Multiple System Involvement Research Articles

The use of glucocorticoids in Behçet’s disease

Behçet’s disease (BD) is a chronic, multifactorial inflammatory disorder characterized by episodic involvement of multiple systems, including mucocutaneous, ocular, vascular, gastrointestinal, joint, and neurological domains. Glucocorticoids (GC) play a pivotal role in managing BD, especially during acute flares and severe organ involvement. This review highlights the tailored use of GC across various manifestations of BD. For mucocutaneous lesions, topical GC are effective, where as short-term low-dose systemic GC are reserved for colchicine-resistantcases. In ocular BD, systemic GC are in dispensable for sight-threatening conditions, often combined with disease-modifying anti-rheumatic drugs (DMARDs) or biologics to minimize GC dependency. In vascular involvement, particularly pulmonary artery aneurysms, high-dose or pulse GC are essential to control vessel wall inflammation, often alongside immunosuppressive agents like cyclophosphamide. Neurological BD necessitate surgent high-dose GC therapy, complemented by DMARDs for sustained control. Joint involvement can be managed with intraarticular GC, reducing systemic exposure. In gastrointestinal BD, GC use is limited due to potential mucosal irritation, with biologics and DMARDs serving as adjunctive options. Across all manifestations, GC tapering is prioritized to mitigate adverse effects, while combination therapy with DMARDs or biologics ensures comprehensive disease control. This comprehensive review underscores the critical role of GC in BD management, advocating for individualized treatment strategies to balance efficacy and safety.

Integrated bioinformatics analysis reveals that CCBP2 and GPR87 are new GPCR-associated biomarkers for preeclampsia

BackgroundPreeclampsia (PE) is a multifaceted pregnancy syndrome marked by multiple system involvement and a significant contributor to maternal mortality. This condition is characterized by a critical lack of early diagnostic measures and viable therapeutic options, underscoring an urgent need for the identification of reliable markers with both diagnostic and therapeutic potential.MethodsThis study utilized Weighted Gene Co-expression Network Analysis (WGCNA) to explore the role of G protein-coupled receptors (GPCRs) in the pathogenesis of PE.ResultsOur analysis pinpointed CCBP2 (ACKR2) and GPR87 as central PE-associated GPCRs. Experimental validation of these findings revealed that both CCBP2 and GPR87 significantly inhibit the proliferation, migration, and invasion of trophoblast cells—core phenomena underlying the pathology of PE.ConclusionThus, our findings add valuable candidates to the growing list of biomarkers for preeclampsia and offer promising targets for future therapeutic development.

Animal models of Long Covid: A hit-and-run disease.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV 2) pandemic has caused more than 7 million deaths globally. Despite the presence of infection- and vaccine-induced immunity, SARS-CoV-2 infections remain a major global health concern because of the emergence of SARS-CoV-2 variants that can cause severe acute coronavirus disease 2019 (COVID-19) or enhance Long Covid disease phenotypes. About 5 to 10% of SARS-CoV-2-infected individuals develop Long Covid, which, similar to acute COVID 19, often affects the lung. However, Long Covid can also affect other peripheral organs, especially the brain. The causal relationships between acute disease phenotypes, long-term symptoms, and involvement of multiple organ systems remain elusive, and animal model systems mimicking both acute and post-acute phases are imperative. Here, we review the current state of Long Covid animal models, including current and possible future applications.

