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Heerfordt's syndrome is a rare subtype of sarcoidosis and features a combination of facial palsy, parotid swelling, and uveitis, associated with a low-grade fever. Cases with two of three symptoms are called "incomplete Heerfordt's syndrome." Heerfordt's syndrome involving other cranial nerve symptoms is relatively rare. We herein report a case of incomplete Heerfordt's syndrome presenting with trigeminal nerve palsy and a reversed halo sign, a rare manifestation of pulmonary sarcoidosis. The histological diagnosis following a biopsy of the parotid gland and endobronchial ultrasound-guided trans-bronchial needle aspiration of the mediastinal lymph nodes was sarcoidosis. The symptoms and lung lesions improved after corticosteroid therapy.

Purpose To describe the ocular manifestations in a cohort of patients with systemic sarcoidosis (SS). Recent advances in the pathophysiology, diagnosis, and therapy of SS are also discussed. Methods Data from 115 Italian patients diagnosed between 2005 and 2016 were retrospectively reviewed. All but the first 17 patients underwent a comprehensive ophthalmologic examination. The diagnosis was based on clinical features, the demonstration of non-caseating granulomas in biopsies from involved organs, and multiple imaging techniques. Data on broncho-alveolar lavage fluid analysis, calcemia, calciuria, serum angiotensin-converting enzyme levels and soluble interleukin-2 receptor levels were retrieved when available. Results Ocular involvement, detected in 33 patients (28.7%), was bilateral in 29 (87.9%) and the presenting feature in 13 (39.4%). Anterior uveitis was diagnosed in 12 patients (36.4%), Löfgren syndrome and uveoparotid fever in one patient each (3%), intermediate uveitis in 3 patients (9.1%), posterior uveitis in 7 (21.2%), and panuveitis in 9 (27.3%). First-line therapy consisted of corticosteroids, administered as eyedrops (10 patients), sub-Tenon’s injections (1 patient), intravitreal implants (9 patients), or systemically (23 patients). Second-line therapy consisted of steroid-sparing immunosuppressants, including methotrexate (10 patients) and azathioprine (10 patients). Based on pathogenetic indications that tumor necrosis factor (TNF)-α is a central mediator of granuloma formation, adalimumab, targeting TNF-α, was employed in 6 patients as a third-line agent for severe/refractory chronic sarcoidosis. Conclusion Uveitis of protean type, onset, duration, and course remains the most frequent ocular manifestation of SS. Diagnostic and therapeutic advancements have remarkably improved the overall visual prognosis. An ophthalmologist should be a constant component in the multidisciplinary approach to the treatment of this often challenging but intriguing disease.

Heerfordt's-Waldenström syndrome is a very rare presentation of neurosarcoidosis characterized by parotid gland enlargement, facial palsy, anterior uveitis and fever. World over only few cases of this syndrome have been reported. We present such a case of Heerfordt-Waldenström syndrome.

Sarcoidosis is a granulomatous disease histologically characterized by non-caseating granulomas. Although it usually affects the lungs, it can affect any organ system and present with a wide variety of symptoms. Heerfordt-Waldenström Syndrome, or uveoparotid fever, is a rare form of sarcoidosis that presents with a combination of fever, parotitis, facial paralysis, and uveitis. In this case report, we demonstrate a rare manifestation of sarcoidosis in a patient who presents with both the aforementioned syndrome and cardiac involvement. This case serves to highlight the importance of identifying the various clinical manifestations and management of systemic sarcoidosis.

Le syndrome de Heerfordt est une manifestation rare de la sarcoïdose associant dans sa forme classique une uvéite, parotidomégalie, paralysie faciale et fièvre. C'est une forme active de la maladie dont le diagnostic est facilité par la biopsie salivaire. Il s'agit d'une observation clinique d'une patiente âgée de 17 ans présentant une uvéite, une parotidomégalie droite et une paralysie faciale droite d'apparition brutale. Après un bilan biologique et une imagerie (échographie parotidienne), une biopsie des glandes salivaires accessoires a conduit au diagnostic de sarcoïdose. Un bilan pré thérapeutique a permis d'instituer une corticothérapie par voie orale avec une évolution favorable et rémission totale. Le syndrome de Heerfordt est une forme clinique rare de sarcoïdose avec un profil évolutif favorable dans la majorité des cas. Il nécessite une approche diagnostique précise (éliminer les diagnostics différentiels surtout les formes incomplètes) en insistant sur les progrès thérapeutiques dans ce domaine.

