Systemic lupus erythematosus associated with sarcoidosis: Case report

BackgroundThe association between systemic lupus erythematosus (SLE) and sarcoidosis has been considered as extremely rare. Most often, sarcoidosis coexists with Sjögren's syndrome. Researching the literature, it seems that the association of SLE and sarcoidosis is much more frequent than previously thought.Aim of the workWe present a case of a Serbian woman who was diagnosed with coexisting sarcoidosis and long-standing SLE.Case presentationThe 40 years old SLE patient was in long-standing remission on oral prednisolone (10 mg/day) and hydroxychloroquine (HCQ) (400 mg/day). She presented with fatigue, chest pain, and dry cough. Chest computerized tomography (CT) showed hilar and mediastinal lymphadenopathy. The biopsy had been performed and results showed sarcoidosis. Diagnosis has been confirmed: eosinophilic granulomas without central necrosis morphologically corresponding to chronic granulomatous lymphadenitis like sarcoidosis. Thereafter, the patient was hospitalized. No new symptoms appeared, and the physical examination was unremarkable. Serum calcium was elevated (2.75 mmol/l), anti-nuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-dsDNA) and anti-Ro antibodies were positive. Angiotensin converting enzyme (ACE) level was high normal (51 IU/L) and QuantiFERON-TB Gold test negative. The dose of prednisolone was increased to 40 mg/day with HCQ and the patient was discharged from hospital. On follow-up the patient improved with reduction of the steroid dose.ConclusionThe association of SLE and sarcoidosis should be considered even though it is reported to be extremely rare. May be the real incidence of these combinations is underestimated. More research about genetics and pathogenesis is needed to completely understand these conditions.     

结节病肺部改变的CT征象分析

目的探讨结节病肺部改变的CT表现及特征。方法回顾性分析90例经手术病理证实的结节病的临床资料及CT表现。结果结节69例（76．7％），主要沿支气管血管束分布37例（41．1％），团块影31例（34．4％），磨玻璃影39例（43．3％），支气管血管束增粗30例（33．3％），小叶间隔线58例（64．4％），纤维化17例（18．9％），包括支气管变形8例（8．9％）、条索影5例（5．6％）、蜂窝影4例（4．4％），空气潴留3例（3．3％），支气管狭窄8例（8．9％），胸膜改变42例（46．7％），肺门纵隔淋巴结增大76例（84．4％）。2种及2种以上肺部病变并存83例（92．2％），肺部病变合并肺门纵隔淋巴结增大76例（84．4％）。结节、团块、磨玻璃影、支气管血管束增粗治疗后随访吸收好转例数分别为25例（25／30）、9例（9／15）、11例（11／16），10例（10／12）；小叶间隔线、支气管变形、条索影、蜂窝影吸收好转例数分别为10例（10／22）、0例（0／4）、1例（1／3），0例（0／2）。结论结节病肺部CT表现形式多样，具有一定特征性，同时结合肺门纵隔淋巴结增大改变，有助于提高诊断正确率。     

Spinal cord sarcoidosis

We present a case of spinal cord sarcoidosis which resembled a disseminated intramedullary tumour. The case is unusual because the spinal cord is only rarely affected by sarcoidosis and the patient developed a neurological deficit as the first manifestation of the disease. This condition thus has to be considered in the differential diagnosis of primary intramedullary tumours, or metastatic disease with involvement of the spinal cord.     

少见肺部原发性恶性肿瘤七例报告

报告我院１９８０年－１９９３年诊治的７例少见肺部原发性恶性肿瘤，包括来源于间叶组织的２种肉瘤，来源于间胚叶软组织肿瘤，来源于气管粘膜腺体上皮的肿瘤，１例由原始生细胞向胚外组织分化而成的内胚窦瘤，全部病例经病理活组织检查证实，重点讨论诊断的体会。     

Nodular sarcoidosis of the liver and spleen: Appearance on MR images

Small nodular lesions in the liver and spleen have been reported as an infrequent manifestation of sarcoidosis. Five patients with this appearance on either dynamic contrast material—enhanced computed tomographic (CT) or ultrasound scans underwent magnetic resonance (MR) imaging with and without dynamic gadolinium enhancement. The lesions were relatively uniform in size, ranging from 0.5 to 1.5 cm. On CT scans, they were hypoattenuating relative to surrounding parenchyma. On MR images, the lesions were hypointense relative to background parenchyma with all sequences. No substantial enhancement was observed in the lesions, although lesion conspicuity decreased over time on serial postcontrast images. Lesion conspicuity was greatest on either T2-weighted fat-suppressed (T2FS) images or early-phase dynamic contrast-enhanced images. Abdominal adenopathy was seen in three of the five patients and was hyperintense relative to liver on T2FS images in two and intermediate in intensity in one patient.     

