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1.
Heliyon ; 10(19): e38345, 2024 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-39386822

RESUMEN

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.

2.
Int J Surg Case Rep ; 124: 110367, 2024 Sep 30.
Artículo en Inglés | MEDLINE | ID: mdl-39353310

RESUMEN

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.

3.
Ear Nose Throat J ; : 1455613241288469, 2024 Oct 05.
Artículo en Inglés | MEDLINE | ID: mdl-39367731

RESUMEN

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.

4.
Front Pediatr ; 12: 1417724, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39359743

RESUMEN

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.

5.
Indian J Otolaryngol Head Neck Surg ; 76(5): 4788-4793, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39376366

RESUMEN

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.

6.
Br J Radiol ; 2024 Oct 08.
Artículo en Inglés | MEDLINE | ID: mdl-39378121

RESUMEN

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.

7.
Br J Radiol ; 2024 Oct 08.
Artículo en Inglés | MEDLINE | ID: mdl-39378132

RESUMEN

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.

8.
Cureus ; 16(10): e70764, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39380781

RESUMEN

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.

9.
Cureus ; 16(9): e69077, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39391405

RESUMEN

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.

10.
J Clin Imaging Sci ; 14: 39, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39391572

RESUMEN

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.

11.
Clin Case Rep ; 12(9): e9370, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39219778

RESUMEN

Key Clinical Message: Pulsed dye laser (PDL) has proven effective in resolving lupus miliaris disseminatus faciei (LMDF) where drug therapies have failed with a lack of treatment consensus for LMDF, considering early PDL intervention is crucial to achieve resolution without scarring, prevent relapse, and enhance overall treatment outcomes. Abstract: Lupus miliaris disseminatus faciei (LMDF) is a rare inflammatory and granulomatous dermatologic disease that primarily affects the face. The optimal treatment for LMDF remains controversial, and there is a lack of consensus on the most effective therapy. This case report highlights the successful use of a 595 nm pulsed dye laser (PDL) in the treatment of LMDF following unsuccessful drug therapy. A 28-year-old male presented with reddish-brown eruptions on his face that had persisted for several months. Clinical examination revealed discrete dome-shaped eruptions in clusters on the central area of the face. Histopathological examination confirmed the diagnosis of LMDF, based on the presence of epithelioid granulomas with central caseous necrosis. Previous treatment with an oral isotretinoin and methotrexate combination also failed to yield satisfactory results. After discontinuing drug therapy, the patient underwent five sessions of PDL treatment. Ten days after the first session, the eruptions began to regress without scarring. Subsequent PDL sessions led to the complete resolution of the eruptions. The patient experienced no relapse during the follow-up period. This case report suggests that PDL treatment may be an effective option for LMDF, particularly in cases where drug therapy has failed. Early initiation of laser treatment may prevent scarring, minimize the adverse effects associated with drug therapy, and reduce the risk of disease relapse. Further research and controlled trials are needed to establish the efficacy of laser therapy in the treatment of LMDF.

12.
J Breast Imaging ; 2024 Sep 04.
Artículo en Inglés | MEDLINE | ID: mdl-39228113

RESUMEN

OBJECTIVE: We describe the demographics, clinical presentation, imaging findings, and treatment response among 235 cases of biopsy-proven idiopathic granulomatous mastitis (IGM) at a single institution. METHODS: An institutional review board-approved retrospective search of the breast imaging database was performed to select patients with biopsy-proven IGM between 2017 and 2022. Retrospective review evaluated clinical presentation, imaging findings with US and mammography, and treatment recommendations (antibiotics, nonsteroidal anti-inflammatory drugs [NSAIDs], warm compresses, or observation only). Response to treatment was evaluated on follow-up US. A favorable treatment response was a decrease in size or resolution of disease on follow-up imaging. Statistical analysis using Poisson regression was performed to evaluate the clinical outcomes associated with each treatment. RESULTS: A total of 235 patients met the selection criteria with a mean age of 38 years (18 to 68). The majority of patients were Hispanic (95%, 223/235). Of all patients, 75.3% (177/235) received treatment (consisting of 1 or any combination of antibiotics, NSAIDs, warm compresses), 24.7% (58/235) were treated with observation, 78.7% (185/235) returned for follow-up imaging, and 21.3% (50/235) were lost to follow-up. Of those with follow-up imaging, disease improvement was seen in 70.3% (102/145) of patients who received treatment compared with 72.5% (29/40) of patients treated by observation alone. Multivariate analysis further showed no difference in clinical outcomes among the treatment of unifocal, multifocal, or recurrent IGM. CONCLUSION: Nonsteroidal treatment of IGM showed no significant improvement on follow-up imaging compared to treatment with observation alone in a predominantly Hispanic patient population.

13.
J Clin Med ; 13(17)2024 Sep 04.
Artículo en Inglés | MEDLINE | ID: mdl-39274446

RESUMEN

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.

