Nodular Lesions Research Articles

Morning Glory Disc Anomaly: Expanding the MR Phenotype.

Morning glory disc anomaly (MGDA) is a congenital malformation characterized by a funnel-shaped optic disc excavation with radiating vessels and a central glial tuft. Imaging is essential to evaluate associated cephalocele and steno-occlusive vasculopathy. The goal of this study was to assess optic nerve, chiasmatic, and sphenoid bone morphology in MGDA. This retrospective study examined all subjects with funduscopically confirmed MGDA diagnosed and imaged with brain MR imaging between 2008 and 2023. Thirty-two children met inclusion criteria. Ocular involvement was unilateral in 29 subjects and bilateral in 3. Segmental optic nerve enlargement ipsilateral to the MGDA was seen in 21 subjects, with 3 also demonstrating a segmental reduction in the size of the ipsilateral optic nerve. Segmental reduction in the size of the ipsilateral optic nerve was present in 3 additional subjects, one with bilateral MGDA. The optic chiasm appeared asymmetrically thickened in 21 subjects, often with deformity. The optic nerves appeared normal in signal intensity in all subjects, with faint peripheral chiasmatic enhancement in 4 of 20 patients who received contrast. Optic nerve findings were stable in 15 subjects with multiple examinations. A persistent craniopharyngeal canal was identified in 17 subjects with sphenoid cephalocele in 1 and mild inferior pituitary gland displacement in 4. Tubular or nodular nasopharyngeal lesions were seen in 10 subjects. One subject had an off-midline sphenoid bone cleft, midbrain deformity, and abnormal thickening of and enhancement around the left oculomotor nerve; the oculomotor nerve finding was present in 1 additional patient. MGDA often manifests with ipsilateral optic nerve thickening, leading to a potential misdiagnosis as optic glioma. MGDA is also commonly associated with a persistent craniopharyngeal canal with variable pituitary gland and infundibular deformity, cephalocele, and tubular or nodular nasopharyngeal lesions.

An immunocompetent lady with invasive aspergillosis presenting as disseminated lesions: a case report

BackgroundInvasive Aspergillosis is a fungal infection caused by Aspergillus species, typically posing life-threatening risks to immunocompromised individuals. While occurrences in immunocompetent hosts are rare, a recent case report documented fulminant pulmonary aspergillosis in an immunocompetent patient during autopsy. Here, we present a case of invasive aspergillosis in an immunocompetent woman, manifesting with disseminated lesions.Case presentationA 29-year-old Asian woman presented to our hospital in March 2022, reporting chest pain and shortness of breath persisting for two months. Upon examination, she appeared thin and unwell, with no notable abnormalities otherwise. Radiographic imaging revealed an ill-defined lesion in her left lung. Subsequent bronchoscopy and lavage were performed, followed by initiation of empirical antibiotic therapy. Lavage results were negative for gram staining, culture, and ZN staining for AFB, but revealed numerous septate hyphae on fungal smear. Histopathological examination indicated chronic granulomatous inflammation with septal fungal hyphae, indicative of aspergillosis. Subsequent culture confirmed Aspergillus species, prompting initiation of voriconazole therapy. Remarkably, the patient exhibited significant improvement, with weight gain and restored appetite observed within a short period. Within 2 months of treatment, her symptoms resolved, and she resumed near-normal daily activities.ConclusionThis case highlights the diagnosis of aspergillosis in an immunocompetent individual presenting with disseminated nodular lesions across the lungs, mediastinum, and abdomen. Clinicians should maintain a high index of suspicion for aspergillosis in cases of non-resolving pneumonia and disseminated nodular lesions, even in patients lacking traditional predisposing factors.

