Annular vasculitis in association with sarcoidosis.

We report a case of cutaneous vasculitis with annular features in association with sarcoidosis. A 36-year-old woman presented with fever, polyarthralgias, erythema nodosum, bilateral hilar lymphadenopathy, and induration of a long-standing scar on the face. In addition, she developed annular, erythematous, and purpuric patches over her thighs and buttocks that were histologically characterized by a small vessel leukocytoclastic vasculitis. The presence of circulating immune complexes in the early stages of sarcoidosis might be related to the occurrence of the vascular damage.     

Amyotrophic lateral sclerosis and neurosarcoidosis: A case report

Amyotrophic lateral sclerosis (ALS) remains a clinical diagnosis without definable biomarkers. The pathomechanism of motor neuron degeneration in ALS has yet to be elucidated. Here we present a case of limb‐onset ALS, with autopsy findings of Bunina bodies and skein‐like inclusions, as well as sarcoid granulomas predominating among motor neurons. The targeting of the motor neurons by the sarcoid inflammation raises questions regarding the role of cellular immunity in the pathomechanisms for ALS. Muscle Nerve, 2009     

大麻素Ⅱ型受体在结节病、环状肉芽肿中的组织分布

目的：研究大麻Ⅱ型受体（CB2）在人正常皮肤及结节病、环状肉芽肿的组织分布。方法：用兔抗人CB2受体抗体做免疫组化,检测人皮肤及结节病、环状肉芽肿的组织CB2受体表达分布情况。结果：CB2受体在人皮肤及结节病、环状肉芽肿均有表达分布,且结节病、环状肉芽肿的CB2受体过度表达。结论：大麻素受体CB2的表达与皮肤结节病、环状肉芽肿关系密切。     

Real-time endobronchial ultrasound-guided transbronchial needle aspiration is useful for diagnosing sarcoidosis

BACKGROUND AND OBJECTIVE: Several studies of real-time endobronchial ultrasound (EBUS)-guided transbronchial needle aspiration (TBNA) have reported a sensitivity of approximately 90% in the diagnosis of mediastinal and hilar malignancies. However, few studies have addressed its role in the diagnosis of sarcoidosis. The aim of the present study was to assess the utility of EBUS-TBNA in confirming a pathological diagnosis of sarcoidosis. METHODS: Fifteen consecutive patients with suspected sarcoidosis and mediastinal and/or hilar lymphadenopathy were investigated prospectively. EBUS-TBNA with an echo-bronchoscope and a dedicated echogenic 22-gauge needle was carried out in patients under conscious sedation, followed by conventional TBNA of the same lesion using a 19-gauge needle. RESULTS: EBUS-TBNA and/or TBNA demonstrated non-caseating epithelioid cell granulomas in 14 of 15 patients (93%). All 14 patients with a pathological diagnosis of sarcoidosis were considered to have sarcoidosis based on subsequent clinical assessments. The single patient with a negative EBUS-TBNA and TBNA had a malignant melanoma diagnosed following surgical biopsy. EBUS-TBNA confirmed a diagnosis of sarcoidosis in 13 of the 14 patients (93%) by identifying non-caseating epithelioid cell granulomas in 18 of 23 lymph nodes (78%) sampled. When two needle aspirates of one or two lymph nodes were carried out, the percentage positive pathological diagnosis for sarcoidosis for (i) EBUS-TBNA; (ii) TBNA; and (iii) the combination of EBUS-TBNA and TBNA were 93% (13 of 14 patients), 93% (13 of 14 patients) and 100% (14 of 14 patients), respectively. There were no complications associated with the procedures. CONCLUSION: EBUS-TBNA is less invasive and acceptably sensitive as a method for obtaining pathological confirmation of sarcoidosis.     

Severely impaired left ventricular function: tissue characterization by cardiovascular magnetic resonance in a clinical dilemma

In patients with symptoms of heart failure, identifying the underlying cause of cardiomyopathy is helpful to establish the diagnosis and to guide therapy. The differential diagnosis of cardiomyopathy can be challenging based on clinical findings. We report the case of a patient who represented a clinical dilemma (cardiac sarcoidosis or ischaemic heart disease), in whom cardiovascular magnetic resonance was a clinically valuable tool to distinguish dual cardiac pathology due to its unique, non-invasive, tissue characterization capabilities.     

World Trade Center "sarcoid-like" granulomatous pulmonary disease in New York City Fire Department rescue workers

BACKGROUND: Previous reports suggest that sarcoidosis occurs with abnormally high frequency in firefighters. We sought to determine whether exposure to World Trade Center (WTC) "dust" during the collapse and rescue/recovery effort increased the incidence of sarcoidosis or "sarcoid-like" granulomatous pulmonary disease (SLGPD). METHODS: During the 5 years after the WTC disaster, enrollees in the Fire Department of New York (FDNY) WTC monitoring and treatment programs who had chest radiograph findings suggestive of sarcoidosis underwent evaluation, including the following: chest CT imaging, pulmonary function, provocative challenge, and biopsy. Annual incidence rates were compared to the 15 years before the WTC disaster. RESULTS: After WTC dust exposure, pathologic evidence consistent with new-onset sarcoidosis was found in 26 patients: all 26 patients had intrathoracic adenopathy, and 6 patients (23%) had extrathoracic disease. Thirteen patients were identified during the first year after WTC dust exposure (incidence rate, 86/100,000), and 13 patients were identified during the next 4 years (average annual incidence rate, 22/100,000; as compared to 15/100,000 during the 15 years before the WTC disaster). Eighteen of 26 patients (69%) had findings consistent with asthma. Eight of 21 patients (38%) agreeing to challenge testing had airway hyperreactivity (AHR), findings not seen in FDNY sarcoidosis patients before the WTC disaster. CONCLUSION: After the WTC disaster, the incidence of sarcoidosis or SLGPD was increased among FDNY rescue workers. This new information about the early onset of WTC-SLGPD and its association with asthma/AHR has important public health consequences for disease prevention, early detection, and treatment following environmental/occupational exposures.     

