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Sarcoidosis is a multisystemic granulomatous disease characterized by the presence of noncaseating granulomas, the exact etiology of which is yet to be determined. Most of patients show granulomas located in the lungs or in the related lymph nodes. However, lesions can affect any organ. Noncaseating granulomas are not a pathognomonic sign of sarcoidosis, being observed also in other diseases, therefore the diagnosis is often of exclusion. We report a case of sarcoidosis with parotid gland involvement in the context of a Heerfordt syndrome, discussing about its clinical presentation, pathogenesis, pathology and differential diagnosis with other granulomatous diseases.

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Heerfordt's syndrome is a rare manifestation of sarcoidosis and is defined as a combination of facial palsy, parotid swelling, and uveitis, associated with a low-grade fever. We report a case of Heerfordt's syndrome presenting with a high fever and increased serum tumor necrosis factor alpha (TNF-α) levels. The patient had facial palsy, parotid swelling, uveitis, and swelling of the right supraclavicular and hilar lymph nodes. Corticosteroid therapy was initiated, and her symptoms soon resolved completely, in tandem with a decrease in TNF-α serum levels.

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We report a case of complete Heerfordt syndrome accompanied by the involvement of small fiber neuropathy (SFN) manifesting as refracory facial pain. A 30-year-old man presented with pyrexia, a 2-week history of facial burning pain, and difficulty of mastication. After admission to our hospital, neurological examinations showed bilateral facial pain, trigeminal motor palsy, left facial nerve palsy, bilateral sensory neural deafness, uveitis and swelling of the parotid gland. Other examinations revealed bilateral hilar lymphadenopathy, high serum titer of angiotensin coenzyme, and no response in a tuberculin-tested, non-caseating epithelioid granuloma from lip biopsy, leading to the diagnosis of complete Heerfordt syndrome. Mandibular skin biopsy with immunostaining for PGP 9.5 showed SFN. High-dose corticosteroids proved somewhat effective against SFN as facial pain, but reducing the corticosteroid dose proved difficult, as symptoms were refractory to other immunosuppressants and pain-control drugs such as anti-epileptics and anti-depressants. The patient died of acute pancreatitis 3 years after disease onset. Autopsy showed no granuloma in hilar lymph node, trigeminal nerve, cranial base, nerve root, and muscle. SFN in this case probably represent a cause of refractory facial pain.

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To determine if complement anaphylatoxin-mediated inflammation contributes to the development and progression of experimental autoimmune uveitis (EAU), we induced disease in wild type and complement anaphylatoxin receptor-deficient mice (C3a receptor(-/-), C5a receptor(-/-) and C3aR(-/-)/C5aR(-/-)) and evaluated the eyes three weeks post-induction. No differences in disease severity or in disease incidence were seen between wild type controls and anaphylatoxin receptor-deficient mice. Our data indicate that C3a and C5a-mediated functions are not critical to the development of EAU.

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BACKGROUND & OBJECTIVES: Growing body of literature on sarcoidosis in India has led to an increased awareness of the disease. With the advent of better imaging tools hitherto under-recognized manifestations of sarcoidosis are likely to be better recognized. We sought to study the rare clinical and radiological manifestations (<5%) in patients with sarcoidosis. METHODS: Retrospective review of records of 164 patients with histopathologically proven sarcoidosis seen over six years in a tertiary care centre in north India, was done. RESULTS: Fifty four rare manifestations were observed in 164 patients. Acute presentation in the form of Lofgren syndrome was seen in eight (4.9%) and Heerfordt's syndrome in two (1.2%) patients. Musculoskeletal manifestations included chronic sarcoid arthritis in three (1.8%), deforming arthritis and bone erosion in one (0.6%) each. Rare initial presentation with dilated cardiomyopathy in one (0.6%), complete heart block in two (1.2%), bilateral sequential facial nerve palsy in two (1.2%), and pyrexia of unknown origin was seen in one (0.6%) patient. Other rare manifestations included chronic respiratory failure in one (0.6%), dysphagia in one (0.6%), sicca syndrome in five (3%), massive splenomegaly in one (0.6%), portal hypertension in two (1.2%), hypersplenism, gastric sarcoidosis, ninth and tenth cranial nerve palsies, moderate pericardial effusion and nephrocalcinosis in one (0.6%) each, and pulmonary artery hypertension in two (1.2%) patients. Rare radiological manifestations included moderate pleural effusion in two (1.2%), pleural thickening in five (3%), calcification of intrathoracic lymph nodes in four (2.4%), alveolar (nodular) sarcoidosis in three (1.8%), and myocardial uptake of 18F-fluorodeoxyglucose (F-18 FDG) in two (1.2%) patients. Fourteen patients had airways obstruction and behaved typically like seasonal bronchial asthma with excellent response to corticosteroids. INTERPRETATION & CONCLUSIONS: Increased awareness of rare manifestations will facilitate better management of these patients. With increasing use of modern diagnostic tools, manifestations hitherto considered rare, are likely to be recognized more frequently in the future.

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Sarcoidosis is a multi-system disease of unknown aetiology characterised by the presence of non-caseating granulomas, the lungs and lymph nodes being the most affected sites. Orofacial manifestations of the condition are increasingly recognised, with several recent case reports where the initial presentation of the disease is in the region. Here, we report six cases of orofacial sarcoidosis which help to illustrate the wide spectrum of the condition.

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Sarcoidosis is a chronic, multisystem disorder of unknown cause characterized by noncaseating epithelioid granulomas. Infectious agents and genetic components have been discussed. Heerfordt's syndrome with uveitis, enlargement of the parotid glands and optional paralysis of the Nn. facialis is a form of sarcoidosis. The diagnosis is confirmed by histology. Further analyses are fiberoptic bronchoscopy with bronchoalveolar lavage or the gallium-67 scan in combination with blood findings. The therapy of choice is glucocorticoids. We present a case report of Heerfordt's syndrome and a review of the literature covering different aspects of sarcoidosis.

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Sarcoidosis, a systemic granulomatous disease of unknown cause, may involve any body organ. The most common manifestation in the head-neck area is lymph node sarcoidosis or, alternatively, Heerfordt's syndrome (with involvement of lacrimal and parotid gland and possible facial palsy). Far less common is a isolated laryngeal sarcoidosis. The typical histopathological appearance proves the diagnosis. However, obtaining representative and sufficient biopsy material in some cases may carry risks for the patients or might technically be difficult. In these cases, the clinician can fall back on two other, noninvasive diagnostic procedures: ACE level activity determination in blood serum and 67gallium citrate scintigraphy. Elevated ACE levels in combination with a positive 67gallium scintigraphy have a specificity (combined) of 99%. As a valuable side effect, this procedure sensitively may discover additional disease in the body and also serves well as a therapy-control examination. The presented case report shows clearly that these clinical methods, if combined, are sufficient for diagnosis of Heerfordt's syndrome/sarcoidosis; no additional histopathological examination is required.

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Three cases of necrotizing sarcoid granulomatosis (NSG) are reported. All 3 patients had pulmonary lesions; one of them had additional extrapulmonary lesions which were ophthalmologic (dacryoadenitis) and digestive (ulcerative colitis). This patient was followed for 5 years and developed several respiratory, ophthalmologic and digestive recurrences. In 2 cases the diagnosis of NSG had been initially overlooked and the authors emphasize the difficulties of this histologic diagnosis in terms of the differential diagnosis with other necrotic and granulomatous pulmonary diseases such as tuberculosis and Wegener's granulomatosis. They stress the possibility of extrapulmonary lesions in NSG and discuss the relationship between NSG and sarcoidosis.

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