Atypical gouty tophus masquerading as a foreign body granuloma

Subcutaneous nodular lesions in a patient with significantly elevated inflammatory markers always raise the possibility of infectious diagnoses such as atypical infection, pyogenic abscess, mycobacterial infection, and malignancy. Definitive diagnosis requires tissue biopsy and histopathologic examination. The atypical presentation of a foreign body granuloma with concurrent intraoperative findings supports the diagnosis of gouty arthritis.We report a case of a 67-year-old man who presented with an inflammatory nodular lesion on the left elbow that was initially suspected to have an infectious cause. Histopathologic examination of the nodular tissue later revealed that the patient had a foreign body granuloma due to urate crystal deposition. The atypical appearance of the gouty arthritis, the low serum urate level, the negative crystal identification in the synovial fluid, and the markedly elevated inflammatory markers, which did not respond to the previous antibiotic and steroid therapy, raised the suspicion of atypical infection in this case.

Multicentric reticulohistiocytosis with oral and laryngeal involvement in association with autoimmune/inflammatory syndrome induced by adjuvants (ASIA): expanding the spectrum of two uncommon entities.

Multicentric reticulohistiocytosis (MRH) is the most frequent entity in the group of reticulohistiocytoses. It is usually accompanied by a symmetrical erosive polyarthritis and is frequently associated with cancer and autoimmune disorders. Autoimmune syndrome induced by adjuvants (ASIA) is an inflammatory syndrome triggered by adjuvants such as those contained in vaccines or by silicone implants. Here we report a 71-years old female with a history of breast cancer treated with surgery and subsequent prosthesis who developed a systemic hyperinflammatory syndrome including seronegative symmetric polyarthritis, multiple skin lesions and two large nodular lesions in the oral cavity and larynx. Clinical picture was consistent with a clinical diagnosis of ASIA, with breast implant rupture and/or vaccination against SARS-CoV-2 as possible triggers. Histopathology of skin, oral and laryngeal nodules revealed cutaneous/mucous and submucosal infiltration of large epithelioid mononuclear or binucleated cells with fine granular ground glass-like cytoplasm and round to kidney-shaped nuclei with prominent nucleoli, without atypical features or relevant pleomorphism, accompanied by sparse giant cells and lymphocytes. These cells stained positive for CD68 and CD45 and negative for S100, CD1a, and markers of epithelial or neural/melanocytic differentiation, altogether consistent with a diagnosis of reticulohistiocytosis. Clinic-pathological correlation allowed the final diagnosis of MRH. To our knowledge, this is the first report of a co-occurrence of MRH with ASIA and this is relevant to broaden the spectrum of those both rare diseases.

Risk factors for Helicobacter pylori infection in children with gastrointestinal symptoms in Vietnam

ObjectivesTo investigate the prevalence and risk factors associated with Helicobacter pylori infection in Vietnamese children. MethodsChildren under 16 years old with gastrointestinal symptoms underwent esophagogastroduodenoscopy and H. pylori infection was diagnosed using rapid urease test. ResultsA total of 246 children with gastrointestinal symptoms were included. The mean age was 8.4 ± 2.6 years. A total 81.3% tested positive for H. pylori. Children infected with H. pylori had a lower rate of nausea but a higher rate of lesions in the duodenal bulb and nodular lesions than children without H. pylori infection (26.5% vs 45.6%, P <0.01; 40.0% vs 23.9%; P = 0.04; and 68.5% vs 30.3%, P <0.0001, respectively). Compared with children aged under 5 years, children aged 11 years and older were four times more likely to be infected with H. pylori, with odds ratio (OR) 3.50, 95% confidence interval (CI) 1.07-11.39, P = 0.04. Washing hands with soap was associated with a reduced risk of H. pylori infection by three times (OR 0.35, 95% CI 0.17-0.69, P = 0.002). Children living in a family where members had a history of H. pylori infection were nine times more likely to be infected with H. pylori (OR 8.87, 95% CI 1.15-68.45, P = 0.04). ConclusionsThe prevalence of H. pylori infection in Vietnamese children with gastroenteritis is high. Our results identified several risk factors and emphasize the role of handwashing with soap before eating and after using the toilet in reducing the risk of H. pylori infection in children.

