Neurosarcoidosis in a 67-Year-Old Male Without Pulmonary Involvement: A Case Report.

Neurosarcoidosis is a rare and complex form of sarcoidosis that affects the nervous system, presenting significant diagnostic and therapeutic challenges due to its varied neurological symptoms and potential complications. We present a case of a 67-year-old immunocompetent male who presented with altered sensorium, prompting a thorough evaluation. His medical history revealed intermittent low-grade fevers, progressive weight loss, and frailty, rendering him bedridden for over a year. Previous blood tests had ruled out specific etiologies, with normal serum calcium and angiotensin-converting enzyme (ACE) levels. Upon presentation, further diagnostic workup included imaging and laboratory tests. Results showed elevated serum calcium, increased ACE levels, and depressed intact parathyroid hormone levels. MRI of the brain with contrast revealed lobulated mucosal thickening in the right sphenoid sinus and adjacent anterior cavernous sinus. A CT scan of the chest was unremarkable. Additionally, a splenic biopsy revealed hypoechoic foci with neutrophilic, lymphocytic, and histiocytic granulomas. Based on imaging and histopathological findings, the patient was diagnosed with neurosarcoidosis. The patient was treated with prednisolone and methotrexate, leading to a prompt improvement in consciousness and symptoms. Follow-up demonstrated continued improvement and stabilization of his condition. This case highlights the importance of considering neurosarcoidosis in patients with unexplained neurological symptoms and underscores the value of a multidisciplinary approach in managing this challenging condition.

Granulomatous mastitis following stereotactic core-needle biopsy: A case report.

Idiopathic granulomatous mastitis is a rare, chronic inflammatory disease of the breast of uncertain etiology that can mimic breast cancer. In rare instances, it may emerge secondary to trauma to the breast. We present a case of a 66-year-old woman who initially underwent a benign stereotactic core-needle biopsy of her left breast complicated by a small hematoma which initially remained unchanged mammographically and sonographically for 1 year; then, it enlarged unexpectedly at the 21-month interval follow-up prompting an ultrasound-guided biopsy revealing granulomatous mastitis.

Current approaches to diagnosing and treating idiopathic granulomatous mastitis: A summary from in-depth clinician interviews.

Background: Idiopathic granulomatous mastitis (IGM) is a rare, chronic inflammatory breast condition primarily affecting women of reproductive age. Its diagnosis is challenging due to similarities with other breast disorders, necessitating exclusion of other granulomatous diseases. The management of IGM remains inconsistent and unclear, with high recurrence rates and varying practices. Methods: This qualitative study involved semi-structured interviews with nine clinicians from Singapore, Malaysia, and Egypt to examine current diagnostic and therapeutic approaches for IGM. Transcripts were analysed using NVivo software for coding and summarisation. Findings: Clinicians predominantly used imaging and histopathology for diagnosis. Treatment commonly involved corticosteroids, though dosages and tapering regimens varied widely. Methotrexate was used sparingly for refractory cases due to associated risks. Surgical interventions were infrequent, reflecting a preference for medical management. There was a consensus on the need for randomised controlled trials (RCTs) to establish standardised treatment protocols. Interpretation: This study reveals the complex nature of IGM diagnosis and treatment from clinicians in Singapore, Malaysia and Egypt. This underscores the need for more specific and definitive diagnostic tests, rather than relying on exclusionary methods, and standardised treatment guidelines. Multi-centre RCTs are essential for developing evidence-based protocols to improve patient outcomes and address regional differences effectively.

Rare Case of Laryngeal Histoplasmosis: A Diagnostic Challenge.

Granulomatous lesions, though rare, can affect diverse body regions, including the larynx, presenting diagnostic challenges. In India, where malignancy and tuberculosis prevail, fungal infections are often overlooked. We present a case of 34 year old male with complaint of change in voice which was approached as malignancy but later turned out to be histoplasmosis on histopathology. Diagnostic challenges arise due to symptoms overlapping with other conditions. Our case underscores the importance of considering fungal etiologies in endemic regions, especially when symptoms persist despite treatment.

Value of contrast-enhanced ultrasound in differentiating granulomatous mastitis from invasive ductal carcinoma.

