Sarcoid-like granulomatous myositis-associated hypercalcemia. An infrequent case to consider / Hipercalcemia asociada a miositis granulomatosa sarcoidea simil- sarcoidea. Un caso infrecuente a considerar

Abstract PTH-independent hypercalcemia due to granulomatous disease is well-documented and sarcoidosis is the most characteristic disease, although there are others. We describe a case of sarcoid-like granulomatous myositis. An 87-year-old man was referred with tetraparesis and hypercalcemia (albumin-corrected calcium of 13.4 mg/dl) following a trip to the Caribbean. The evaluation showed a suppressed PTH, 25-hydroxy vitamin D of 7.5 ng/ml, 18F-FDG PET/CT showed markedly increased uptake in intercostal, back, shoulder, but tock and thigh muscles and a deltoid biopsy confirmed extensive granulomatous myositis. He was prescribed glucocorticoids which resulted in normalized plasma calcium levels and complete recovery from tetraparesis. Sarcoid-like granulomatous myositis should be incorporated into the differential diagnosis of PTH-independent hypercalcemia, especially in the absence of clinical features of sarcoidosis and with special emphasis on the use of 18F-FDG PET/CT to ensure a correct approach.

Resumen La hipercalcemia PTH-independiente asociada a enfermedades granulomatosas está bien documentada y la sarcoidosis es la enfermedad más característica, a pesar de que existen otras. Des cribimos un caso de miositis granulomatosa simil-sarcoidea. Un hombre de 87 años consultó por tetraparesia e hipercalcemia (calcio corregido por albúmina 13.4 mg/dl) luego de un viaje al Caribe. La evaluación mostró una PTH suprimida, 25-hidroxivitamina D 7.5 ng/ml, 18F-FDG PET/CT mostró marcado aumento de captación a nivel de musculatura intercostal, dorsal, deltoidea, glúteos y muslos. Una biopsia deltoidea confirmó una miositis granulomatosa extensa. Se prescribieron glucocorticoides, resultando en normalización del calcio plasmático y completa recuperación de la tetraparesia. La miositis granulomatosa simil-sarcoidea debe ser incorporada den tro del diagnóstico diferencial de la hipercalcemia PTH-independiente, especialmente en ausencia de hallazgos clínicos de sarcoidosis y con especial énfasis en el uso de 18F-FDG PET/CT para su correcta aproximación.

Symptomatic cholelithiasis may be the first sign of sarcoidosis

SUMMARY Sarcoidosis is a multisystemic noncaseating granulomatous disease that rarely affects the gastrointestinal system. The initial diagnosis of sarcoidosis with gallbladder/gallbladder-associated lymph node involvement is a very rare condition in the literature. Herein, we aimed to report a case of newly diagnosed sarcoidosis with lymph node involvement associated with the gallbladder.

RESUMO A sarcoidose é uma doença granulomatosa multissistêmica não-caseosa que raramente afeta o sistema gastrointestinal. O diagnóstico inicial de sarcoidose com envolvimento de linfonodo da vesícula biliar ou associado à vesícula biliar é muito raro na literatura. Aqui, o nosso objetivo foi relatar um caso de sarcoidose recém-diagnosticado com envolvimento de linfonodos associados à vesícula biliar.

Giant cell myocarditis causing sudden death in a patient with sarcoidosis

Giant cell myocarditis (GCM) is a rare and rapidly fatal cardiovascular condition most often seen in young adults. It is characterized microscopically by myocardial necrosis with multinucleated giant cells in the absence of well-defined granulomas. This disorder has typically been attributed to manifest as heart failure, but in some individuals, GCM may present as sudden cardiac death. Herein, we present a fatal case of GCM in a 36-year-old male with a history of autoimmune disorders. The decedent presented to the emergency room due to vomiting and was treated for nausea due to suspected dehydration. He was discharged that night and found dead on his bathroom floor the following day. Postmortem examination revealed psoriasis and granulomatous lesions in the lungs consistent with sarcoidosis, further supporting circumstantial evidence existing between GCM and autoimmune disorders. Additionally, this case provides an opportunity to distinguish GCM from the distinct clinical entity of cardiac sarcoidosis (CS), especially in the setting of systemic sarcoidosis. We hope to raise awareness of this rare disease process and its potential to cause sudden cardiac death so that it may be considered in a differential diagnosis as immunosuppression and early cardiac transplantation largely determine the prognosis.

