scholarly journals Chronic Inflammatory Arthropathy Preceding Acute Systemic Manifestations of Sarcoidosis: A Possible Overlap of Idiopathic Juvenile Arthritis and Sarcoidosis

2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Matheus Campello Vieira ◽  
Priscilla Gomes Tosta ◽  
Fernanda Morello Nicole ◽  
Lucas Enock V. Roberto ◽  
Júlia Guasti P. Vianna ◽  
...  
Keyword(s):  
Elevated Serum ◽  
Lymphoproliferative Disease ◽  
Juvenile Arthritis ◽  
Unknown Etiology ◽  
Multisystem Disease ◽  
Idiopathic Juvenile Arthritis ◽  
Diagnostic Hypothesis ◽  
Liver Surface ◽  
Systemic Manifestations ◽  
Noncaseating Granulomas

Sarcoidosis is a multisystem disease with unknown etiology, marked by T lymphocytes and macrophages agglomeration, which leads to the formation of noncaseating granulomas in the affected tissues. We describe a case of a 40-year-old black patient referred to our service for evaluation of nephrolithiasis and persistent elevation of plasma creatinine. He reported important weight loss, fever episodes, and abdominal and low back intermittent pain in the past 6 months. The investigation revealed elevated serum calcium level, hepatosplenomegaly, retroperitoneal lymphadenopathy, anemia, thrombocytopenia, and nephrolithiasis. The initial diagnostic hypothesis was lymphoproliferative disease, but the laparoscopic propaedeutic showed multiple white lesions on the liver surface, which biopsy identified as noncaseating granulomas with asteroid corpuscles, suggestive of sarcoidosis. He was treated with corticosteroids with significant improvement in symptoms and in calcium and creatinine levels. Besides, the patient presented a long-term large joints arthropathy, especially on the knees (with bilateral prosthesis), wrists, and ankles, of unknown etiology. We discuss the systemic manifestations of sarcoidosis related to the reported case, as well as the possible overlapping of idiopathic juvenile arthritis with sarcoidosis.

Blurry Vision, Hearing Loss, and Constant Headache

2018 ◽  
pp. 49-54
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Pulmonary Involvement ◽  
Giant Cells ◽  
Skin Lesions ◽  
Neurological Involvement ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Initial Symptoms ◽  
Blurry Vision ◽  
Systemic Manifestations ◽  
Noncaseating Granulomas

Sarcoidosis is a multisystem inflammatory disorder of unknown etiology, characterized pathologically by the presence of noncaseating granulomas and multinucleated giant cells. For unknown reasons, the disorder tends to be more common among African Americans and Scandinavians. Among the most common systemic manifestations are pulmonary involvement, lymphadenopathy, and skin lesions. Nervous system involvement occurs in 5–10% of cases. Despite the rarity of neurological involvement, 50–70% of the time, the neurological symptoms are the initial symptoms. In most patients with neurosarcoid, there is evidence for involvement in other systems, either at presentation or during the course of the condition. The presentation in neurosarcoidosis is varied. Steroids are the first-line treatment, but some patients may also require steroid-sparing agents to control the condition.

scholarly journals Perigastric Hyaline-Vascular Variant Castleman’s Disease

2021 ◽  
pp. 632-638
Author(s):  
Yu Ming Jin ◽  
Gui Ying Jing
Keyword(s):  
Histopathological Examination ◽  
Immunohistochemical Analysis ◽  
Lymphoproliferative Disease ◽  
Castleman Disease ◽  
Unknown Etiology ◽  
Mesenchymal Tumors ◽  
Imaging Characteristics ◽  
Contrast Enhanced ◽  
Hyaline Vascular Variant ◽  
Vascular Variant

Castleman disease (CD) is a rare chronic lymphoproliferative disease with unknown etiology and pathogenesis disease. When the lesion is located in the mediastinum, the diagnosis of CD is easy. However, if the lesion presents as a perigastric mass mimicking other subserosal gastric mesenchymal tumors, the diagnosis can be challenging. As few sonographic manifestations of hyaline-vascular variant CD, especially contrast-enhanced ultrasound (CEUS) imaging, as well as computed tomography (CT) and histopathological imaging, have been reported in literature, this case may provide a vivid example of a comprehensive CEUS and CT usage in the diagnosis and surgery with regard to CD. This report presents a case of a 50-year-old female diagnosed with hyaline-vascular variant CD in a random physical examination, the ultrasound examination first revealed a 24.3 mm × 15.4 mm hypoechogenic lesion abutting the stomach, esophagus, and liver, which was under the suspicion of gastrointestinal stromal tumor. Following a series of medical examinations, including CEUS, CT, postoperative histopathological examination, and immunohistochemical analysis, the patient was diagnosed with hyaline-vascular variant unicentric CD. After the mass was completely excised through laparoscopic surgery, the woman recovered very well without recurrence during a follow-up period of 15 months. Thus, mastering ultrasound and CT-imaging characteristics of CD and applying ultrasound and CT examination together would do help to preoperative diagnosis.