结节病5例的诊断

本文报告了5例结节病的临床、X线、CT表现,追踪了疾病的转归,重点描述了重型系统性结节病,提醒临床医师予以重视。     

Diagnostic relevance of Langerin detection in cells from bronchoalveolar lavage of patients with pulmonary Langerhans cell histiocytosis,sarcoidosis and idiopathic pulmonary fibrosis

The diagnosis of pulmonary Langerhans cell histiocytosis might be refined by demonstrating reliability of a new cell marker, i.e., Langerin (CD207), used on bronchoalveolar lavage fluid. For this purpose, we collected material from patients with this disease and also with sarcoidosis and idiopathic pulmonary fibrosis as controls. In addition to the immunocytochemical detection of Langerin, we examined the expression profiles of CD1a and the macrophage tandem-repeat mannose receptor (CD206). To test accessibility of Langerin, a C-type lectin, for mannosides, we employed reverse lectin histochemistry using mannose-containing neoglycoproteins. The analysis revealed a significantly increased percentage of CD1a- and Langerin-positive cells in pulmonary Langerhans cell histiocytosis in comparison with both other studied diseases. No expression of the 175-kDa mannose-binding lectin (CD206) in Langerhans cells was observed. Evidently, binding sites on the cells were not accessible for the mannose-containing neoglycoligand. These results provide evidence for the usefulness of Langerin-directed immuno- and glycohistochemical monitoring of bronchoalveolar lavage fluid in the diagnosis of pulmonary Langerhans cell histiocytosis.     

Immunolocalization of extracellular matrix proteins and integrins in sarcoid lymph nodes

 To improve our understanding of the role of extracellular matrix (ECM) proteins and integrins during the processes of granuloma formation in sarcoidosis, we examined the distribution of ECM proteins and the expression of integrins in sarcoid lymph nodes by immunohistochemical methods. We also examined the expression of transforming growth factor-β1 (TGF-β1), which is one of major regulators for synthesis of ECM proteins. Most ECM proteins were detected in the periphery of the granulomas in a concentric pattern, and fibronectin was diffusely detected from an early to a regressive stage. Compared with normal lymph nodes, most β1-integrin subfamilies (α1, α4, α5 and α6) were more strongly expressed on lymphocytes around the granulomas. Epithelioid cells exhibited strong expression of the α5 molecule. Fibroblasts exhibited the expression of the α2 and α5 molecules surrounding ECM proteins. The α5β1 molecule had a distribution similar to that of fibronectin. TGF-β1 was detected in epithelioid cells throughout the various evolutional stages and its expression was especially marked in mature granulomas. Interaction of fibronectin and the α5β1 molecule may have an important role in the process of formation of sarcoid granuloma. The expression of TGF-β1 may be involved in the regression of sarcoid granuloma by initiating fibrosis and atrophy of epithelioid cells. Received: 2 December 1997 / Accepted: 19 February 1998     

Pulmonary and mediastinal "sarcoidosis" following surgical resection of cancer

Previous reports indicate that enlarged hilar and mediastinal lymph nodes caused by sarcoid-like reactions may develop after curative resection of cancer, and their presence does not necessarily denote neoplastic recurrence. Reports further suggest that coexisting pulmonary infiltrates in this setting may be related to sarcoidosis. In this study, we describe two patients who had resected lung and gastric cancer and who later developed pulmonary interstitial infiltrate, concurrent with progressive mediastinal lymphadenopathy initially thought to be caused by intrathoracic dissemination of their cancer. These changes were shown by open lung biopsy to be a benign, granulomatous reaction interpreted as sarcoidosis. Thus, it is important to recognize this clinical pattern when pulmonary infiltrates develop after complete treatment of cancer in an otherwise relapse-free patient and to encourage lung or lymph node biopsy in these particular settings in order to confirm a sarcoid-like reaction, thereby avoiding unnecessary chemotherapy for presumed tumor recurrence.