Understanding the pathogenesis of infertility in endometriosis - literature review

Introduction and objective Endometriosis is a complex systemic condition characterized by the growth of functional endometrial tissue outside the uterine cavity, affecting 10-15% of women of reproductive age. Up to 50% of these women face infertility challenges. Although there is a scientifically established connection between endometriosis and infertility, the underlying mechanisms remain not fully understood. Review methods The PubMed database was searched using phrases related to the topic of endometriosis-associated infertility. The search included original research articles, review papers, and guidelines, including the Polish guideline on managing women with endometriosis. Ultimately, 25 relevant sources presenting the latest knowledge were selected. Abbreviated description of the state of knowledge The pathogenesis of infertility associated with endometriosis is complex and involves multiple factors. The most frequently cited contributors in the scientific literature include pain and dyspareunia, mechanical factors, reduced ovarian reserve, oxidative stress, changes in embryo and oocyte quality, impaired ovulation, and compromised endometrial receptivity. Summary Endometriosis affects a growing number of women worldwide, with nearly half experiencing infertility. The complexity and involvement of multiple organ systems make it difficult to pinpoint a single cause, posing a challenge for clinicians. Addressing these diverse factors is essential for improving fertility management in women with endometriosis.

Essential Competencies for Oncology in Physical Therapist Professional Education Programs: Results of a Mixed Methods Modified Delphi Study.

The objective was to establish consensus-based competencies for oncology within physical therapist professional education programs in the United States. A mixed-methods approach implementing a sequential exploratory design that included 3 phases was used to establish oncology competencies for physical therapist professional education programs. Participants in each phase were physical therapists representing diverse practice settings, experience levels, and geographical regions. Student physical therapists were included in phases 2 and 3. Three online focus groups were followed by an in-person group discussion to establish cancer-related themes, domains of practice, and competencies. Participants evaluated the competencies in a 3-round modified Delphi study for relevance and clarity. Each competency required 80% consensus using a Likert scale (1 = not at all relevant/clear, 5 = extremely relevant/clear). It was not accepted if a competency did not meet the 80% threshold by the end of round 3. Six domains of practice and 28 competencies were developed and evaluated. Within the 6 domains, 21 competencies were accepted: general cancer concepts (n = 4), musculoskeletal system (n = 3), neurologic system (n = 5), integumentary system (n = 2), cardiovascular and pulmonary system (n = 5), and involvement of multiple systems across the lifespan (n = 2). Along with the 21 competencies, participants also recommended 11 overarching oncology themes to incorporate into physical therapist professional education programs. Delivering cancer content using a body systems approach was recommended. As the number of survivors of cancer continues to grow, integration of these essential competencies within physical therapist professional education programs will improve the profession's capacity to provide quality care to meet the societal need of persons living with and beyond cancer. Academic and clinical educators should integrate these competencies to ensure that physical therapist professional education programs appropriately prepare physical therapists for providing care for persons living with and beyond cancer across the lifespan.

Systemic Lupus Erythematosus, An Unusual Presentation: A Case Report In Azal Hospital, Sana’a, Yemen

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by various clinical presentations and potential involvement of multiple organ systems; it exhibits a significant female predominance, with a peak incidence of middle-aged individuals. In this article, we report a case of a 19 years old female patient who presented with fever, headache, fatigue and neck stiffness. Initial investigations revealed a normal white blood cell count (WBC) with neutrophilia, leading to a diagnosis of bacterial meningitis. The patient responded to antibiotic and analgesic treatment. However, subsequent development of arthritis and skin lesions raised suspicion of systemic lupus erythematosus, which was confirmed by positive anti-dsDNA and antinuclear antibody (ANA) tests.

Early diagnosis of lupus: A possibilty. A multicentric study from SLE Special Interest Group (SIG) of Indian Rheumatology Association (IRA).