Uveoparotid fever, also known as Heerfordt-Waldenstrom syndrome, is an uncommon acute presentation of systemic sarcoidosis. Patients may have features of complete/classic or incomplete disease. Early diagnosis and multidisciplinary care should be initiated to prevent sequelae. Herein, the authors report a rare case of retrospectively diagnosed incomplete uveoparotid fever in a patient with anterior uveitis, parotid gland enlargement, and fever who presented to our dermatology clinic with cutaneous sarcoidosis.

I read with great interest the article entitled ‘Sarcoidosis with multiorgan involvement’ in the ‘Images’ section of the August 2020 print issue.1 The authors have detailed the diagnostic dilemma encountered as the patient was initially treated for tuberculosis without any improvement before further work-up eventually pointed to sarcoidosis. This quandary is often seen in world regions where tuberculosis (TB) is endemic and thus, TB often becomes the forefront diagnosis for clinicians. The myriad presentation of sarcoidosis that is shared by many other diseases further adds to the perplexing situation. Particularly, the article has drawn my attention to Heerfordt-Waldenstrom Syndrome (HWS) which is a manifestation of sarcoidosis comprising a clinical tetrad of fever, anterior uveitis, parotid enlargement and facial nerve palsy.2 HWS is regarded as a pathognomonic feature of sarcoidosis. It is noteworthy that the reported patient only exhibited …

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.

Heerfordt-Waldenström syndrome is a rare subacute variant of sarcoidosis, characterized by enlargement of the parotid or salivary glands, facial nerve paralysis and anterior uveitis. Granulomas with a peripheral lymphocyte deficit are found in the anatomic pathology of affected organs. It is normally self-limiting, with cure achieved between 12 and 36 months, but some prolonged cases have been reported. Diagnosis of the syndrome is clinical, and treatment depends on the degree of systemic impairment. Oral corticosteroids represent the first line treatment option. The mortality rate ranges between 1 and 5% of cases.

We report a 39-year-old female admitted for fever. She showed physical findings of bilateral granulomatous uveitis, swelling of the bilateral parotid glands, and paralysis of the left second branch of the trigeminal nerve. Her chest X-ray showed evidence of bilateral hilar lymphadenopathy. We performed biopsy of her parotid gland, and leading to a diagnosis of noncaseating epithelioid granuloma characterized by lymphocyte and multinucleated giant cell invasion. Therefore, she was diagnosed with the abortive type of Heerfordt syndrome which is a subtype of sarcoidosis. This is the only case associated with paralysis of the trigeminal nerve without paralysis of facial nerves to be reported in Japan.

Heerfordt's syndrome (HS) consists in its complete form of uveitis, parotid or salivary gland enlargement and cranial nerve palsy. The objective of the present study was to analyse if there are also links between HLA-DRB1* alleles and HS, as it is a specific phenotype of sarcoidosis. 1,000 patients with sarcoidosis, out of whom 83 had symptoms associated with HS, were included in the study together with a group of 2,000 healthy individuals from the same population, matched for sex and age. HLA-DRB1* allelic groups were determined for all individuals, and comparisons were made between different disease subgroups and between patients and healthy controls. We found that the HLA-DRB1*04 allele was overrepresented in patients with symptoms associated with HS. 83 (8.3%) of all patients had one or more of the symptoms and 46 (55%) of them were HLA-DRB1*04 positive. 44 (55%) of the patients with ocular sarcoidosis, i.e. the most common symptom associated with HS, were HLA-DRB1*04 positive, compared with 35.9% of healthy controls (p=0.0008), and only 26.6% of the whole group of sarcoidosis patients (p

History and clinical findings A 29-year-old patient complained of recurrent swelling of the eyelids and cheeks, fever, diffuse headaches, night sweat and weight loss (8 kg during 8 weeks). Both parotid glands showed swelling which were solid, plain and non-painful to pressure. Palpation did not reveal enlarged lymph nodes. There were no other pathological findings except for anisocoria and disturbed motoric function of the right eye's pupil. Investigations Laboratory findings, chest X-ray, abdominal sonography and ilium crest biopsy showed no signs of lymphoma or other tumour. MRT showed enlarged parotid glands and hypointensity of a few small (up to 2 cm) areas. Because of these findings Heerfordt syndrome with involvement of the third cranial nerve and the lacrimal glands was suspected and verified by parotid biopsy. Treatment and course Under corticosteroid therapy started with 80 mg/d symptoms rapidly ceased. Therapy was continued for one year. 8 months after therapy had been stopped the patient had no complaints and investigations showed no signs of recurrence nor pulmonary or any other organ manifestation. Conclusion If swelling of the parotid glands with night sweat, fever and weight loss occur, parotid biopsy should be performed early to exclude malignancy. With parotid gland biopsy also diagnosis of other diseases which have similar signs, such as Sjogren syndrome and parotid gland sarcoidosis, can be established early.

