals over these affected LNs. These patients received oral steroid treatment or follow up only. All of them were followed up from 5 months to 11 years with satisfactory results. Conclusion： VATS biopsy is a minimally invasive, safe and effective procedure. It can be used as a diagnostic altermative of transbronchial lung biopsy （TBLB）, and can harvest larger and more areas of specimens than mediastinoscopy for staging patients with sarcoidosis. PET-CT can provide us more accurate information about the characteristics and localization of these lesions before biopsy. VATS combined with PET-CT can provide more accurate and earlier diagnosis of patients with unknown intrathoracic lesions, including the sarcoidosis.

Spontaneous resolution of systemic sarcoidosis in a patient with chronic hepatitis C without interferon therapy

A 39-year-old male patient complaining of bilateral hand joint arthralgia was evaluated and found to have chronic hepatitis C and systemic sarcoidosis involving lung, skin, liver, and spleen. Hepatic and cutaneous sarcoidoses were confirmed by the presence of numerous noncaseating granulomas on histological examination. Pulmonary and splenic involvements were diagnosed by imaging studies. Fifteen months later, the sarcoidotic lesions in lung, liver, and spleen were resolved by radiological studies and a liver biopsy showed no granuloma but moderate to severe inflammatory activity, systemic sarcoidosis is a rare comorbidity of chronic hepatitis C which may spontaneously resolve.

Historical transition of management of sarcoidosis

Sarcoidosis is a systemic granulomatous disease of undetermined etiology, and it primarily affects the lungs and lymphatic system but may involve other organs. Recently, there have been several new insights in Japanese patients. The frequency of cardiac, ocular, and cutaneous sarcoidosis has increased in Japan, whereas, the proportion of patients with bilateral hilar lymphadenopathy decreased from 1960 to 2004.Propionibacterium acnes(P. acnes) has been studied extensively as one of the causative microorganism for granuloma formation, particularly in Japan. P. acnes-specific monoclonal antibodies are useful for diagnosing sarcoidosis. The potential association between smoking and sarcoidosis has been evaluated in a Japanese study, which found a higher prevalence of sarcoidosis among young smokers than that in previous reports. Recently, 18F-fluorodeoxyglucose positron-emission tomography, which permits visualization of activated inflammation, and endobronchial ultrasonography-guided transbronchial needle aspiration have been increasingly used to diagnose sarcoidosis. Cardiac sarcoidosis is found to be the main cause of death in Japan. The 2006 revised Japanese guidelines for diagnosing cardiac sarcoidosis are useful, particularly for subclinical cardiac sarcoidosis patients. Further studies and international communication and evaluation are needed to determine the causes of sarcoidosis, identify the risk factors for progressive disease, and develop new and effective treatments.

Systemic Sarcoidosis Associated to IgA Nephropathy

The sarcoidosis was rarely associated to IgA nephropathy. We report a 38-year-old man presented decreased visual acuity and xerostomia. He had two axillary lymphadenopathies and pitting edema of legs in physical examination. The ophthalmological examination revealed a right posterior uveitis. Biological investigations showed a mild renal insufficiency and elevated serum level of angiotensin-converting enzyme, β2 microglobulin and IgA. He had a proteinuria and a microscopic hematuria. The kidney echography was without abnormalities. Histological study of the renal biopsy found results in favor to IgA nephropathy. Biopsies performed in accessory salivary gland and lymph nodes revealed non-necrotising epitheloid and gigantocellular granulomatous inflammation suggesting a sarcoidosis. The diagnosis of a sarcoidosis associated to IgA nephropathy was posed. The treatment was based on oral prednisolone with gradual tapering doses. He regained normal vision. The renal function had not worsened. No relapse of sarcoidosis was noted during our follow up.

BORRELIA BURGDORFERI DNA IN BIOLOGICAL SAMPLES FROM PATIENTS WITH SARCOIDOSIS USING THE POLYMERASE CHAIN REACTION TECHNIQUE

Polymerase chain reaction (PCR) was used to detect the presence of Barrelia burgdoferi DNA in biological samples from patients with sarcoidosis. The target DNA sequence was of chromosomal origin. The amplified DNA sequence was analyzed by agarose gel electrophoresis, PAGE with silver staining, and the identity of amplified DNA was confirmed by restriction enzyme cleavage and DNA-DNA hybridization with a 32P-labelled probe. The assay was sensitive to fewer than two copies of B. burgdorferi genome, even in the presence of a 104-fold excess of human eukaryotic DNA , and was also specific to different B. burgdorferi strains tested. Sera serologically positive to B. burgdcrferi (n=26), bronchoalveolar lavage fluid and supernatant of BALF (n=26) and peripheral blood (n=9) from sarcoidosis patients were tested. The positive rate was low (4/26, 2/26. and 0/9, respectively). It was considered that DNA from B. burgdor ferimay be identified in a minority of patients with sarcoidosis, and it may play a pathogenetic role in such cases. More studies need to be done before advancing the hypothesis of an etiologic role of B. burgdorferi in sarcoidosis.

Colonic sarcoidosis:Unusual onset of a systemic disease

Sarcoidosis is a multisystem chronic inflammatory condition of unknown etiology that has the potential to involve every tissue in the body.Sarcoidosis in the gastrointestinal system,and particularly the colon,is very rare.Here,we report the case of a 57-yearold man with no previous diagnosis of sarcoidosis who presented with new onset of abdominal pain and constipation.A colonoscopy revealed that the abdominal pain was caused by an obstructing lesion in the cecum-ascending colon and lacked a clear histologic diagnosis.Radiologic investigation revealed concentric wall thickening of the cecum-ascending colon with multiple satellite lymphadenopathies,highly suggestive of a malignancy.The patient underwent a laparotomy and a right hemicolectomy was performed.A diagnosis of colonic sarcoidosis was made after the resected specimen was examined.Additionally,a chest computed tomography scan revealed lung involvement with atypical radiologic features in the absence of respiratory symptoms.Only histologic examination of the surgical specimen can yield a diagnosis of gastrointestinal sarcoidosis due to the non-specificity of endoscopic and radiologic findings.

A histologically proven case of progressive liver sarcoidosis with variceal rupture

Sarcoidosis is a chronic multi-systemic granulomatous disease,and liver involvement frequently occurs.in most cases,no evidence of liver dysfunction is ob-served,and portal hypertension due to sarcoid liver diseases is a rareoccurrence.Moreover,no case of liver sarcoidosis has ever been reported with confirma-tion of the disease progression.Herein we describe a patient having hepatic sarcoidosis with severe portal hypertension and liver dysfunction.The diagnosis was histologically confirmed from granulomatous status to established liver cirrhosis over 10 years.A 46-year-old woman developed massive hematemesis due to the rupture of gastric cardial varices.She underwent emer-gency endoscopic injection sclerotherapy,and clear evi-dence of chronic hepatic failure.Twelve years ago,she was diagnosed as having sarcoidosis with respiratoryclinicalsymptoms.Liver biopsy revealed asymptomatic incidental granulomas without fibrosis development.After a couple of years,features of liver dysfunction were manifest and progressed.Ten years after the first biopsy,a second liver biopsy was performed,and well established dense fibrosis was revealed.Although significant liver dysfunction with portal hypertension is rarely seen in sarcoidosis,this case indicates that we have to consider the possibility that sarcoidosis may cause end-stage liver cirrhosis.