Enfermedad granulomatosa hepática / Hepatic granulomatous disease

Resumen Los granulomas son lesiones circunscritas compuestas principalmente por células mononucleares que surgen en respuesta a estímulos antigénicos pobremente degradables. Se encuentran en 2 a 15 % de las biopsias hepáticas; su hallazgo puede significar desde un fenómeno incidental, hasta la manifestación de una enfermedad sistémica de origen infeccioso, autoinmune o neoplásico. El cuadro clínico suele apuntar a la patología subyacente, sin embargo, la lista de condiciones asociadas es amplia y difiere con base en los antecedentes epidemiológicos y a las características basales del paciente. El elemento de mayor utilidad para su estudio es la historia clínica exhaustiva, con énfasis en viajes recientes, exposición de riesgo y consumo de fármacos o alimentos crudos o exóticos. El análisis histopatológico detallado puede auxiliar en la identificación de la etiología, por ejemplo, la presencia de granulomas epitelioides con necrosis caseosa indica tuberculosis y su ausencia, sarcoidosis; la abundancia de eosinófilos es señal de reacciones farmacológicas o infecciones parasitarias; la presencia de cuerpos extraños puede ser la causa de la enfermedad granulomatosa hepática. En este artículo describimos los aspectos clínico-patológicos básicos de esta enfermedad y proveemos un breve resumen de las etiologías más comunes, principalmente en la región de Latinoamérica.

Abstract Granulomas are circumscribed lesions mainly composed of mononuclear cells that arise in response to poorly degradable antigenic stimuli. They are found in 2-15 % of liver biopsies and the meaning of their finding can range from an incidental phenomenon to the manifestation of a systemic disease of infectious, autoimmune or neoplastic origin. Clinical presentation usually points at the underlying pathology; however, the list of associated conditions is extensive, and differs based on patient epidemiological history and baseline characteristics. The most useful element for their study is a thorough medical history, with an emphasis on recent trips, exposures and consumption of drugs or raw or exotic foods. Detailed histopathological analysis may help identify the etiology. For example, the presence of epithelioid granulomas with caseous necrosis indicates tuberculosis and, its absence, sarcoidosis; eosinophil abundance can be associated with drug reactions or parasitic infections; and the presence of foreign bodies can be the cause of granulomatous liver disease (GLD). In this article, we describe the basic clinical-pathological aspects of GLD, and provide a brief summary of the most common etiologies, with an emphasis on the Latin-American region.

Distúrbios de condução e arritmias na sarcoidose cardíaca - relato de caso / Conduction disturbances and arrhythmias in cardiac sarcoidosis - case report

A forma cardíaca isolada da Sarcoidose é pouco frequente. no entanto, o envolvimento cardíaco na sarcoidose é mais prevalente e ocorre em 5 % desses pacientes. O diagnóstico de sarcoidose cardíaca é um desafio por causa das manifestações inespecíficas, sendo a sensibilidade e especificidade das modalidades diagnósticas limitadas. Este relato de caso teve por objetivo descrever o caso de um paciente sem diagnóstico prévio de sarcoidose com arritmias e distúrbio de condução ventricular compatíveis com acometimento cardíaco da doença. Optou-se para o tratamento um implante de marcapasso definitivo e terapia com corticosteroides