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.

Sarcoidosis is a granulomatous disease of unclear etiology, which commonly presents with cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum. Heerfordt-Waldenström syndrome, a rare presentation of sarcoidosis, is characterized by the presence of parotid gland enlargement, facial palsy, anterior uveitis, and fever. Here we present a case of a 59-year-old nonsmoking African American woman who presented with 3 days of progressively worsening left facial droop, difficulty swallowing, and blurred vision. Over the prior 4 months, she had had a productive cough, fevers, night sweats, and an unintentional 30-pound weight loss. Physical examination revealed a left facial droop involving the forehead, cheek, and chin with an inability to close the left eyelid. Her serum angiotensin-converting enzyme level was twice the upper limit of normal. Prominent hilar markings were identified on chest x-ray, but no focal opacity was seen. Fine-needle aspiration of a preauricular lymph node revealed noncaseating granulomas consistent with granulomatous lymphangitis. The patient was given a diagnosis of Heerfordt-Waldenström syndrome, or uveoparotid fever. Treatment with a high-dose steroid improved her parotid gland enlargement, facial palsy, and anterior uveitis.

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.

As a subtype of the clinical presentations associated with sarcoidosis, the combination of uveitis, parotid gland swelling, and facial nerve palsy is known as Heerfordt's syndrome. This syndrome is an extremely rare disorder that has been estimated to affect only 4.1-5.6% of patients with sarcoidosis. We present 2 cases of Heerfordt's syndrome associated with radiculopathy in the trunk. The 2 patients experienced unilateral or bilateral radiculopathy in the trunk and in the trigeminal nerve area associated with Heerfordt's syndrome. Radiculopathy is also a rare manifestation in patients with neurosarcoidosis. A literature review revealed that only 51 cases of radiculopathy associated with sarcoidosis have been documented. A diagnosis of Heerfordt's syndrome was observed in 7 out of these 51 cases. Together with our 2 cases, 9 out of 53 patients with radiculopathy associated with sarcoidosis have been diagnosed as having Heerfordt's syndrome (estimated frequency, 16.9%). In conclusion, radiculopathy is a common neurological manifestation in patients with Heerfordt's syndrome. On the basis of our experience, we suggest that physicians consider the possibility of Heerfordt's syndrome in cases of radiculopathy with unknown cause.

Summary 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.

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 (

This article reports the case of a 14-year-old boy who was presented in the case conference with symptoms of decreased visual acuity, scintillating scotomas and photophobia. Physical examination revealed right facial paralysis, parotid gland swelling, high fever and poor general condition. Ophthalmoscopy revealed anterior and posterior uveitis including macular edema and chorioretinal infiltrates. Angiography revealed a dense pattern of hyperfluorescent lesions and these observations resulted in the diagnosis of Heerfordt syndrome. Under systemic prednisolone therapy, symptoms were reduced and visual acuity recovered.

A 50-year-old male patient demonstrated an existing left proptosis for several weeks. The patient was suffering from physical exhaustion and had lost considerable weight. Furthermore, we observed greatly enlarged parotid and submandibular glands on both sides. MRI of the neck showed multiple, sharply circumscribed lesions in the major salivary glands and both lacrimal glands as well as in the orbit. Initially we suspected Heerfordt's syndrome, a manifestation of sarcoidosis, but laboratory diagnosis could not reveal a pathological erythrocyte sedimentation rate or an increased ACE titer. After exploratory excision from the right submandibular gland, histological examination revealed Castleman's disease. Therefore, we initiated an immunomodulatory therapy with interleukin-6 receptor antagonists.Castleman's disease is one of the very rare, benign, lymphoproliferative processes that have a tendency to turn malignant. Isolated findings of Castleman's disease should be completely resected. There are no clear treatment strategies for multiple localizations of Castleman's disease. The approaches range from systemic glucocorticoid therapy with chemotherapy to immunomodulatory treatment. In contrast to isolated findings, the prognosis for multicentric occurrence is unfavorable.