OBJECTIVE: This study aims to analyze the imaging manifestations of granulomatous mastitis (GM) and invasive ductal carcinoma (IDC) using conventional ultrasound (US) and contrast-enhanced ultrasound (CEUS). The objective is to investigate the clinical value of CEUS in differentiating between GM and IDC. METHODS: We retrospectively enrolled 39 GM patients and 64 IDC patients between January 2020 and June 2023. All diagnoses were confirmed via core needle biopsy or surgical pathology. The characteristics of both conventional US and CEUS in these patients were analyzed to distinguish GM from IDC. RESULTS: Based on CEUS features, GM lesions most commonly presented as hypoechoic areas (43.6%), followed by pseudocysts (28.2%), hypoechoic nodules (15.4%), and honeycomb cysts (12.8%). The diffuse enhancement pattern was an independent characteristic for distinguishing GM from IDC, with the ROC analysis revealing an area under the curve (AUC) value of 0.794. CONCLUSION: US is the preferred initial examination for GM, and both its conventional and CEUS features can enhance diagnostic accuracy and guide clinical treatment. CEUS demonstrates high differential diagnostic value in distinguishing GM from IDC. ADVANCE IN KNOWLEDGE: This study categorizes GM manifestations on CEUS into four types, each corresponding to different pathological stages of GM. We identified that the diffuse enhancement pattern on CEUS is a distinctive characteristic associated with GM, aiding in its differentiation from IDC.

Multiparameter magnetic resonance imaging features of prostatic malakoplakia.

OBJECTIVE: To summarize the multiparameter magnetic resonance imaging (mpMRI) features of prostatic malakoplakia. METHODS: Eleven patients (median age 62 years; IQR 59-71 years) with pathologically confirmed prostatic malakoplakia were included in this study. MpMRI features of the lesions were retrospectively reviewed and summarized. RESULTS: The Prostate Imaging-Reporting and Data System (PI-RADS) scores of the patients were 4 or 5, with a medium diameter of 3.7 cm (IQR 2.0-5.2 cm). All the lesions involved the peripheral zone, of which 3 cases (3/11, 27.3%) involved the transition zone simultaneously. A diffuse symmetrical distribution was found in 5 cases, a focal distribution in 5 cases, and a focal symmetrical distribution in 1 case. Both the surgical capsule and prostatic margin were bulged and compressed, but no signs of infiltration were found. Nearly all the lesions had moderate hypointensity on T2-weighted images and slightly higher signal intensity on T1-weighted images. Among the lesions, early enhancement occurred in all cases, prolonged enhancement occurred in 8 cases, and ring enhancement persisted in 1 case. All lesions displayed periprostatic enhancement in the delayed phase. CONCLUSIONS: Lesions distributed diffusely and symmetrically in the peripheral zone with preserved surgical capsule and margin are characteristic features of malakoplakia as opposed to cancer, especially in those with slightly high T1 signal intensity and delayed periprostatic enhancement. Those locally distributed lesions with the above signs should also be suspected. ADVANCES IN KNOWLEDGE: Prostatic malakoplakia are more likely to distribute diffusely and symmetrically, with slightly high T1 signal intensity and delayed periprostatic enhancement.

Incidence of an Identifiable Organism in Children Who Underwent a Surgical Procedure for Granulomatous Cervical Lymphadenopathy.

Objectives: The incidence of cervical lymphadenopathy due to nontuberculous mycobacteria is rising in the pediatric population. Our goal with this study was to review the number of pediatric patients with granulomatous cervical adenitis and determine the incidence of identification of a specific organism as both healthcare providers and parents are interested in identifying the causative pathogen. Methods: A retrospective chart review was conducted of patients at a high-volume tertiary care children's hospital between 2017 and 2023. Children were included if they underwent a surgical procedure for lymphadenopathy. Pathology, microbiology, and other laboratory reports were reviewed to document the presence of granulomatous cervical adenitis and the incidence of identification of a specific organism. Additional data collected included patient demographics and type of procedure. Results: Of the 1538 charts reviewed, 163 patients underwent an inclusionary procedure. Mean patient age was 10.7 years (range 2.4 months-20 years), 70 (43%) were female, 25 (15%) had granulomatous cervical adenitis, and a specific organism was identified in 9 of these. Conclusion: Despite the availability of a number of ancillary tests, our data demonstrate that the identification of a specific pathogen in cases of granulomatous cervical lymphadenitis is rare. As a result, physicians should be prepared to rely primarily on the history and physical exam findings to determine a working diagnosis as well as a medical and/or surgical treatment plan.