Systemic Lupus Erythematosus (SLE) warrants an early diagnosis and prompt management. Delay in diagnosis can result in repeated flares, permanent damage, and even death. There is a large variability in the time taken to diagnose SLE across the world. We undertook this study to determine the time taken for diagnosis of SLE in India and to identify the factors associated. Patients with SLE diagnosed within the previous 1year as per Systemic Lupus Erythematosus International Collaborating Clinics criteria (SLICC) 2012 criteria were included in a cross-sectional multicentre questionnaire-based survey. Demographic profile, self-reported socioeconomic status as per Kuppuswamy classification of socioeconomic status (version 2022) (SES), and several healthcare related parameters including referral pattern were recorded. Median time taken for diagnosis was used to demarcate early or late diagnosis and associated factors were explored. We included 488 patients with SLE from 10 rheumatology centres. The median time to diagnosis was 6months Interquartile Range (IQR 3,14.7) and within 3months in about one third [150(30.7%)]. Very early diagnosis (<1month) was established in 78(16.0%) patients. The mean SLE Disease Activity Index (SLEDAI) at diagnosis was 10.28+7.24. In univariate analysis, an older age, lower SES, non-southern state of residence and larger family size were significantly associated with late diagnosis. In the multivariate analysis, higher SES (AOR 0.95, 95% CI: 0.92-0.98), multiple organ system involvement at initial presentation (AOR1.75 95%CI: 1.08-2.84) and place of residence in south Indian states (AOR1.92 95%CI: 1.24-2.97) had lesser odds of being associated with late diagnosis. Distance from the closest medical centre/professional did not influence the time to diagnosis. Majority of patients had first consulted a medical graduate (42.5%) or postgraduate doctor (48.2%), and referral to rheumatologist was largely done by postgraduate (65%) doctors. More than half of our patients (61%) self-finance their treatment. Median time to diagnosis of SLE was 6months, 1/3rd being diagnosed within 3months and 78(16.0%) with 1month of symptom onset. Delay in diagnosis was noted in those belonging to lower socioeconomic strata and those with single organ disease. Distance to the health care facility did not influence time to diagnosis.

Diagnostic challenge of cutis Verticis Gyrata (CVG) in a patient presenting clinical features of Noonan or turner syndrome

Cutis Verticis Gyrata (CVG) is an uncommon condition, often classified as primary (idiopathic) or secondary to other diseases or syndromes. Its pathogenesis remains poorly understood, and its association with genetic syndromes is particularly rare. Noonan and Turner syndromes are distinct genetic disorders with characteristic phenotypes and multiple systemic involvements. This report aims to highlight the diagnostic complexities when CVG presents in the backdrop of these syndromes. A 38 years old patient was presented with chief complaints of receding hairline, dropping eyelids, cerebral deformations with deep furrows and thickened dermis. On the basis of patient's complaints, Noonan or turner syndrome was considered as possible diagnosis. This particular report presents a case of patient suffering from CVG having history of noonan and turner syndrome. With the detailed MRI, histology etc. CVG was finally confirmed. The novelty of this case lies in its rarity, diagnostic complexity, and the need for a multidisciplinary approach to unravel and manage the intersecting conditions. It contributes valuable insights to the existing medical literature, enhancing our understanding of the interplay between dermatological and genetic conditions. Patients with Noonan and turner syndrome exhibit clinical signs and symptoms that are strikingly similar to those of CVG, suggesting that this presents a significant diagnostic problem. An unfavorable outcome could arise from delayed or incorrect diagnosis. Because of this, it is recommended that healthcare fraternities should include uncommon illnesses like CVG as differential diagnosis. Considering CVG in differential diagnosis is crucial for early identification, accurate diagnosis, and comprehensive management. It ensures that associated systemic and genetic conditions are not overlooked and that patients receive holistic and personalized care.

A Complex Case of Partial Digeorge Syndrome with Multiple System Involvement: Seizure Disorder, Hypothyroidism and Recurrent Infection

A Complex Case of Partial Digeorge Syndrome with Multiple System Involvement: Seizure Disorder, Hypothyroidism and Recurrent Infection

An efficient and successful outcome after haematopoietic stem cell transplantation in a patient with an LPS-responsive beige-like anchor gene mutation.

