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Manifestations of sarcoidosis are variable and non-specific. We are reporting on the case of a 23-year-old man who presented multiple bilateral pulmonary nodules spiculated with peripherical micronodules (galaxy sign). He had no clinical symptom. Investigations led to the diagnosis of sarcoidosis. The galaxy sign is a rare manifestation of sarcoidosis which can be useful for diagnosis.

Sarcoidosis is a systemic granulomatous disease that can reduce life expectancy mainly due to pulmonary fibrosis resulting from granulomatous inflammation The lack of vascularization within pulmonary granulomas suggests that macrophages localized in the center of these structures are hypoxic. Hypoxia signaling pathways are known to be pro-inflammatory and pro-fibrotic in various pathological conditions. Recent data suggest an involvement of the transcription factor hypoxia-inducible factor (HIF) in the pathogenesis of sarcoidosis. This could represent a new research approach for the understanding and therapeutic management of sarcoidosis.

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive procedure designed to explore mediastinal lymphadenopathy. Its use and indications have increased recently and severe, though rare, complications have been reported.EBUS-TBNA was performed in a 64-year-old patient presenting with mediastinal lymphadenopathy, probably due to sarcoidosis, but without histological proof. Within hours of the aspiration of subcarinal lymph nodes (station 7), the patient developed fever and dry cough associated with progressive dysphagia and dysphonia that persisted for four weeks. Mediastinitis was diagnosed after a CT-scan revealed a collection in the subcarinal space previously tapped using CT guidance. Intravenous antibiotics were started and both symptoms and the mediastinal collection resolved without need of a surgical procedure. The patient recovered fully.EBUS-TBNA is associated with a risk of mediastinitis that may manifest as an isolated fever arising within hours of the procedure. The pathogens responsible are usually contaminants from the oropharynx such as Streptococcus sp, probably inoculated directly into the mediastinum during transbronchial needle aspiration. Rapid diagnosis and treatment are necessary in order to reduce morbidity and mortality associated with mediastinitis.

Endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA) has undergone a large increase in France since 2007. The aim is to study the evolution of the indications for EBUS-TBNA in our region during the period 2008-2013.We conducted a retrospective observational study including all the patients who underwent an EBUS-TBNA procedure in Picardie from 2008 to 2013. The respective proportion for each indication was noted.During the study period, 1036 EBUS-TBNA procedures were performed with a continuous increase in number (86 in 2008 versus 275 in 2013). We observed an increase in the proportion of procedures performed for a suspected diagnosis of sarcoidosis (OR=1.31; IC 95% [1.09-1.58]; P=0.005) and for the simultaneous diagnosis and staging of lung cancer (OR=1.12; IC 95% [1.02-1.24]; P=0.022). For the diagnosis of sarcoidosis, we observed an improvement in the diagnostic yield between the periods [2008-2010] and [2011-2013] (42.9% versus 72.5%).A continuous increase in the number of EBUS-TBNA procedures was observed during the period 2008-2013. It was associated with a modification in practice with an increased proportion of procedures performed for the diagnosis of sarcoidosis.

Cardiopulmonary exercise testing (CPET) is the most comprehensive investigation for understanding the mechanisms responsible for dyspnea in patients with chronic respiratory disease. The two observations presented here illustrate how CPET can contribute to the management of patients with interstitial lung diseases. A 60-year-old woman had been followed for 20 years for non-progressive pulmonary sarcoidosis, untreated for many years. CPET led to the diagnosis of an atrial septal defect. A 76-year-old man was treated for idiopathic pulmonary fibrosis. Before pulmonary rehabilitation, CPET was performed which revealed significant aortic valve stenosis, which had been to that point asymptomatic. In these two observations, CPET determined the presence of an associated disease, distinct from the interstitial lung disease.

Sarcoidosis is a multisystem benign granulomatous disease of unknown etiology. It can sometimes cause diagnostic confusion by presenting in the form of a pseudotumor, thus constituting a trap for the unwary.The authors report two cases of pseudotumor sarcoidosis. In the first, the pseudotumor occurred in the context of multisystem disease in a 48-year-old man. The response to treatment with systemic corticosteroids was complicated by the development of disseminated tuberculosis, which was rapidly fatal. The second case, by contrast, was about a 58-year-old woman and the disease was self-limiting, resolving spontaneously in less than 3 months.Through these two cases, the authors focus on the particularities of the pseudotumor manifestation of pulmonary sarcoidosis. The diagnosis is often difficult. The mismatch between the clinical picture and the extent of radiological lesions should, however, suggest the diagnosis. Histological evidence is needed to eliminate other etiologies including malignant tumors. The condition usually resolves either spontaneously or after treatment with systemic corticosteroids. However, relapses are possible, including on discontinuation of corticosteroid therapy.