Ultrasonographic features and diagnostic accuracy of FNA and CNB in secondary thyroid malignancies: A retrospective study

ObjectiveThis study aims to examine the ultrasonographic features of secondary thyroid malignancies and compare the diagnostic efficacy of fine-needle aspiration (FNA) and core needle biopsy (CNB) in this condition. MethodsA retrospective analysis was conducted on 29 patients with secondary thyroid malignancies treated at our center between July 2011 and October 2022. Ultrasound images and clinical data were analyzed, and the lesions were classified according to the American College of Radiology Thyroid Imaging Reporting and Data System (ACR TI-RADS). ResultsAmong the 29 patients studied, primary tumor sites were predominantly the esophagus, lung, and nasopharynx. Comprehensive ultrasound data was available for 28 of these patients, revealing nodular lesions in 24 cases and diffuse lesions in 4 cases. Nodular lesions were predominantly solid or nearly solid hypoechoic nodules with parallel growth and extrathyroidal extension features, with a few showing macrocalcifications. Most patients had varying degrees of metastasis to neck lymph nodes. FNA accurately diagnosed 31.6 % of the lesions as secondary thyroid malignancies, while 5.3 % were misdiagnosed as papillary thyroid carcinoma (PTC). However, CNB demonstrated 100 % reliability in diagnosing secondary thyroid malignancies. ConclusionThis study's categorization of secondary thyroid malignancy ultrasonographic features identifies nodular and diffuse patterns, with the application of ACR TI-RADS proving effective for nodular types. In detecting these lesions, CNB demonstrates superior sensitivity compared to FNA. Thus, in cases of thyroid lesions suspected to be malignant, particularly with enlarged neck lymph nodes and in patients with a history of malignancy, CNB is recommended as the diagnostic method of choice.

A case of mantle cell lymphoma with involvement of the entire gastrointestinal tract - Multiple endoscopic findings.

Mantle cell lymphoma (MCL) is an aggressive B-cell lymphoma that occurs in some patients with gastric and intestinal involvement, but esophageal involvement is rare, and involvement of the entire gastrointestinal (GI) tract is even rarer. The endoscopic manifestations of MCL are mainly nodular or polypoid lesions. We report a rare case of MCL containing esophageal involvement of the entire GI tract with multiple endoscopic findings.

Macroscopic and Histological Effects of Polycaprolactone Dermal Filler in the Orofacial Region: A Study in Rats

The objective of our study was to evaluate early and late macroscopic and histological changes associated with the use of polycaprolactone dermal filler (PCL) in the orofacial region. Forty-eight female Wistar rats were divided into the PCL group and the control group. The material was applied to the ventral tongue and submandibular region, and the animals were euthanized at three time points—24 h, and 30 and 90 days. In the PCL group, yellowish nodules were observed on the tongue at all experimental time points. At the 24 h mark, the histological analysis revealed the presence of the PCL and a predominance of lymphocytes, plasma cells, and neutrophils. At 30 and 90 days, macrophages and multinucleated giant cells predominated around the PCL spheres. Collagen density in the dermis was higher in the PCL group when compared to the control at 30 and 90 days. In the submandibular glands, an inflammatory process similar to that observed at other sites was noted, with no alterations in acinar or ductal morphology. The results of this study highlight the effectiveness of PCL as a collagen biostimulator. Nevertheless, the development of nodular lesions on the tongue signals the potential risk of complications in mobile anatomical structures.

LEPROMATOUS LEPROSY REVEALED BY PALMOPLANTAR NODULAR LESIONS

Leprosy is a chronic debilitating disorder caused by the acid-fast bacilli Mycobacterium leprae (M. leprae).This bacilli exhibit a distinctive predilection for the skin and peripheral nerves, although they can potentially impact any system in the body.Palmoplantar involvement has been infrequently reported in leprosy and is an easily misdiagnosed entity.We report a case of a young female presented with a palmo plantar nodular lesions for wich a skin biopsy showed histological signs consistent with lepromatous leprosy.