The isolated cardiac form of sarcoidosis is infrequent. However, cardiac involvement in sarcoidosis is more prevalent and occurs in 5% of these patients. The diagnosis of cardiac sarcoidosis is a challenge because of non-specific manifestations, and the sensitivity and specificity of the diagnostic modalities are limited. This case report aimed to describe the case of a patient without previous diagnosis of sarcoidosis with arrhythmias and ventricular conduction disturbance compatible with cardiac involvement of the disease. A definitive pacemaker implant and corticosteroid therapy were chosen for the treatment

Granulomatous intersticial nephritis secondary to sarcoidosis / Nefrite intersticial granulomatose secundária a sarcoidose

ABSTRACT Introduction: Granulomatous interstitial nephritis is a rare condition, in which renal involvement is uncommon. Its etiology is variable, and may be medicinal, infectious or inflammatory origin. Case report: This is a 65-year-old male patient with renal lesions of unknown etiology, associated with hypercalcaemia. During the investigation, cardiac insufficiency with diastolic dysfunction and interstitial lung involvement on chest tomography were evidenced. Renal function (glomerular filtration rate) has partially improved with clinical measures. Renal biopsy was performed, which showed moderate interstitial lesion with tuberculoid granulomas without caseous necrosis. Conclusion: The objective of the article was to describe a case of NIG and to alert to the importance of its clinical investigation. In this case, renal biopsy, associated with systemic clinical manifestations, contributed to the diagnosis of sarcoidosis.

RESUMO Introdução: a nefrite intersticial granulomatosa é uma condição rara, na qual o envolvimento renal é incomum. Sua etiologia é variável e pode ter origem medicinal, infecciosa ou inflamatória. Relato de caso: trata-se de um paciente do sexo masculino, com 65 anos de idade, com lesões renais de etiologia desconhecida, associadas à hipercalcemia. Durante a investigação, evidenciaram-se insuficiência cardíaca com disfunção diastólica e envolvimento pulmonar intersticial à tomografia torácica. A função renal (taxa de filtração glomerular) melhorou parcialmente com medidas clínicas. Foi realizada biópsia renal, que apresentou lesão intersticial moderada com granulomas tuberculoides sem necrose caseosa. Conclusão: o objetivo do artigo foi descrever um caso de GIN e alertar para a importância de sua investigação clínica. Neste caso, a biópsia renal, associada a manifestações clínicas sistêmicas, contribuiu para o diagnóstico de sarcoidose.

Cutaneous sarcoidosis and secondary open-angle glaucoma in a patient: case report and literature review

Abstract The current report presents the case of a 41-year-old male patient with a two-month history of asthenopia and plaques in the frontotemporal region. Computed tomography revealed bilateral hilar and mediastinal lymphadenopathy. Ophthalmological examination showed elevated intraocular pressure. Skin biopsy demonstrated aa dermal inflammatory infiltrate composed mainly of epithelioid cells and a few multinucleated giant cells, but no obvious lymphocytes. Findings of thorough physical examinations and auxiliary examinations suggested the presence of cutaneous sarcoidosis and secondary open-angle glaucoma. Treatment consisted mainly of oral methylprednisolone. Skin lesions, bilateral hilar, and mediastinal lymphadenopathy resolved completely. Cutaneous sarcoidosis is often accompanied by extracutaneous organ involvement. Dermatologists must be aware of the disease's extracutaneous manifestations to ensure accurate diagnosis for further treatments.

Sarcoid-like cutaneous lesions in chronic adult paracoccidioidomycosis: report of two cases

ABSTRACT This study reports two cases of chronic paracoccidioidomycosis with sarcoid-like cutaneous lesions. The patients began the treatment in 2013 at Hospital Universitário Clementino Fraga Filho (HUCFF) of the Universidade Federal do Rio de Janeiro (UFRJ). The first case (mild form) was treated with trimethoprim-sulfamethoxazole (8 mg /kg per day, orally) for three months and, then, with half the dose for nine months; the second (moderate form), with itraconazole (200 mg per day, orally) for 12 months. We point out the rareness of the sarcoid-like cutaneous lesions and the differential diagnoses for other granulomatous diseases.