Unusual coexistence of low-grade mucinous cystic neoplasm and idiopathic granulomatous hepatitis.

INTRODUCTION AND IMPORTANCE: Mucinous cystic neoplasms (MCNs) of the liver are rare precancerous lesions, accounting for less than 5 % of all hepatic cysts. The coexistence of MCNs with idiopathic granulomatous hepatitis is exceedingly uncommon and lacks documentation in the existing literature. CASE PRESENTATION: A 43-year-old Tunisian woman with no significant medical history presented with persistent right hypochondrium pain. Clinical examination revealed a palpable mass in the right hypochondrium. Laboratory tests indicated elevated liver enzymes and cholestasis. Imaging studies identified an 18 × 11 cm cystic formation in the right liver lobe, initially suspected to be a type I hydatid cyst. The patient underwent a right hepatectomy, and histological examination confirmed the presence of a low-grade MCN of the liver coexisting with idiopathic granulomatous hepatitis. The patient experienced an uncomplicated postoperative recovery. CLINICAL DISCUSSION: In our case, the concomitant presence of MCN and idiopathic granulomatous hepatitis was incidental, without any causal link. The definitive diagnosis of these two conditions relies on histopathological examination. It is essential to surgically remove the MCN and identify the cause of granulomatous hepatitis to effectively manage the patient. CONCLUSION: This case underscores the uncommon occurrence of both MCN and idiopathic granulomatous hepatitis in the liver, highlighting the diagnostic challenges associated with the latter. Accurate diagnosis through thorough evaluation is essential for effective management.

A case report navigating CVID and sarcoidosis overlaps in pediatric nephritis.

Common variable immunodeficiency (CVID) can be complicated by granulomatous disease, often granulomatous lymphocytic interstitial lung disease (GLILD). Granulomatous interstitial nephritis represents an atypical presentation in pediatrics. Our patient is a previously healthy 13-year-old white male with a recent diagnosis of CVID. He presented with a rash and laboratory findings included pancytopenia (white blood cells 2.6 cells × 103/µl, hemoglobin 11.8 g/dl, platelets 60 × 103/µl), hypercalcemia (14.9 mg/dl), elevated Vit D 1,25 OH level (>200 pg/ml), hyperuricemia (8.8 mg/dl), and acute kidney injury (AKI) (serum creatinine 1.1 mg/dl; baseline 0.64 mg/dl). A broad infectious workup was unremarkable. The rash improved with empiric doxycycline. Hypercalcemia and hyperuricemia were managed with fluid resuscitation, calcitonin, and zoledronic acid. Evaluation for malignancy including a positron emission tomography scan, revealed multiple mediastinal hypermetabolic lymph nodes and pulmonary ground glass opacities, later reported as small pulmonary nodules by computed tomography (CT). Splenomegaly was confirmed by ultrasound and CT. Peripheral smear, bone marrow biopsy, and genetic testing were non-revealing. His angiotensin-converting enzyme level was elevated (359 U/L), raising concerns for sarcoidosis. Given Stage 1 AKI, a renal biopsy was pursued and identified non-caseating granulomatous interstitial nephritis. Treatment with 60 mg of prednisone began for presumed sarcoidosis for 4 months, causing steroid-induced hypertension and mood changes. Zoledronic acid minimally reduced serum creatinine. Pneumocystis jirovecii pneumonia prophylaxis was initiated due to T-cell cytopenia. Chest CT findings showed a suboptimal response to steroids. A bronchoalveolar lavage demonstrated >50% lymphocytes (normal <10%) and the lung biopsy exhibited non-caseating granulomas, indicating GLILD. Rubella was identified by staining. Following a fever, he was found to have elevated liver enzymes and confirmed hepatitis with portal hypertension on CT. A liver biopsy revealed epithelioid non-caseating granuloma and HHV6 was detected by PCR. He was treated with four cycles of rituximab and granulocyte-colony stimulating factor for persistent neutropenia. Subsequent treatment with mycophenolate led to the resolution of the granulomatous lesions and cytopenias. The rare complication of granulomatous interstitial nephritis in CVID illustrates the intricate nature of diagnosis. This case underscores the necessity for a holistic view of the patient's clinical and immune phenotype, including distinctive radiological presentations, for precise diagnoses and tailored management of CVID.