A translational model of chronic diabetic nephropathy in the Nile grass rat.

Diabetic nephropathy (DN) is a major healthcare challenge for individuals with diabetes and associated with increased cardiovascular morbidity and mortality. The existing rodent models do not fully represent the complex course of the human disease. Hence, developing a translational model of diabetes that reproduces both the early and the advanced characteristics of DN and faithfully recapitulates the overall human pathology is an unmet need. Here, we introduce the Nile grass rat (NGR) as a novel model of DN and characterize key pathologies underlying DN. NGRs spontaneously developed insulin resistance, reactive hyperinsulinemia, and hyperglycemia. Diabetic NGRs evolved DN and the key histopathological aspects of the human advanced DN, including glomerular hypertrophy, infiltration of mononuclear cells, tubular dilatation, and atrophy. Enlargement of the glomerular tufts and the Bowman's capsule areas accompanied the expansion of the Bowman's space. Glomerular sclerosis, renal arteriolar hyalinosis, Kimmelsteil-Wilson nodular lesions, and protein cast formations in the kidneys of diabetic NGR occurred with DN. Diabetic kidneys displayed interstitial and glomerular fibrosis, key characteristics of late human pathology as well as thickening of the glomerular basement membrane and podocyte effacement. Signs of injury included glomerular lipid accumulation, significantly more apoptotic cells, and expression of KIM-1. Diabetic NGRs became hypertensive, a known risk factor for kidney dysfunction, and showed decreased glomerular filtration rate. Diabetic NGRs recapitulate the breadth of human DN pathology and reproduce the consequences of chronic kidney disease, including injury and loss of function of the kidney. Hence, NGR represents a robust model for studying DN-related complications and provides a new foundation for more detailed mechanistic studies of the genesis of nephropathy, and the development of new therapeutic approaches.

Ear lobule reduction using a sub-antitragal groove technique in patients with angiolymphoid hyperplasia with eosinophilia on the earlobe: a case report and literature review.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare condition characterized by nodular lesions predominantly in the head and neck region, often causing discomfort or pain. Treatment remains challenging because of its rarity and the lack of established guidelines. This report presents a case of ALHE affecting the earlobes that was successfully managed using ear lobule reduction surgery and subsequent intralesional steroid injections. A 31-year-old woman with a history of recurrent earlobe masses underwent a partial excision to avoid the loss of the earlobe. Histopathological examination confirmed Kimura disease, a variant of ALHE. Subsequent local methylprednisolone injections effectively controlled the remaining lesions, resulting in significant size reduction without notching. Various treatment modalities have been attempted for this condition; however, recurrence rates remain high. Surgical resection combined with intralesional corticosteroid injections is the preferred approach. In this case, a sub-antitragal groove technique for earlobe reduction was employed to preserve the lateral edge of the ear lobule, minimize the risk of deformity, and achieve a predictable outcome. The sub-antitragal groove technique offers an approach to reduce earlobe size without compromising aesthetics. Further research is required to elucidate the pathogenesis of ALHE and establish standardized treatment protocols for this rare condition.

Lower Limb Elephnatiasis Nostras Verrucosa: Excision with full thickness skin grafting with aid of dermal regeneration template

Elephnatiasis Nostras Verrucosa (ENV) is a rare clinical condition, a progressively disfiguring disorder characterized by verrucous, nodules and cobblestone-like papules associated with non-pitting edema of the lower extremities. Histopathology is characterized by epidermal hyperkeratosis and dermal fibrosis. We report the history of a 32-year-old man who presented with 5 years history of right lower limb edema covered by nodular and papular skin lesions. Proper investigations put the diagnosis of ENV. The patient was successfully treated by debridement followed by skin graft using the technique of dermal regeneration. Management of ENV remains challenging. In the absence of randomized controlled trials on the best modalities of treatment, it is important to report such rare cases and their treatment outcome.