Sarcoidosis is a systemic granulomatosis of unknown origin with a high incidence in the Afro-American population. There is no epidemiologic data regarding Afro-Caribbean population. The aim of our study was to evaluate the incidence and epidemiologic data of the disease, on the island of Guadeloupe, (French West Indies; 402 500 inhabitants, 90% Afro-Caribbean people) during the 13-years period between 1/01/1997 and 31/12/2009.We performed a retrospective study including exclusively patients with a diagnosis of sarcoidosis confirmed by histological examination of the involved tissues.One hundred and thirteen patients were enrolled in the study. One hundred and eleven patients (98%) were black Caribbean of African European descent. Eighteen patients (16%) were more than 65 years old. The crude annual incidence over the study period was 2.9 cases per 100,000 inhabitants (IC 95%: [2,4-3,4]). The main localization of the disease was the chest (89%). Radiographic stages were distributed as following: I (31%), II (39%), III (15%), IV (2%).Our study showed a low incidence rate of sarcoidosis on the island of Guadeloupe over the study period with a high rate of old patients.

TNF blockers are widely used to treat inflammatory rheumatic diseases and also in the treatment of extrapulmonary sarcoidosis. TNFα plays a major role in the development and persistence of sarcoid granulomata. However, recent studies have reported the involvement of anti-TNF therapies in the development of granulomatosis associated with the clinical and radiological features of sarcoidosis.A 54-years-old man with ankylosing spondylitis was treated with etanercept for two years. He was admitted with symptoms of bronchitis associated with radiological evidence of bilateral pulmonary nodules and a right upper lobe infiltrate. Anti-TNF therapy was stopped even though the patient had received 3 months of prophylactic treatment with rifampicin and isoniazid before starting etanercept. Bronchoalveolar lavage excluded infection, particularly tuberculosis. The chest CT-scan showed bilateral pulmonary nodules with peribronchovascular micronodules and enlarged mediastinal lymph nodes. Surgical lung biopsy was performed and revealed non-caseating granulomata. All the data were consistent with a diagnosis of pulmonary sarcoidosis. The patient remained symptomatic despite discontinuation of etanercept for ten months. Corticosteroids were then introduced, leading to a clinical, functional and radiological improvement.This case report underlines the importance of studying the pulmonary complications of TNF blockers. The first priority is to exclude tuberculosis but a diagnosis of sarcoid-like granulomatosis has to be considered. Twenty-three cases have been described in the literature to date.

Familial features of sarcoidosis and observations in monozygotic twins affected by the condition suggest the presence of a genetic predisposition. Various genetic associations have been described with genes coding for proteins involved in immune regulation in particular at the level of interaction between T-lymphocyte and antigen presenting cell. We review the various genetic targets described with techniques ranging from classic human lymphocyte antigen genotype to genome wide linkage scans. The 6p21 region has been highlighted, which includes relevant genes such as MHC class II, BTNL2 and TNFα. These studies show that the genetics of sarcoidosis are complex, that patient sub-groups exist, which may explain some of the heterogeneity in the results of genetic studies and that the interactions between genetic and environmental factors remains to be elucidated.

The management of chronic forms of sarcoidosis can be a difficult therapeutic problem. The purpose of this observational study was to analyze the effectiveness and tolerance of infliximab in chronic sarcoidosis. This multicentre retrospective study involved 31 cases of chronic, systemic, and/or pulmonary sarcoidosis treated by infliximab. Disease had been present for 9 years and involved a mean of four organs. Patients had received several immunosuppressive drugs and 30/31 were treated with corticosteroids (19 ± 16 mg prednisone/day) with the addition in 17 cases, of one or more other immunosuppressive agents. The duration of infliximab therapy was 13 ± 12 months. A beneficial response to infliximab was observed in 62% of the cases across all organs involved: 65% for lung involvement, 67% for skin lesions and 50% for central nervous system lesions. For other organs, responses were disparate. The corticosteroid sparing effect was small (2.8 ± 9.7 mg/day). Effectiveness was more frequent in patients who were treated with additional immunosuppressive agents. Thirteen (41.9%) patients developed side effects; in seven out of 13, side effects were severe, sometimes requiring infliximab to be stopped. Our study supports the continuing interest in the use of infliximab for the treatment of chronic sarcoidosis, but also highlights the frequency and severity of side effects. Indications are difficult to specify, and currently, its use should be restricted to clinical trials.