Extensive Peritoneal and Pelvic Granulomatous Inflammation Due to Syphilis Infection Manifesting as Primary Infertility: A Case Report.

Infertility affects millions of couples worldwide and can result from various factors, including sexually transmitted infections. Although syphilis is known to contribute to a small number of infertility cases through chronic pelvic inflammatory disease, which ultimately impairs fertility, detailed descriptions of such cases are limited. In this report, we present a case of primary infertility caused by extensive peritoneal granulomatous inflammation, adhesions, and tubal obstruction resulting from syphilis.

Case report: granulomatous hepatitis due to Mycobacterium avium in an Atlantic yellow-nosed albatross (Thalassarche chlororhynchos) from Southern Brazilian coast.

This study reports a granulomatous hepatitis caused by Mycobacterium avium in an Atlantic yellow-nosed albatross (Thalassarche chlororhynchos) found dead on Brazil southern coast. At necropsy, the albatross was cachectic and the liver was severely enlarged with multifocal to coalescing white nodules. Histopathological evaluation revealed multifocal to coalescing granulomas with caseous necrosis, surrounded by an infiltrate of macrophages and multinucleated giant cells, and a thin capsule of fibrous connective tissue. The Fite-Faraco staining technique revealed multiple acid-fast bacilli (AFB) stained in magenta, predominantly in the areas of necrosis. Bacterial culture and polymerase chain reaction (PCR) analysis confirmed the presence of Mycobacterium avium in liver samples. This case underscores the importance of wildlife surveillance in coastal regions. Pelagic birds like the Atlantic yellow-nosed albatross can harbor pathogenic agents that represent a threat to wildlife and domestic animals. Enhanced monitoring and research are essential to understand the epidemiology and potential risks associated with such infections in coastal ecosystems.

Sarcoidosis and Cancer: The Role of the Granulomatous Reaction as a Double-Edged Sword.

Background/Objectives: The relationship between sarcoidosis and the occurrence of neoplasia deserves to be investigated, but this relation has been observed in different and heterogeneous populations, leading to conflicting data. To clarify the causal relationship between these two diseases, different risk factors (e.g., smoking), concurrent comorbidities, corticosteroid therapy, and metastasis development-as an expression of cancer aggressiveness-were investigated. Methods: In a retrospective study on 287 sarcoidosis outpatients at the Pneumological Department of the Gemelli Foundation (Rome, Italy) between 2000 and 2024, the diagnosis of cancer was recorded in 36 subjects (12.5%). Results: The reciprocal timeline of the diseases showed three different scenarios: (1) cancer preceding sarcoidosis or sarcoid-like reactions (63.8%); (2) cancer arising after sarcoidosis diagnosis (8.3%); and (3) sarcoidosis accompanying the onset of malignancy (27.8%). Only two subjects with sarcoidosis and cancer showed metastasis, and one of them was affected by lymphoma. Conclusions: These data suggest that granulomatous inflammation due to sarcoidosis may assume an ambivalent role as a "double-edged sword", according to the M1/M2 macrophage polarization model: it represents a protective shield, preventing the formation of metastasis through the induction of immune surveillance against cancer while, on the other hand, it can be a risk factor for carcinogenesis due to the persistence of a chronic active inflammatory status. Low-dose steroid treatment was administered in only 31.6% of the cancer-sarcoidosis subjects for less than six months to control inflammation activity, with no promotive effect on carcinogenesis observed.