Sarcoidose ocular: a nossa realidade nos últimos seis anos / Ocular sarcoidosis: our reality for the past six years

RESUMO Introdução: Na sarcoidose, as manifestações oculares são comuns e podem constituir a manifestação inicial da doença ou mesmo a única. O objetivo deste trabalho foi analisar os parâmetros demográficos, manifestações clínicas, terapêutica e principais complicações oculares em doentes com sarcoidose ocular. Métodos: Estudo descritivo e retrospectivo que incluiu doentes com o diagnóstico de sarcoidose ocular, observados nas consultas de Inflamação Ocular e de Doenças Auto-Imunes do Hospital Prof. Doutor Fernando Fonseca, no período entre 2009 e 2015. Resultados: Foram identificados 11 doentes com o diagnóstico de sarcoidose ocular, com predomínio do sexo feminino (54,5%) e caucasianos. A média da idade ao diagnóstico foi de 45±14 anos. A sarcoidose manifestou-se de forma exclusivamente ocular em 36% dos casos. O envolvimento ocular foi a manifestação inicial em 90,9% dos casos. Identificaram-se 9 casos de uveíte, 1 de esclerite anterior nodular e 1 de queratite intersticial. O tratamento com corticoterapia tópica foi realizado em 100% dos casos, sendo o tratamento único em apenas 1 doente. Nos restantes, foi necessário associar corticoterapia oral. Em 4 desses doentes, pela gravidade da doença e atingimento binocular, utilizou-se também corticoterapia pulsada endovenosa. O tratamento adjuvante imunossupressor mais frequentemente utilizado foi o metotrexato (45%). Um doente necessitou de terapia biológica com infliximabe para controle da doença. Conclusão: A manifestação ocular mais comum foi a uveíte, com predomínio da panuveíte. O tratamento mais utilizado e com maior taxa de controle da doença foi a corticoterapia sistêmica em associação com o metotrexato.

ABSTRACT Purpose: In sarcoidosis, ocular manifestations are common and can be the initial or even the only clinical manifestation. The aim of this study was to analyze the demographic parameters, clinical manifestations, treatment and the major ocular complications in patients with ocular sarcoidosis. Methods: We conducted a descriptive and retrospective study that included patients with the diagnosis of ocular sarcoidosis, followed by inflammatory ophthalmology and immune-mediated disease consults at the Prof. Doutor Fernando Fonseca Hospital, between 2009 and 2015. Results: Eleven patients with the diagnosis of ocular sarcoidosis were identified, with a predominance of females (54,5%) and Caucasians. The average age at diagnosis was 45 ± 14 years. Sarcoidosis was exclusively ocular in 36%. The first manifestation of sarcoidosis was eye disease in 90.9 % of cases. Nine cases of uveitis, one of nodular scleritis and one of interstitial keratitis were observed. Topical corticoid treatment was applied in 100% of cases, with only one achieving remission of the disease. Oral corticoid treatment was necessary in 10 cases, four of which needed a high dose methylprednisolone induction. Methotrexate was the adjunctive immunosuppressive treatment of choice in 45% of cases. There was one refractory case for conventional immunosuppressive therapy, having achieved remission with biologic agent infliximab. Conclusion: Uveitis was the commonest ocular manifestation, and there was a predominance of panuveitis. Systemic corticoid and methotrexate were the most used immunosuppressive treatments for maintaining the controlled stated of the disease.

Sarcoïdose systémique du sujet âgé et amylose : complication ou association fortuite ? A propos d'une observation