In a patient with basal alveolar shadowing the diagnosis of exogenous lipoid pneumonia (ELP) requires a past history of chronic ingestion of liquid paraffin and the presence of numerous macrophages containing oil droplets in the bronchial lavage (BL). Additional radiological abnormalities suggest an associated disease, notably infection or cancer, as has been described in the literature.We report the case of a 50 year old woman presenting with alveolar shadowing in the left lung associated with ipsilateral mediastinal nodes and a pleural effusion in addition to two hepatic nodules. As the diagnosis of ELP did not explain the radiological features a thoracotomy and liver biopsies were performed. Histological examination of the hepatic, pulmonary and lymph node biopsies excluded cancer and mycobacterial disease and showed a florid granulomatous foreign body reaction associated with pulmonary and hepatic sarcoidosis. After a 3 month course of oral corticosteroids the mediastinal lymphadenopathy, pleural effusion and hepatic nodules resolved. The patient has maintained her recovery without further treatment for 4 years.The final diagnosis was ELP and systemic sarcoidosis with nodular hepatic involvement.

In pulmonary sarcoidosis, vascular involvement is usually limited to the small and medium-sized vessels. Enlarged hilar lymph nodes are usually considered to be soft and hence unlikely to cause pressure on adjacent bronchi or blood vessels.We report a rare symptomatic compression of a major pulmonary artery by lymphadenopathy in a case of sarcoidosis. Our patient presented with exertional dyspnoea, with enlarged hilar lymph nodes responsible for segmental pulmonary arterial hypertension, without other thoracic abnormalities. The symptoms and lymphadenopathy regressed with corticosteroid therapy.Enlarged hilar lymph nodes can compress major pulmonary arteries and cause segmental pulmonary hypertension. The prognosis can be grave in the absence of treatment.

Renal complications of sarcoidosis are rare but they may lead to renal failure. The two most common mechanisms are interstitial nephritis and acute hypercalcaemic renal failure. We report the case of a woman who presented both of these complications.

Sarcoïdosis is a benign systemic granulomatosis whose aetiology remains unknown. Lung is the most frequently involved organ. The pseudoalveolar form of this disease is known to have an acute onset and is quite uncommon. Therefore, diagnosing such a rare variety of sarcoidosis is rather often challenging.In the present article, the authors report two cases of pseudoalveolar sarcoidosis. The patients, both young adults, showed no suggestive signs of sarcoidosis at first presentation. This resulted in a considerable delay to diagnosis and to the corticosteroid therapy.The authors emphasize the rarity of the pseudoalveolar form of sarcoidosis. They insist on its roentgenographic characteristics and demonstrate the functional benefits allowed by the precocious medical management. They also propose a current review of the literature.

In spite of CD4+ T-lymphocytopenia and corticosteroids-induced immune suppression, the risk of opportunistic infection is not usually considered to be increased in sarcoidosis.We describe 5 cases of opportunistic infection in patients with sarcoidosis and CD4+ T- lymphocytopenia. A systematic review of the literature was done.We describe 2 cases of chronic necroziting aspergillosis, one case of Mycobacterium avium complex pneumonia, one case of pneumocystis pneumonia, and one case of cryptoccocal meningitidis in five patients with sarcoidosis. Four patients were receiving corticosteroids at time of diagnosis. Four patients had CD4+ T-lymphocytopenia. In the literature, we documented 65 cases reports of sarcoidosis complicated by opportunistic infection. At the time of infection diagnosis, 36 patients were receiving corticosteroids. CD4+ T-lymphocytopenia was present in 5 of 11 reported cases. Cryptococcosis was the most common reported infection.Opportunistic infectious complications are rare in patients with sarcoidosis. Opportunistic infections mainly occur in patients receiving corticosteroids, and with CD4+ T-lymphocytopenia. Except for cryptococcosis, sarcoidosis by itself does not appear to be a risk factor of opportunistic infection.