>Efficacy of disease-modifying antirheumatic drugs in the treatment of granulomatous mastitis: a systematic review.

INTRODUCTION: Idiopathic granulomatous mastitis (IGM) is an inflammatory breast disorder of unknown etiology. This benign condition can mimic the clinical presentation of breast cancer and is characterized by symptoms such as breast pain, erythema, and swelling. Over the past few years, Disease-Modifying Antirheumatic Drugs (DMARDs) have been increasingly used to manage this condition. However, strong evidence to support their use is lacking. OBJECTIVES: This systematic review aimed to summarize the evidence and evaluate the efficacy of DMARDs in the management of IGM. METHODS: A systematic literature review, adhering to PRISMA guidelines, was conducted across electronic databases, including PubMed, EMBASE, SCOPUS, directory of open access journals (DOAJ) and Cochrane Library from their inception until May 2024. We included retrospective and prospective studies while excluding case reports and case series of less than 10 patients. RESULTS: Eighteen studies met our eligibility criteria. Fifteen studies were retrospective, while 2 were prospective. No randomized controlled trials were identified. Of these, 16 papers examined the effect of methotrexate on IGM, revealing significant disease improvement in most cases. Several of the studies indicated that patients treated with azathioprine and mycophenolate mofetil also achieved favorable responses. CONCLUSION: Given the rarity of IGM, only a limited number of studies have explored the use of DMARDs as a pharmacological treatment option. A significant barrier to advancing our understanding is the substantial heterogeneity in the quality and volume of data provided by these studies. Therefore, there is a need for well-designed, randomized, placebo-controlled trials to rigorously assess the efficacy of DMARDs in the treatment of IGM.

Neurosarcoidosis: Clinical, biological, and MRI presentation of central nervous system disease in a national multicenter cohort.

INTRODUCTION: Neurosarcoidosis (NS) is a systemic inflammatory granulomatous disease affecting of patients with sarcoidosis. Its diagnosis is difficult as there is no specific test for it. Because of its rarity, the management of NS has so far only been described in case series and short retrospective cohorts. The objective of this study is description of the clinical, paraclinical presentation and the therapeutic management of central nervous system (CNS) involvement in NS patients in France. METHODS: This multicenter, retrospective, observational study involved patients hospitalized between 2010 and 2019 with a diagnosis of sarcoidosis and CNS involvement. RESULTS: We included 118 patients (38 with isolated NS, 80 with NS associated with systemic sarcoidosis). NS was the initial presentation in 78% of patients, with cranial nerve involvement (36%), medullary symptoms (23%), and seizures (21%). Twenty-one percent of the patients had already been diagnosed with systemic sarcoidosis. The most frequent biological abnormality was lymphopenia (62.5%), while angiotensin-converting enzyme was increased in 21%. Meningitis was present in 45% and hyperproteinorachia in 69.5% of cases. MRI mainly revealed white matter abnormalities and leptomeningeal enhancement (34%). Corticosteroids were the most useful treatment, and immunosuppressive agents were used in steroid-resistant patients and to limit side effects. Methotrexate, cyclophosphamide, and anti-TNFα were also used, exhibiting good efficacy. CONCLUSIONS: This cohort contributes to a better understanding of the clinical phenotype and associated imaging and biological abnormalities. Sharing of clinical, biological, and imaging data, as well as the therapeutic responses, of patients with NS helps to better understand and manage this disease that affects a small number of patients per center. A database project could be implemented in the future to enable this.

An Atypical Presentation of a Patient With Neurosarcoidosis: A Case Report.

Neurosarcoidosis is a disease in which noncaseating granulomas, characteristic of sarcoidosis, are found within organs of the nervous system such as the brain and spinal cord. This case report highlights a 57-year-old male with worsening bilateral lower extremity weakness and numbness in addition to ptosis and oculomotor nerve palsy of the right eye. Computed tomography (CT) imaging showed mediastinal and hilar lymphadenopathy, which raised suspicion for neurosarcoidosis. Multiple biopsies were taken from lymph nodes in the mediastinal region, which resulted in non-necrotizing epithelioid cell granulomas, consistent with the suspected neurosarcoidosis. Medical providers must include neurosarcoidosis within a much broader differential diagnosis when encountering patients that present with a similar presentation shown in this case report so that treatment can be promptly initiated as soon as possible.