Darier Ferrand's dermato fibrosarcoma of the breast: About a case and literature review

Darier Ferrand's dermato fibrosarcoma protruding is the most common tumor of cutaneous sarcomas, recognized by its high potential for aggressiveness and local recurrences after surgery of up to 40%. The location of the DFS on the breast has been described as extremely rare. Histologically consisting of bundles of spindle cells arranged in a storiform pattern, but due to the immunoreactivity of CD34 in DFSP, its presentation can be confused with other malignancies or even benign lesions. The objective of this observation is to describe the anamnestic, clinical, histological, therapeutic and evolutionary aspects of a DFS located on the infero-outer quadrant of the left breast in a 38-year-old patient compared to the data in the literature. Our patient had consulted for a skin mass located on the infero-external quadrant of the left breast, with irregular surface, with multiple protruding and exophytic nodular lesions evolving for several years, without ulcerations, pain and no axillary lymphadenopathy. We find in his history a notion of chest trauma. Wide and deep surgical excision with a margin of 5cm in healthy skin was done and histopathological examination of the operative specimen confirmed the diagnosis of DFS. Adjuvant radiotherapy 60Gray at a rate of 2Gray per fraction 5 days per week was administered. This reputedly rare location overlaps in several aspects (anamnestic, clinical, epidemiological, evolutionary and therapeutic) with data from the literature. Keywords: Dermatofibrosrcoma, Darier and Ferrand, Cutaneous sarcomas, Recurrence.

Clinical management and case review of a presumptive novel clinical presentation of protothecosis in donkeys causing ulcerated nodular lesions in the ear pinnae of 18 animals

AbstractThis is a retrospective case series that describes a novel syndrome seen in 18 donkeys in Morocco that presented to this author,'s clinic with ulcerated nodular lesions of the ear pinnae. In the majority of cases, the lesions are confined to the ears, they are always bilateral and can occasionally progress to other areas of the body. Macroscopically, the lesions in all 18 donkeys are very uniform in presentation, even if the size of the nodules varies. This case series reviews the presentation, investigation, treatment and outcome for 18 cases presented to these authors’ clinics from 2011 to 2021. Skin biopsies from a subset of eight cases showed similar changes in chronic pyogranulomatous, dermatitis with myriad intra‐ and extra‐histiocytic organisms, the morphology of which is most consistent with that of Prototheca spp. infection. This is a novel presentation that represents a significant welfare issue for donkeys, as well as having important economic consequences for owners in the developing world.

Clinicopathological Features of 26 Intraoral Polymorphous Adenocarcinomas from a Single Brazilian Institution.

This study describes a large, well-documented case series of salivary gland polymorphous adenocarcinomas (PAC) from a single Brazilian center. Demographic data, clinical presentation, histopathological and immunohistochemical features from 26 cases of PAC were analyzed and discussed in detail. Most patients were females (n = 21), with a ratio of 1:4.2 (male: female) with a mean age of 58.8years (ranging from 36 to 84years). The most common clinical presentation was a fibrocollagenous, firm nodular lesion, with a mean size of 2.46cm (ranging from 0.5 to 3cm). Most lesions occurred on the palate (n = 16), followed by buccal mucosa (n = 3), upper lip (n = 3), buccal vestibule (n = 2) and alveolar ridge (n = 1). Histologically, various growth patterns were observed, including tubular, solid, cribriform, papillary, and cystic. Additionally, glomeruloid slit-like structures, mucous, and clear cells were noted. Surface papillary epithelial hyperplasia was observed in a few cases. Nine cases exhibited myxoid and collagenous areas, while two cases showed fusiform areas and another case demonstrated squamous differentiation. Clear cell predominance was noted in two cases, and peri- and intraneural invasion was seen in eight cases. Immunohistochemical analysis revealed positivity for S-100, p63 and CK7, and negativity for p40 in all cases. The Ki-67 proliferation index was markedly low in most cases, with a mean of 2.5%. We have provided a broad, detailed description of the clinical and microscopic features of PAC in a large, Brazilian cohort. These findings, in a resource-limited area, may be quite useful for establishing a proper diagnosis.