Acute presentation of sarcoidosis with the combination of uveitis, parotid gland enlargement, facial nerve palsy, and fever is called Heerfordt's syndrome. Clinically recognizable involvement of the nervous system occurs in10% of patients with sarcoidosis, and polyneuropathy in 24% with neurosarcoidosis.A 28-year-old woman diagnosed with Guillain-Barré syndrome was admitted and treated for a month in the Department of Neurology, Mie University hospital. Her visual acuity decreased 2 weeks after discharge. She was admitted to the Department of Ophthalmology, Mie University Hospital. She presented typical optic sarcoidosis. As she had uveitis, facial nerve palsy, parotid gland enlargement and fever in the clinical course, we diagnosed her condition as Heerfordt's syndrome.On rare occasions a Heerfordt's syndrome patient may present with Guillain-Barré-like symptoms.

International audience; M. tuberculosis is a possible cause of uveitis; the diagnosis is often difficult. The aim of this study is to evaluate, in this context, the application of an interferon gamma assay, the SpotTB™. The prospective study, in patients presenting with uveitis without obvious cause and not resolving with local treatment, compared the therapeutic approach (giving antituberculous treatment or not) with and without the result of the SpotTB™. The analyses included 23 patients: 78% had received BCG; 78% had isolated ocular involvement; 22% had associated mediastinal lymphadenopathy. The results of the IDR and the SpotTB™ were concordant in 61%, discordant in 22% and non-evaluable in 17% of cases. In the patients with isolated ocular involvement the SpotTB™ avoided treatment in five and reinforced the decision not to treat in 13 others. In the patients with mediastinal lymphadenopathy, the SpotTB™ reinforced the diagnosis of sarcoidosis in four cases (one lost from view). In conclusion, when infection with M. tuberculosis is considered in the aetiology of uveitis the SpotTB™ allows, in a significant number of cases, the avoidance of antituberculous treatment.

We found no cases of familial sarcoidosis with Heerfordt syndrome in the literature. A 25-year-old woman presented with high grade fever and parotid gland swelling in April 2004. An accompany with Right peripheral facial palsy developed in June, following which she was treated with corticosteroid therapy for 7 days, and her condition temporarily improved. Four months later she came to our hospital complaining of glare, then uveitis was diagnosed. She was referred to our department for work-up for treatment of sarcoidosis in January 2005. TBLB/BALF findings by bronchoscopy for definitive diagnosis were compatible with sarcoidosis. Various clinical symptoms indicated Heerfordt syndrome. Interestingly, pericardial effusion was also detected. In addition, her sister also suffers from sarcoidosis. Our patient had a favorable response to oral corticosteroid therapy (PSL 30 mg/day), gradually tapered 2.5-5 mg every 4 weeks, then stopped at the eighth month after commencing therapy. However, pulmonary lesions have relapsed 3 years after the discontinuation of the therapy. It is well known that relapses can happen when corticosteroids are used, and we should consider the progression of this disease and the indications of further treatment in this case.

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by the JC virus; the mortality rate is high and it is usually refractory to treatment. In non-HIV patients, PML occurs as a late consequence of hematologic malignancies or during prolonged immunosuppression for transplantation or autoimmune disease. We describe a 34-year-old PML patient with incomplete Heerfordt syndrome, a rare type of sarcoidosis, who had not received any immunosuppressants, including steroids, at the onset and who was clinically and radiologically responsive to the antiviral drug cidofovir.

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.

BACKGROUND: Heerfordt syndrome is rare and is characterized by fever, uveitis, parotid gland enlargement, and facial nerve palsy. We hereby present a case of Heerfordt syndrome with unilateral facial nerve palsy as a presentation of sarcoidosis. HISTORY AND SIGNS: A 29-year-old male patient from Sri Lanka presented with eye redness OU, blurred vision OD, fever, headache, night sweat, fatigue, and weight loss (5 kg over 1 month). Examination revealed mild anterior uveitis OU, mild vitritis OD, fundus whitish lesions OU, left otalgia, taste disorders, bilateral parotid gland enlargement, and left facial nerve palsy. Work-up for infection or tumour was negative. Chest computed tomography and transbronchial lymph node biopsy set the diagnosis of sarcoidosis. THERAPY AND OUTCOME: The patient recovered completely within 2 months under therapy with prednisone and azathioprine. One year after onset of treatment, no recurrence was noted. CONCLUSIONS: Heerfordt syndrome is a rare manifestation of neurosarcoidosis and has to be included in the differential diagnosis of facial nerve palsy.