scholarly journals Spontaneous dissolution of a cyst located within the septum pellucidum in a patient with sarcoidosis: a case report

2021 ◽  
Vol 10 (1) ◽  
pp. 205846012098551
Author(s):  
Martina Kastrup Loft ◽  
Søren Rafael Rafaelsen ◽  
Malene Roland V Pedersen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Septum Pellucidum ◽  
Unknown Etiology ◽  
Mediastinal Lymph Nodes ◽  
Resonance Imaging ◽  
Multisystem Disease ◽  
Meningeal Enhancement ◽  
Cerebral Magnetic Resonance Imaging ◽  
Cavum Septum Pellucidum ◽  
Contrast Enhanced

Sarcoidosis is a granulomatous multisystem disease of unknown etiology. Typically, the disease affects the lungs, causing enlargement of the mediastinal lymph nodes, but other organs can be affected. Neurosarcoidosis is reported in 5–10% of the patients. This case represents a 39-year-old male patient diagnosed with lung sarcoidosis. Due to neurological symptoms, a contrast-enhanced cerebral magnetic resonance imaging was performed. Neurosarcoidosis was presented with meningeal enhancement adjacent to a cyst located within the cavum septum pellucidum. The cyst dissolved spontaneously within six months. The finding of a cyst located within the septum pellucidum is rare.

scholarly journals Evaluation of nasal mucociliary clearance in rheumatoid arthritis: a comparative analysis using saccharin test

2017 ◽  
Vol 5 (11) ◽  
pp. 5026 ◽  
Author(s):  
Shelja Deswal ◽  
Jyoti Yadav ◽  
Mohit Deswal ◽  
Harpreet Singh
Keyword(s):  
Rheumatoid Arthritis ◽  
Disease Duration ◽  
Mucociliary Clearance ◽  
Systemic Disease ◽  
Unknown Etiology ◽  
Control Group ◽  
Ciliary Activity ◽  
Multisystem Disease ◽  
Organic Particles ◽  
Nasal Mucociliary Clearance

Background: Rheumatoid arthritis (RA) is a chronic multisystem disease of unknown etiology characterized by persistent inflammatory synovitis, usually involving peripheral joints in a symmetric distribution. RA is a systemic disease often associated with cutaneous and organ-specific extra-articular manifestations the mucociliary clearance system protects the lower respiratory system by trapping and removing inhaled pathogenic viruses and bacteria, in addition to nontoxic and toxic particulates (e.g., pollen, ash, mineral dust, mold spores, and organic particles), from the lungs. Effective clearance requires both ciliary activity and the appropriate balance of periciliary fluid and mucus.Methods: This was a case control study conducted in the Department of Physiology, Pt. B.D. Sharma PGIMS, Rohtak in 50 females of age group 30-50 years. Control group comprised of 25 healthy volunteer females while study group comprised of 25 rheumatoid arthritis female patients with disease duration of more than five years. Proven cases of RA (as per 1987 ACR criteria) were taken with disease duration of more than five years from Rheumatology clinic of Pt. B.D. Sharma PGIMS, Rohtak. Nasal mucociliary clearance time was evaluated by saccharin method.Results: The results of our study showed abnormal mucociliary clearance in rheumatoid arthritis patients.Conclusions: The study shows an abnormal mucociliary clearance in rheumatoid arthritis patients. Impairment of mucociliary clearance seems to be the result of qualitative and quantitative alterations in respiratory secretions.

scholarly journals Clinical Presentations, Pathogenesis, and Therapy of Sarcoidosis: State of the Art

2020 ◽  
Vol 9 (8) ◽  
pp. 2363
Author(s):  
Francesca Polverino ◽  
Elisabetta Balestro ◽  
Paolo Spagnolo
Keyword(s):  
Clinical Course ◽  
State Of The Art ◽  
Systemic Disease ◽  
Unknown Etiology ◽  
Therapeutic Approaches ◽  
Epidemiologic Factors ◽  
Adaptive Immune ◽  
Clinical Presentations ◽  
Specific Organ ◽  
Noncaseating Granulomas

Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas that can occur in any organ, most commonly the lungs. Early and accurate diagnosis of sarcoidosis remains challenging because initial presentations may vary, many patients are asymptomatic, and there is no single reliable diagnostic test. Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement. From a pathobiological standpoint, sarcoidosis represents an immune paradox, where an excessive spread of both the innate and the adaptive immune arms of the immune system is accompanied by a state of partial immune anergy. For all these reasons, the optimal treatment for sarcoidosis remains unclear, with corticosteroid therapy being the current gold standard for those patients with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease. This review is a state of the art of clinical presentations and immunological features of sarcoidosis, and the current therapeutic approaches used to treat the disease.

scholarly journals Langerhans-Cell Histiocytoses - Epidemiology, Classification, Clinical Features, Diagnosis, Complications, Treatment and Prognosis

2016 ◽  
Vol 9 (1) ◽  
pp. 3-16 ◽  
Author(s):  
Vera E. Papochieva ◽  
Dimitrinka S. Miteva ◽  
Penka I. Perenovska ◽  
Guergana Petrova
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
Multisystem Disease ◽  
The Past ◽  
System Disease ◽  
Young Smokers ◽  
Underlying Pathology

Summary Histiocytoses comprise a group of diverse diseases of unknown etiology with various clinical presentation and evolution. The underlying pathology is characterised by accumulation and infiltration of variable numbers of cells of the monocyte-macrophage line in the affected tissues and organs. Histiocytoses are divided into three major classes: Langerhans cell histiocytosis (LCH), non- Langerhans cell histiocytosis, and malignant histiocytic disorders. The term LCH (also known in the past as histiocytosis X) encompasses the following rare diseases: Eosinophilic Granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, in which accumulation of pathologic Langerhans cells (LCs) leads to tissue damage. LCs usually reside in the skin and ensure protection against infections by destroying foreign substances. LC accumulation is caused by antigen stimulation and inadequate immune response. Thus, clinical LCH manifestations range from isolated disease with mono- or multifocal bone lesions to disseminated multisystem disease. LCH is a rare disease, affecting mainly children and young smokers, aged 20-50 years. Lung involvement in LCH usually presents as a mono-system disease and is characterized by Langerhans cell granulomas (LCG) infiltrating and impairing the distal bronchioles. The definite diagnosis is based on lung biopsy of CAT selected LCG areas. So far, there is no an effective treatment, but the better understanding of the mechanisms involved in the pathogenesis of the disease would help in the development of effective therapeutic strategies in the future.

Progressive polyepiphyseal dysplasia with arthropathy: A distinct disorder from idiopathic juvenile arthritis and pseudorheumatoid dysplasia?

2012 ◽  
Vol 158A (7) ◽  
pp. 1754-1758 ◽  
Author(s):  
Bertrand Isidor ◽  
Sylvaine Poignant ◽  
Georges Picherot ◽  
André Mégabarné ◽  
Pierre Quartier ◽  
...  
Keyword(s):  
Juvenile Arthritis ◽  
Idiopathic Juvenile Arthritis

scholarly journals Primary biliary cirrhosis and hepatic sarcoidosis: A case report

2014 ◽  
Vol 71 (1) ◽  
pp. 83-86 ◽  
Author(s):  
Tamara Alempijevic ◽  
Aleksandra Sokic-Milutinovic ◽  
Ljubisa Toncev ◽  
Aleksandra Pavlovic-Markovic ◽  
Srdjan Djuranovic ◽  
...  
Keyword(s):  
Case Report ◽  
Primary Biliary Cirrhosis ◽  
Liver Function ◽  
Elevated Serum ◽  
Liver Function Tests ◽  
Unknown Etiology ◽  
Biliary Cirrhosis ◽  
Laboratory Findings ◽  
Function Tests ◽  
Elevated Liver Function Tests

Introduction. Primary biliary cirrhosis (PBC) is an immunemediated chronic progressive inflammatory liver disease leading to destruction of small interlobular bile ducts. Sarcoidosis is a chronic disorder of unknown etiology characterized by non-caseous granulomas. Case report. We reported a 69-year-old female patient with abdominal pain, malaise, vertigo, headaches, hands tremor and partial loss of hearing. Initial laboratory findings revealed elevated liver function tests and cholesterol with positive antimytochondrial and antinuclear antibodies. Liver biopsy revealed granuloma typical for PBC and granulomatous lesions typical for sarcoidosis. Elevated serum angiotensin-converting enzyme and granulomatous lesion on the brain magnetic resonance imaging (MRI) were detected and the patient was diagnosed with overlap of PBC and liver sarcoidosis and neurosarcoidosis. The patient was treated with ursodeoxicholic acid (UDCA) and prednisolone. Six months later the patient was symptom-free with laboratory findings within normal range. Conclusion. In PBC patients it is important to consider coexisting granulomatous liver diseases if elevated liver function tests persist despite UDCA therapy.

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