A new nomogram based on ultrasound and clinical features for distinguishing epididymal tuberculosis and nontuberculous epididymitis

Using ultrasound findings and clinical characteristics, we constructed and validated a new nomogram for distinguishing epididymal tuberculosis from nontuberculous epididymitis, both of which share similar symptoms. We retrospectively examined data of patients with epididymal tuberculosis and nontuberculous epididymitis hospitalized between January 1, 2013, and March 31, 2023. Eligible patients were randomly assigned to derivation and validation cohorts (ratio, 7:3). We drew a nomogram to construct a diagnostic model through multivariate logistic regression and visualize the model. We used concordance index, calibration plots, and decision curve analysis to assess the discrimination, calibration, and clinical usefulness of the nomogram, respectively. In this study, 136 participants had epididymal tuberculosis and 79 had nontuberculous epididymitis. Five variables—C-reactive protein level, elevated scrotal skin temperature, nodular lesion, chronic infection, and scrotal skin ulceration—were significant and used to construct the nomogram. Concordance indices of the derivation and validation cohorts were 0.95 and 0.96, respectively (95% confidence intervals, 0.91–0.98 and 0.92–1.00, respectively). Decision curve analysis of this nomogram revealed that it helped differentiate epididymal tuberculosis from nontuberculous epididymitis. This nomogram may help clinicians distinguish between epididymal tuberculosis and nontuberculous epididymitis, thereby increasing diagnosis accuracy.

Lung Involvement in Pulmonary Vasculitis: A Radiological Review.

Pulmonary vasculitis identifies a heterogeneous group of diseases characterized by inflammation, damage and necrosis of the wall of pulmonary vessels. The most common approach to classify vasculitis is according to etiology, therefore dividing them into primary and secondary, with a further sub-classification of primary vasculitis based on the size of the affected vessels (large, medium, and small). Pulmonary involvement is frequently observed in patients with systemic vasculitis and radiological presentation is not pathognomonic, but may vary between diseases. The main findings using high-resolution computed tomography (HRCT) include small vessel wall thickening, nodular lesions, cavitary lesions, reticular opacities, ground-glass opacities (GGO), consolidations, interlobular septal thickening, tracheobronchial stenosis, and aneurysmal dilatation of pulmonary arteries, with or without pleural effusion. Radiological diagnosis alone is difficult since signs and symptoms of lung vessel involvement are often non-specific and might overlap with other conditions such as infections, connective tissue diseases and neoplasms. Therefore, the aim of this review is to describe the most common radiological features of lung involvement in pulmonary vasculitis so that, alongside detailed clinical history and laboratory tests, a prompt diagnosis can be performed.

BULBOSITIES AND INTERVORTEX VENOUS ANASTOMOSIS IN VENOUS OVERLOAD CHOROIDOPATHY MASQUERADING AS POLYPOIDAL CHOROIDAL VASCULOPATHY.