Lipopolysaccharide (LPS)-responsive beige ankyrin (LRBA) gene mutations were first reported as the cause of immunodeficiency syndromes and autoimmunity in 2012. The majority of LRBA patients have multiple organ system involvement and a complex clinical phenotype. Herein we present a comprehensive account on the disease progression and transplantation procedure in a patient with LRBA deficiency who exhibited progressive autoimmune disease symptoms along with recurrent pulmonary infections since the age of 6 years old. Despite receiving abatacept therapy and immunoglobulin replacement treatments to manage the symptoms, but the symptoms still progressed. Therefore, nine years after disease onset, patients were treated with allogeneic haematopoietic stem cell transplantation (allo-HSCT). The patient experienced acute and chronic graft-versus-host disease (GVHD) and recurrent infections after transplantation. During one and a half years of follow-up, we found that allogeneic haematopoietic stem cell transplantation can relieve the symptoms of autoimmune disease in patients with LRBA deficiency, and marked clinical improvement and recovery of immune function were observed following stem cell transplantation.

Integrated re-analysis of transcriptomic and proteomic datasets reveals potential mechanisms for Zika viral-based oncolytic therapy in neuroblastoma.

Paediatric neuroblastoma and brain tumours account for a third of all childhood cancer-related mortality. High-risk neuroblastoma is highly aggressive and survival is poor despite intensive multi-modal therapies with significant toxicity. Novel therapies are desperately needed. The Zika virus (ZIKV) can access the nervous system and there is growing interest in employing ZIKV as a potential therapy against paediatric nervous system tumours, including neuroblastoma. Here, we perform extensive data mining, integration and re-analysis of ZIKV infection datasets to highlight molecular mechanisms that may govern the oncolytic response in neuroblastoma cells. We collate infection data of multiple neuroblastoma cell lines by different ZIKV strains from a body of published literature to inform the susceptibility of neuroblastoma to the ZIKV oncolytic response. Integrating published transcriptomics, interaction proteomics, dependency factor and compound datasets we propose the involvement of multiple host systems during ZIKV infection. Through data mining of published literature, we observed most paediatric neuroblastoma cell lines to be highly susceptible to ZIKV infection and propose the PRVABC59 ZIKV strain to be the most promising candidate for neuroblastoma oncolytic virotherapy. ZIKV induces TNF signalling, lipid metabolism, the Unfolded Protein Response (UPR), and downregulates cell cycle and DNA replication processes. ZIKV infection is dependent on sterol regulatory element binding protein (SREBP)-regulated lipid metabolism and three protein complexes; V-ATPase, ER Membrane Protein Complex (EMC) and mammalian translocon. We propose ZIKV non-structural protein 4B (NS4B) as a likely mediator of ZIKVs interaction with IRE1-mediated UPR, lipid metabolism and mammalian translocon. Our work provides a significant understanding of ZIKV infection in neuroblastoma cells, which will facilitate the progression of ZIKV-based oncolytic virotherapy through pre-clinical research and clinical trials.

Landscape of Invasive Fusariosis in Pediatric Cancer Patients: Results of a Multicenter Observational Study From Latin America.

Invasive fusariosis (IF) is a life-threatening opportunistic infection that affects vulnerable hosts. We conducted a multicenter and multinational retrospective study to characterize the natural history and clinical management of IF in pediatric cancer patients. We selected patients <18 years old who were sequentially hospitalized in 10 Latin American medical centers with a diagnosis of IF between 2002 and 2021. Data were collected using an electronic case report form complemented by a dictionary of terms. We assessed mortality rates at 30, 60, and 90 days. We collected data from 60 episodes of IF (median age, 9.8 years) that were mostly documented in patients with hematologic cancer (70%). Other risk conditions found were lymphopenia (80%), neutropenia (76.7%), and corticosteroid exposure (63.3%). IF was disseminated in 55.6% of patients. Skin lesions was present in 58.3% of our patients, followed by pulmonary involvement in 55%, sinusitis in 21.7%, bone/joint involvement in 6.7% and 1 case each of endocarditis and brain abscess. Positive blood and skin biopsy cultures were detected in 60% and 48.3% of cases, respectively. Fusarium solani complex was the most commonly identified agent (66.6%). The majority of patients received monotherapy within the first 72 hours (71.6%), either with voriconazole or amphotericin B formulation. The mortality rates at 30, 60, and 90 days were 35%, 41.6%, and 45%, respectively. An important factor affecting mortality rates appears to be disseminated disease. The high percentage of patients with fungal involvement in multiple organs and systems highlights the need for extensive workup for additional sites of infection in severely immunocompromised children.