Sarcoidosis presenting as an osteolytic skull lesion: a case report and review of literature on skull sarcoidosis

Bone sarcoidosis of the skull is an infrequent presentation of sarcoidosis. We describe a 51-year-old man who consulted due to inflammatory-appearing nodulation in the right supraorbital region. Images showed a solitary osteolytic lesion extending to soft tissues with increased scintigraphic uptake. The anatomopathological study revealed the presence of non-caseating epithelioid granulomas, compatible with sarcoidosis. Steroid treatment led to a marked remission of the lesion, without evidence of relapse during a follow-up period of 1 year. The literature dealing with skull sarcoidosis is reviewed.     

Higher Monocyte Expression of TLR2 and TLR4, and Enhanced Pro-inflammatory Synergy of TLR2 with NOD2 Stimulation in Sarcoidosis

Introduction  Sarcoidosis is an inflammatory disease of unknown etiology. However, an infectious cause has been proposed suggesting a role for pattern-recognition receptors, such as Toll-like receptors (TLRs) and nucleotide-binding domain, leucin-rich repeat containing family proteins (NLRs), in the pathogenesis. Objective  Our aim was to investigate whether differences in TLR2 and TLR4 expression, and the response to TLR2, TLR4, and NOD2 stimulation, are associated with sarcoidosis. Materials and Methods  Blood mononuclear cells from sarcoidosis patients (n = 24) and healthy subjects (n = 19) were incubated with the TLR2 ligands PGN and Pam3CSK4, the TLR4 ligand LPS, the NOD2 ligand MDP, or medium alone. After 16 h, monocyte TLR2 and TLR4 expression and cytokine secretion, including TNFα, IL-1β, IL-6, IL-8, IL-10, and IL-12p70, were measured using flow cytometry and cytometric bead array. Results  TLR2 and TLR4 expression at baseline was significantly higher in patients. Combined TLR2 and NOD2 stimulation induced a four-fold higher secretion of TNFα and a 13-fold higher secretion of IL-1β in patients. Additionally, there was a synergistic effect of TLR2 with NOD2 stimulation on induction of IL-1β in patients, whereas IL-10 was synergistically induced in healthy subjects. Conclusion  Increased TLR expression and enhanced secretion of pro-inflammatory cytokines after combined TLR2 and NOD2 stimulation may be related to the pathogenesis of sarcoidosis. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users. This study was supported by the Swedish Heart–Lung Foundation, King Oscar II Jubilee Foundation, the Swedish Research Council, the U.S. National Institutes of Health (Grant No. 1 R21 HL077579-01), the Stockholm County Council and Karolinska Institutet.     

Churg‐Strauss syndrome presenting as scar reactivation: histopathologic features and an illustration of ‘locus minoris resistentiae’

We report a 33‐year‐old female with cutaneous involvement by Churg‐Strauss syndrome confined to surgical scars that were obtained 13 years before. She presented to the emergency department with 2‐day history of fever, night sweats, right‐sided weakness, hoarseness and worsening asthma symptoms. She was found to have an eosinophilia and two sub‐5‐mm pulmonary nodules. The patient also reported that the scars on her right thumb, inner wrist and back had been swollen, red and painful for 2 days. Examination revealed tender, erythematous, well‐healed edematous scars studded with small skin colored papules. She had no clinical findings that were classic for cutaneous vasculitis. A skin biopsy of a scar revealed perivascular and palisading granulomatous inflammation consisting of histiocytes and neutrophils with leukocytoclasia. Focal vascular injury was identified. Scattered tissue eosinophils were seen. Special stains were negative for infection. Thereafter, she was started on intravenous steroids, at which point the fever, pulmonary and cutaneous symptoms subsided. Although scar sarcoidosis is a well‐described phenomenon, granulomatous inflammation and vasculitis seen in Churg‐Strauss syndrome exclusively manifesting in well‐healed surgical scars highlights the unique features seen in this case and draws attention to the concept of locus minoris resistentiae. This case also highlights how a skin biopsy in the setting of suspected systemic vasculitis can confirm the presence of vasculitis and/or granulomatous inflammation and obviate the need for more invasive, higher risk procedures such as lung biopsy.     

Orbital Sarcoidosis Presenting as Diffuse Swelling of the Lower Eyelid

The author reports a case of orbital Sarcoidosis in a 70-year-old female that initially presented as diffuse swelling of the lower eyelid. The patient complained of painless swelling of the left lower lid without palpable mass, and a computerized tomography (CT) scan of the orbit was unremarkable. A serum angiotensin converting enzyme level was elevated, and hilar lymphadenopathy was noted on the chest CT. The patient underwent surgical debulking for histologic confirmation, which led to a final diagnosis of sarcoidosis involving the orbital fat. Unexplained chronic eyelid swelling without a mass should be considered a possible ophthalmic manifestation of orbital sarcoidosis.