To describe a patient with venous overload choroidopathy in whom venous bulbosities masqueraded as polyps and intervortex venous anastomosis mimicked a branching vascular network, giving the appearance of polypoidal choroidal vasculopathy. The patient had complete ophthalmic examination including indocyanine green angiography and optical coherence tomography. Venous bulbosities were defined on indocyanine green angiography as focal dilations in which the diameter of the dilation is two times that of the host vessel. A 75-year-old woman presented with combined subretinal and subretinal pigment epithelium hemorrhages in the right eye. During indocyanine green angiography, focal nodular hyperfluorescent lesions connected to a network of vessels were observed, which looked like polyps and branching vascular network in polypoidal choroidal vasculopathy. In both eyes, the midphase angiogram had multifocal choroidal vascular hyperpermeability. There was late-phase placoid staining nasal to the nerve in the right eye. During enhanced depth imaging-optical coherence tomography evaluation, there were no retinal pigment epithelium elevations that would be expected with polyps or branching vascular network in the right eye. A double-layer sign was seen corresponding to the placoid area of staining. Diagnosis of venous overload choroidopathy and choroidal neovascularization membrane was made. She was treated with intravitreal antivascular endothelial growth factor injections for the choroidal neovascularization membrane. Indocyanine green angiography findings in venous overload choroidopathy may mimic polypoidal choroidal vasculopathy, but differentiation is essential because it has implications for treatment. Similar findings may have been misinterpreted in the past and may have previously contributed to conflicting clinical and histopathologic descriptions of polypoidal choroidal vasculopathy.

Cutaneous Leiomyoma of the Leg: A Case Report and Literature Review

Cutaneous leiomyoma is a rare benign smooth muscle tumor that can be mistaken for other nodular cutaneous lesions. We present the case of a 27-year-old female graduate who developed a swelling on her left leg over a period of 2 years. Initially painless, the lesion became painful a year later, with pain exacerbated by heat. The lump measured 1 x 1 x 1 cm, was tender, and not warm. Initially diagnosed clinically as an epidermal inclusion cyst, she underwent excision biopsy revealing leiomyoma upon histological examination. Subsequent to excision, all symptoms resolved completely, with no recurrence noted. Hence, it is crucial to consider cutaneous leiomyoma as a potential differential diagnosis for such cutaneous lesions.

Co-existence of ANCA–associated vasculitides with immune-mediated diseases: a single-center observational study

BackgroundAntineutrophil cytoplasmic antibody-associated vasculitides (AAV) is a group of systemic necrotizing small vessel autoimmune diseases, with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) being the two most common. The co-existence of AAV with different immune-mediated diseases (autoimmune disesases - AID) might affect the clinical presentation of the primary disease. The purpose of the study was to assess the co-existence of AAV with AID and to investigate whether it affects the characteristics and the course of AAV.MethodsA retrospective single-center study was performed to identify patients with a diagnosis of MPA or GPA and concomitant AID, and to investigate their clinical features and characteristics. The group consisted of consecutive unselected AAV patients treated at a large university-based hospital, since 1988 with follow-up until 2022.ResultsAmong 284 patients diagnosed either with GPA (232) or MPA (52), 40 (14,1%) had co-existing AIDs. The most frequent were: Hashimoto thyroiditis (16 cases), rheumatoid arthritis (8 cases), followed by psoriasis (6 cases), pernicious anemia (3 cases), and alopecia (3 cases). Patients with autoimmune comorbidities had a significantly longer time between the onset of symptoms and the diagnosis (26 vs. 11 months, p < 0.001). Laryngeal involvement (20.0% vs. 9.0%, p = 0,05), peripheral nervous system disorders (35.0% vs. 13.9%, p < 0.001), and neoplasms (20.0% vs. 8.6%, p = 0,044) were more common in patients with AID comorbidities, compared to subjects without AID. In contrast, renal involvement (45.0% vs. 70.9%, p = 0.001) and nodular lung lesions (27.5% vs. 47.5%, p = 0.044) were significantly less frequent in patients with co-morbidities. Following EUVAS criteria, patients with autoimmune co-morbidities had a generalized form of the disease without organ involvement (52.5% vs. 27.2%, p = 0.007), while the others had a higher percentage of generalized form with organ involvement (38.3% vs. 20.0%, p = 0.007).ConclusionsThe coexistence of AAV with different autoimmune diseases is not common, but it might affect the clinical course of the disease. Polyautoimmunity prolonged the time to diagnosis, but the AAV course seemed to be milder. Particular attention should be paid to the increased risk of cancer in these patients. It also seems reasonable that AAV patients should receive a serological screening to exclude the development of overlapping diseases.