14.
Radiol Case Rep ; 19(11): 4747-4750, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39228927

RESUMEN

The case presented illustrates a very rare chronic granulomatous disease called the Madura foot or also called mycetoma. It is a chronic granulomatous disease, caused by a contained infection that penetrates the subcutaneous tissues and spreads to the nearby bone by damaging the affected area's vascularization. Early diagnosis is greatly aided by clinical history and imaging, especially if the "dot-in-circle" sign-a pathognomonic marker of mycetoma-is seen on the MRI. Compared to ordinary radiographs, computed tomography offers a more accurate study of changes in the bone. In order to apply the appropriate course of treatment, it is critical to identify the causal species, which can be caused by either a true fungus (eumycetoma) or a bacterium Actinomyces (Actinomycetoma). Treatment can be extremely challenging and can even result in amputation, particularly if the diagnosis is delayed.

15.
Eur J Breast Health ; 20(3): 228-230, 2024 07 01.
Artículo en Inglés | MEDLINE | ID: mdl-39257016

RESUMEN

Systemic lupus erythematosus (SLE) and sarcoidosis are two of the most well-recognized, chronically diagnosed conditions in the United States, with a plethora of known multisystem manifestations. With regard to breast pathology, lupus mastitis is a relatively uncommon manifestation of SLE, commonly involving both the mammary gland and subcutaneous soft tissues of the breast. Sarcoidosis in the breast is a similarly, exceedingly rare manifestation of this multi-system disorder, classically presenting with non-caseating granulomas. Both present with non-specific mammographic and sonographic features. We present a 62-year-old female with known diagnosis of discoid lupus and Graves' disease who presented initially with an abnormal screening mammogram, ultimately undergoing mammographic work-up and subsequent biopsy demonstrating lupus mastitis, including vasculitis, panniculitis, and fibrosis with chronic inflammation. The patient was also found to have small non-caseating granulomas, some in a perivascular distribution, classically seen in sarcoidosis. Given the rarity of both manifestations, our case explores the coexistence of these autoimmune processes and this atypical presentation.

16.
Lung Cancer (Auckl) ; 15: 135-142, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39224877

RESUMEN

Background: Pulmonary spindle cell carcinoma (PSCC), a highly malignant tumor, often exhibits cell pleomorphism, a histopathological characteristic. Owing to its extremely low incidence, atypical imaging and clinical presentations, and insufficient awareness among clinicians, PSCC is often misdiagnosed, which results in delays in treatment. Herein, we reported a rare case of PSCC that was initially misdiagnosed as granulomatous inflammation. Case Presentation: A 66-year-old male visited a local hospital with symptoms such as cough and hemoptysis. A computed tomography (CT) scan of the chest revealed a mass in his right lung, and no mediastinal lymphadenopathy was observed. Bronchoscopy showed no major abnormalities, and the results of fine needle aspiration biopsy showed granulomatous inflammation. Even though the patient received anti-infection treatment, his symptoms did not improve markedly. After two months, a follow-up CT scan of the lung showed a noticeably enlarged mass accompanied by multiple instances of mediastinal lymphadenopathy in the upper lobe of the right lung. Consequently, he underwent a second CT-guided lung biopsy at our hospital. The pathology report indicated PSCC. Due to financial constraints, genetic testing was not performed. Given his poor overall physical condition, the patient was unable to undergo systemic chemotherapy and instead received palliative radiotherapy. The prescribed radiotherapy dose for the right upper lobe lung cancer and multiple metastatic lymph nodes was 60 Gy, administered in 30 fractions. Unfortunately, he failed to adhere to scheduled follow-ups and succumbed to the disease 6 months later, as confirmed during a telephone follow-up. Conclusion: PSCC is a rare but highly malignant lung cancer. Multiple pathological biopsies are necessary to accurately and promptly diagnose the disease, which is crucial for early treatment intervention as well as improving patient prognosis.

17.
Cureus ; 16(8): e65935, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39229421

RESUMEN

We discuss the case of a 60-year-old male who presented with ankle pain, a necrotic rash, and progressive weakness in both lower limbs and the right upper limb. An infectious workup of the skin lesions came back negative. Additionally, his kidney function tests indicated an acute kidney injury. This prompted investigations for vasculitis etiologies, which revealed a positive cytoplasmic antineutrophil cytoplasmic autoantibody (c-ANCA). His neurological deficits were also investigated, and imaging suggested embolic infarcts. Cardiac imaging showed valve vegetations and blood culture showed a lack of growth suggestive of a noninfective nature of these lesions. Based on all these findings, a kidney biopsy was obtained and demonstrated pauci-immune segmental vasculitis consistent with ANCA-associated glomerulonephritis. As such, the patient showed improvement with heavy pulse steroid and immunomodulator therapy. Although skin, heart, and CNS involvement have been previously reported with ANCA-associated vasculitis, it is rare, especially together, and can prove a diagnostic challenge. Therefore, it is important to consider vasculitis etiology in patients presenting similarly. In addition, this case highlights the overlapping clinical picture between infective endocarditis and vasculitis with valvular involvement, making differentiation between the two challenging.