Severe Clinical Phenotypes of Heterozygous Females With X-Linked Chronic Granulomatous Disease.

CLINICAL IMPLICATIONS: Female X-linked chronic granulomatous disease (XL-CGD) carriers may develop severe clinical disease including infections with CGD-defining pathogens and inflammatory disorders. Similar to males with XL-CGD, female carriers warrant ongoing evaluation and prophylaxis where indicated.

Cornus Officinalis Total Glycosides Alleviate Granulomatous Lobular Mastitis via the B7-CD28/CTLA-4 Costimulatory Pathway.

Cornus officinalis total glycosides (COTG) derived from the traditional Chinese medicine Cornus officinalis, is a natural immunosuppressant and has been extensively studied in immunomodulation and immunosuppression. This study aimed to explore the effects of COTG on granulomatous lobular mastitis (GLM) and its associated mechanisms. Compared to the model group, COTG effectively ameliorated histopathological damage to breast tissue, reduced mammary gland suppuration, and enhanced the blood-milk barrier. Additionally, COTG treatment reduced the total number of T cells and B cells in GLM rats, significantly improving clinical indicators such as P-selectin, E-selectin, and intercellular cell adhesion molecule-1. We also observed downregulation of CD28 and B7 expression levels, an upregulation of cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) expression, and a significant decrease in inflammatory marker levels in the COTG group. COTG exerts an anti-inflammatory effect in GLM by stimulating CTLA-4, inhibiting the B7-CD28 signaling pathway affecting T cell activation, and promoting the blood-milk barrier. These findings suggest that COTG could be a promising therapeutic option for managing GLM, potentially improving patient outcomes by modulating immune responses and reinforcing the blood-milk barrier.

Pulmonary actinomycosis misdiagnosed as lung cancer: a case report.

Pulmonary actinomycosis is a rare pulmonary infectious disease that is often challenging to diagnose early and has a high misdiagnosis rate. In some cases, it can be particularly difficult to distinguish pulmonary actinomycosis from lung cancer. We herein report a rare case of pulmonary actinomycosis in which the preoperative examinations strongly suggested lung cancer, leading to the patient undergoing right upper lung resection and bronchoplasty. The patient had a good postoperative recovery; however, the postoperative pathology report indicated pulmonary actinomycosis. In this report, we summarize the key aspects of the diagnosis and treatment of pulmonary actinomycosis to aid clinicians in reducing the likelihood of misdiagnosis.

The Use of Intralesional Corticosteroids in Idiopathic Granulomatous Mastitis: A Systematic Review.

Idiopathic granulomatous mastitis (IGM) is a debilitating, chronic, inflammatory condition of the breast. Several studies have emerged evaluating intralesional steroid (ILS) injection and topical steroid administration as a treatment for IGM. However, there is a dearth of international consensuses with regards to the management of IGM. Therefore, we have systematically reviewed the effectiveness of ILS in the management of IGM. A systematic search was conducted in PubMed and Cochrane Library databases, the Google Scholar website and by citation searching up to June 15th, 2023. Eight articles were selected and analyzed. A total of 397 IGM patients were included in the review. The mean patient age was 35.7 years, ranging from 23-62 years. The mean pre-treatment diameter of lesions was 27.5 mm. A total of 184 patients were treated with ILS. The mean complete clinical response time was 2.6 months. The overall complete response rate was 92.8%. Complications following ILS were minor, with hematoma, skin atrophy and hyperemia being commonly described, while avoiding the systemic side effects of oral steroid use, such as weight gain and hirsutism, which were the most commonly reported side effects with oral steroids. The recurrence rates in the ILS group (6.6%) appear to be lower than in the oral steroid group (25.8%) and surgery group (26.3%). ILS seem to show a favorable outcome in terms of complete response rate, complete clinical response time and has a lower recurrence rate and complication rate when compared to other intervention strategies. However, more comparative studies with standardized protocols are necessary to ascertain the optimum type, dosage and frequency of ILS regimens.