Introduction : La sarcoïdose est une granulomatose systémique de cause inconnue. Son association avec une amylose a été exceptionnellement décrite. Nous en rapportons une observation association.Observation : Une patiente âgée de 68 ans était admise dans notre service pour exploration de troubles psycho-comportementaux associés à une macro-polyadénopathie. Le diagnostic d'une sarcoïdose à localisation médiastino-ganglionnaire, cutanée et neurologique était retenu. La biopsie des salivaires accessoires mettait en évidence des dépôts amyloïdes. Le bilan de retentissement de l'amylose objectivait une altération de la fonction rénale et uneprotéinurie à 2,36 g/24H. La corticothérapie associée à l'hydroxy-chloroquine a été instituée. L'évolution était favorable. Conclusion : Cette observation illustre l'intérêt de la recherche systématique d'une amylose au cours de la sarcoïdose. La biopsie des glandes salivaires accessoires est de par son innocuité, la technique de référence dans l'amylose systémique du sujet âgé

Apendicitis granulomatosa sarcoidal: una infrecuente entidad clínico-patológica. Caso clínico / Granulomatous appendicitis caused by sarcoidosis: Report of one case

Granulomatous appendicitis is uncommon and can be caused by Crohn's disease, foreign body reactions, infections or sarcoidosis. We report a 26-year-old female consulting in the emergency room for pain in the right lower abdomen. She was released with analgesics and consulted 14 days later for the same pain. This time she was operated with the preoperative diagnosis of appendicitis. The pathological study of the surgical piece was compatible with a granulomatous appendicitis caused by sarcoidosis.

Musculoskeletal involvement in sarcoidosis / Acometimento músculo-esquelético na sarcoidose

Sarcoidosis is a multisystem inflammatory disorder of unknown cause. It most commonly affects the pulmonary system but can also affect the musculoskeletal system, albeit less frequently. In patients with sarcoidosis, rheumatic involvement is polymorphic. It can be the presenting symptom of the disease or can appear during its progression. Articular involvement is dominated by nonspecific arthralgia, polyarthritis, and Löfgren's syndrome, which is defined as the presence of lung adenopathy, arthralgia (or arthritis), and erythema nodosum. Skeletal manifestations, especially dactylitis, appear mainly as complications of chronic, multiorgan sarcoidosis. Muscle involvement in sarcoidosis is rare and usually asymptomatic. The diagnosis of rheumatic sarcoidosis is based on X-ray findings and magnetic resonance imaging findings, although the definitive diagnosis is made by anatomopathological study of biopsy samples. Musculoskeletal involvement in sarcoidosis is generally relieved with nonsteroidal anti-inflammatory drugs or corticosteroids. In corticosteroid-resistant or -dependent forms of the disease, immunosuppressive therapy, such as treatment with methotrexate or anti-TNF-α, is employed. The aim of this review was to present an overview of the various types of osteoarticular and muscle involvement in sarcoidosis, focusing on their diagnosis and management.

A sarcoidose é um distúrbio inflamatório multissistêmico de causa desconhecida, frequentemente afetando o sistema pulmonar e também o sistema músculo-esquelético, mas de forma menos frequente. Em pacientes com sarcoidose, o acometimento reumático é polimórfico, podendo ser o sintoma de apresentação da doença ou aparecer durante sua progressão. O acometimento articular é dominado por artralgia inespecífica, poliartrite e síndrome de Löfgren, que é definida como a presença de adenopatia pulmonar, artralgia (ou artrite) e eritema nodoso. Manifestações esqueléticas, especialmente dactilite, aparecem principalmente como complicações de sarcoidose crônica e em vários órgãos. O acometimento muscular na sarcoidose é raro e geralmente assintomático. O diagnóstico de sarcoidose reumática baseia-se em achados radiográficos e de ressonância magnética, embora o diagnóstico definitivo seja feito pelo estudo anatomopatológico de amostras de biópsia. O acometimento músculo-esquelético na sarcoidose é geralmente aliviado com o uso de anti-inflamatórios não esteroidais ou corticosteroides. Em formas da doença resistentes ao corticosteroide ou corticosteroide dependentes, a terapia de imunossupressão, como o tratamento com metotrexato ou anti-TNF-α, é utilizada. O objetivo desta revisão foi apresentar uma visão geral dos vários tipos de acometimento osteoarticular e muscular na sarcoidose, com foco no diagnóstico e manejo.