18.
Cureus ; 16(8): e66412, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39246938

RESUMEN

INTRODUCTION: Granulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is an autoimmune disease marked by necrotizing granulomatous inflammation and vasculitis affecting small-sized vessels. It commonly impacts the renal and respiratory systems. MATERIALS AND METHODS: This retrospective case series sampling conducted in a tertiary care hospital between May 2023 and April 2024 examined six newly diagnosed GPA patients who were proteinase 3 cytoplasmic-antinuclear cytoplasmic antibody (PR3 c-ANCA) positive and had concurrent respiratory infections. None of them had any prior immunosuppressive conditions. The age range was 18-47 years with a mean of 35.0 (standard deviation: 11.83). All the patients had pneumonia (N=6, 100%). Out of all, five had bacterial pneumonia (N=5, 83.3%) and one had tuberculous pneumonia (N=1, 16.7%). A high level of PR3 c-ANCA (>150 RU/mL) was noted in four patients (N=4, 66.7%). Common symptoms included dry cough (N=5, 83.3%), loss of weight and appetite (N=2, 33.3%), and fever (N=2, 33.3%). Three patients had otitis media and/or nasal polyposis (N=3, 50%). Two patients (N=2, 33.3%) with life-threatening organ dysfunction were given concurrent antibiotics and steroids; the antibiotics were later modified based on culture and sensitivity results. One of these patients received antituberculosis therapy as Mycobacterium tuberculosis (MTB) was detected after 27 days of incubation in mycobacterial growth indicator tube broth. The remaining four patients (N=4, 66.7%) received antibiotics initially for 5-7 days until clinical resolution of pneumonia. Ultimately, they all showed clinical and radiological resolution (N=6, 100%) within 3-6 months of treatment. RESULTS: The patients exhibited constitutional symptoms such as fever and weight loss; lower airway disease symptoms including dry cough and hemoptysis; nasal and ear disease symptoms like epistaxis, ear pain, and ear discharge; and a renal disease symptom, hematuria. Computed tomography of the thorax revealed bilateral consolidations, most of which were cavitating. Bronchoalveolar lavage cultures grew Escherichia coli, Burkholderia cepacia, Pseudomonas aeruginosa, Klebsiella pneumoniae, and MTB, whereas pus swab cultures from otitis media grew Pseudomonas aeruginosa, Staphylococcus aureus, and coagulase-negative staphylococci. DISCUSSION: This study highlights the therapeutic challenges of GPA complicated by concurrent infections. Patients exhibited typical GPA signs, confirmed by PR3 c-ANCA levels. Concurrent infections require cautious antibiotic treatment before starting immunosuppressive therapy, except in life-threatening organ dysfunction. A unique case presented with both tuberculosis and GPA. Tailored treatment regimens combining antibiotics and immunosuppressives, including corticosteroids, methotrexate, and rituximab, resulted in clinical and radiological improvement in all the patients within 3-6 months. The addition of co-trimoxazole reduced the incidence of non-severe GPA relapses. CONCLUSION: Tailored treatment plans addressing both infectious and autoimmune aspects are essential for optimal care in GPA complicated by concurrent infections. This study highlights the need for a multidisciplinary approach involving pulmonologist, rheumatologist, microbiologist, and pathologist in the diagnosis and treatment of GPA, emphasizing the importance of individualized treatment plans tailored to the specific clinical scenario.

19.
Cureus ; 16(8): e67799, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39323683

RESUMEN

Common variable immunodeficiency (CVID) is a primary disorder characterized by impaired B cell differentiation and defective immunoglobulin production. This condition often presents with a wide range of clinical manifestations, including increased frequency and severity of infections, autoimmune diseases, and inflammatory disorders, which can lead to delays in diagnosis. Granulomatous involvement of the brain is an extremely rare but severe manifestation of CVID. We present a case of a woman in her 30s with a history of Evans syndrome and lymphocytic alveolitis who was admitted with persistent headache without neurological symptoms. Imaging revealed multiple infiltrative brain lesions. Despite the absence of recurrent infections, the patient's history of autoimmune manifestations and immunoglobulin deficiencies led to the diagnosis of CVID without the need for a brain biopsy. Treatment with intravenous immunoglobulin and immunosuppressive therapy resulted in significant clinical improvement and resolution of brain lesions. This case highlights the importance of considering CVID in patients with autoimmune manifestations and the effectiveness of prompt immunoglobulin replacement and immunosuppression in managing severe presentations of this condition.

20.
Eur J Breast Health ; 20(4): 233-240, 2024 Sep 26.
Artículo en Inglés | MEDLINE | ID: mdl-39323266

RESUMEN

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.

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