Sarcoidosis is a diffuse granulomatous inflammatory disorder of unknown aetiology. Involvement of the nasal sinuses has been reported only rarely.This multicentre prospective study was undertaken on patients suffering from histologically confirmed sarcoidosis between October 2001 and August 2002. It comprised a questionnaire for nasal symptoms, a nasal endoscopy and a CT scan of the nasal sinuses.Among the 62 patients enrolled 38 reported at least one nasal sinus symptom. An abnormality of the CT scan was present in 70% of cases. None were specific for sarcoidosis. Rhinoscopy revealed a mucosal lesion in 11 cases. The presence of mucosal nodules on the turbinates was suggestive of sarcoidosis and was proven histologically in 4 cases (6.5%). In these 4 cases there were symptoms of nasal sinus involvement and extensive CT changes.Involvement of the nasal sinuses is rare in the course of sarcoidosis. Anterior rhinoscopy allows diagnosis before the development of typical lesions and also the taking of guided biopsies.

The case for endobronchial biopsy in the diagnosis of sarcoidosis: Faced with the continuous development of new diagnostic tests, the clinician needs to be able to critically assess their value and their clinical applicability. Using a practical example, the performance of endobronchial biopsy in diagnosing sarcoidosis, this review provides a guide for critically appraising a study evaluating a diagnostic test. The three main steps described are: 1) assessment of the validity of the results based on the scientific methods used; 2) analysis of the results with reference to sensitivity, specificity, predictive value, likelihood ratio, and precision of the diagnostic test in question; 3) determination of whether the results are applicable to an individual patient or to a specific patient population.

We report 5 cases of sarcoidosis in 4 men and 1 woman who presented multinodular pulmonary lesions. Seldom described (1 to 4% of all cases of sarcoidosis), a multinodular pulmonary presentation is suggestive of metastatic disease. In our patients, the parenchymal opacities were multiple, peripheral and exhibited fuzzy limits, measuring 1 to 7 cm in size and frequently associated with mediastinal adenopathies. The radiographic pattern contrasted with the clinical manifestations (3 of the 5 cases were fortuitous discoveries). Pathological proof required to rule out other disease, especially tumor formation, was acquired; mediastinoscopy allowing the diagnosis in 3 out of 5 cases. The clinical course was favorable in all cases without treatment within 8 +/- 5 months and a mean follow-up of 5 years (range 9 months to 14 years).

Hemoptysis is a rare but often severe event in sarcoidosis. It usually occurs in patients with advanced, fibrotic lung disease. We herein report the case of a 36-year old female patient with type II pulmonary sarcoidosis who presented with abundant hemoptysis very early during the course of her disease. Two attempts to embolize bronchial arteries remained unsuccessful and surgery was eventually required to stop the bleeding. Clinical, microbiological, radiological and pathological data indicate that haemoptysis was caused by systemic hypervascularization around sarcoidosis granuloma.

Ulcerative colitis and sarcoidosis are seldom associated. A 33-year-old diabetic man developed simultaneously a rectitis and a non-productive cough leading to the diagnosis of ulcerative colitis and sarcoidosis. These diagnoses are discussed because of the possible gastrointestinal lesions from sarcoidosis or the respiratory disorders due to ulcerative colitis. Some pathophysiological evidence can be found in this case in favor of a non-fortuitous association.

We report the first description of a Sjögren's syndrome associated with nectrotizing sarcoid-like granulmatosis. A 62-year old woman was admitted for diagnostic exploration of fatigue, weight loss, and fever at 38 degrees C which had progressed for more than 3 months. Chest X-ray showed several pulmonary opacities. There was a history of tuberculosis and thyroiditis at the age of 20 years. Physical examination revealed a sicca syndrome. There was no objective evidence of respiratory disease. Her heart and chest were clear on auscultation. The patient had autoantibodies for SSa and SSb specific antigens. Minor salivary gland biopsy gave a score of 3 in the Chisholm classification. Chest X-ray and CT-scan showed diffuse infiltrative opacities which were dense and peripheral with no retraction. Bronchoalveolar lavage, and bronchial and transbronchial biopsies were non-specific. Surgical lung biopsy showed typical aspects of necrotizing sarcoid-like granulomatosis. After high-dose pulse corticosteroid therapy, the opacities disappeared with no recurrence under oral steroids at three years.

Sarcoidosis is an uncommon cause of secondary amyloidosis. We describe in this paper the case of a 39 years old patient, with a pulmonary and hepatosplenic sarcoidosis. A nephrotic syndrome led to a renal biopsy which showed AA type amyloidosis. As no other cause of amyloidosis has been found we admitted that it was a result of sarcoidosis which was associated with the unusual inflammatory syndrome.