Subcutaneous sarcoidosis presenting as a suprapubic mass, acute kidney injury, and hypercalcemia

No abstract available.

Tuberculosis Lymphadenitis in a Well Managed Case of Sarcoidosis.

Differentiation between tuberculosis (TB) and sarcoidoisis is sometimes extremely difficult. Sequential occurrence of sarcoidosis and TB in the same patient is uncommon. We present the case of a young man, with a proven diagnosis of sarcoidosis who later developed TB after completion of treatment for sarcoidosis. A 32-year-old male patient presented with low-grade fever since two months. Physical examination revealed cervical lymphadenopathy. Initial fine needle aspiration cytology (FNAC) of the cervical lymph node was suggestive of granulomatous inflammation; the chest radiograph was normal. Repeat FNAC from the same lymph node was suggestive of reactive lymphoid hyperplasia. The patient was treated with antibiotics and followed-up. He again presented with persistence of fever and lymphadenopathy and blurring of vision. Ophthalmological examination revealed uveitis, possibly due to a granulomatous cause. His repeat Mantoux test again was non-reactive; serum angiotensin converting enzyme (ACE) levels were raised. This time an excision biopsy of the lymph node was done which revealed discrete, non-caseating, reticulin rich granulomatous inflammation suggestive of sarcoidosis. The patient was treated with oral prednisolone and imporved symptomatically. Subsequently, nearly nine months after completion of corticosteroid treatment, he presented with low-grade, intermittent fever and a lymph node enlargement in the right parotid region. FNAC from this lymph node showed caseating granulomatous inflammation and the stain for acid-fast bacilli was positive. He was treated with Category I DOTS under the Revised National Tuberculosis Control Programme and improved significantly. The present case highlights the need for further research into the aetiology of TB and sarcoidosis.

Muscular Sarcoidosis Detected by F-18 FDG PET/CT in a Hypercalcemic Patient

Sarcoidosis is a systemic granulomatous disease of unknown etiology that involves many organs, occasionally mimicking malignancy. We herein report a 50-yr-old woman of muscular sarcoidosis of chronic myopathic type, manifested by hypercalcemia and muscle wasting. Besides insignificant hilar lymphadenopathy, her sarcoidosis was confined to generalized atrophic muscles and therefore, F-18 FDG PET/CT alone among conventional imaging studies provided diagnostic clues for the non-parathyroid-related hypercalcemia. On follow-up PET/CT during low-dose steroid treatment, FDG uptake in the muscles disappeared whereas that in the hilar lymph nodes remained. PET/CT may be useful in the evaluation of unexpected disease extent and monitoring treatment response in suspected or known sarcoidosis patients.

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.

Sarcoidosis in north indian population: A retrospective study.

Background. Sarcoidosis is a systemic granulomatous disease of unknown origin most commonly involving the lungs. Sarcoidosis is frequently misdiagnosed due to its clinico-radiological resemblance to tuberculosis (TB). Hence, the present study was undertaken with the aim of studying the clinico-radiological profile of sarcoidosis in the Indian context. Methods. We retrospectively studied 146 patients diagnosed to have sarcoidosis during the period 2001-2010 at one of the respiratory units at Vallabhbhai Patel Chest Institute. Results. Majority of them (70%) were more than 40 years of age; females comprised 58.2% of the patients. Before coming to our clinic, 30% patients had been misdiagnosed to have TB. Cough (89.7%) was the most common presenting symptom; joint symptoms (28.8%) and end inspiratory crepitations at lung bases (49.3%) were other salient manifestations. Cutaneous involvement and digital clubbing were rarely seen. Pulmonary function testing showed restriction with impaired diffusion in 72.7% patients. The most common radiological feature was bilaterally symmetrical hilar lymphadenopathy. Transbronchial lung biopsy (TBLB) had a very high diagnostic yield (90.8%). Conclusions. Sarcoidosis is often misdiagnosed as TB in India. Transbronchial lung biopsy has high diagnostic yield in sarcoidosis.