“An unprecedented occurrence: a case report of pulmonary hypertension manifestation in Donohue syndrome”

IntroductionDonohue syndrome (DS), also referred to as leprechaunism, is a remarkably uncommon autosomal recessive disorder that primarily affects the endocrine system. Its incidence rate is exceedingly low, with only 1 case reported per 4 million live births. The syndrome is distinguished by a series of characteristic clinical features.Case presentationWe present a case of a twenty-month-old male with DS who experienced a range of dysmorphic and clinical features with the involvement of multiple systems. These features include skin hyperpigmentation, hypertrichosis, distinct facial features, abdominal distension, and microcephaly, with the involvement of the endocrine, renal, respiratory, and cardiac systems.ConclusionThe primary features of DS involve severe insulin resistance and growth abnormalities, the association with pulmonary hypertension (PHTN) has not been reported before. This finding adds more complexity to the condition. To the best of the author’s knowledge, this is the first report for a patient with DS who has PHTN. Further investigation is required since the mechanisms behind the development of PHTN in DS are not entirely understood. Shedding light on this association will contribute to better management strategies and outcomes for affected patients.

ALK positive histiocytosis with multiple system involvement: report of a case

ALK positive histiocytosis with multiple system involvement: report of a case

Clinical features and genetic analysis of three patients with Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome due to variants of FOXP3 gene

To analyze the clinical characteristics of three patients with Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. Three patients with IPEX syndrome diagnosed at the Children's Hospital of Fudan University from January 24, 2013 to July 29, 2019 were selected as the study subjects. Their clinical features, laboratory investigations and results of genetic testing were summarized. Treatment and prognosis were also explored. All of the three children had developed the disorder during infancy. One child had initial features including diabetes and diabetic ketoacidosis, whilst the other two had initiated by diarrhea. All patients had gastrointestinal involvement, and one was diagnosed as very early onset inflammatory bowel disease by colonoscopy and biopsy. Two children also had endocrine glands involvement. One child had manifested type 1 diabetes and positivity for thyroglobulin and thyroid peroxidase antibodies, though his thyroid function had remained normal. Another one had hypothyroidism and was treated by levothyroxine. Genetic testing revealed that all children had harbored missense variants of the FOXP3 gene, including c.1222G>A (p.V408M), c.767T>C (p.M256T) and c.1021A>G (p.T341A). The clinical symptoms of one patient were alleviated following allogeneic hematopoietic stem cell transplantation. One patient was stable after treatment with infliximab plus insulin, and one child had died of refractory septic shock and multiple organ dysfunction syndrome at 3 months old. FOXP3 gene variant-associated IPEX syndrome may have very early onset and diverse clinical manifestations. For male patients with infantile onset chronic diarrhea, multiple endocrine or multiple system involvement, genetic testing is recommended, which may facilitate early diagnosis, treatment and genetic counseling.

Clinicopathological profile of multiple myeloma at a tertiary care hospital in a resource-poor setting: A retrospective study

Objectives: The objective of this study was to study the clinicopathological profile of multiple myeloma (MM) presenting to Jawaharlal Nehru Hospital and Research Center (JLNH&amp;RC) Bhilai and document the disease in central India. Material and Methods: This was a retrospective observational study using patient data from January 2013 to December 2019. The clinical and radiological findings, laboratory parameters, and bone marrow examination were analyzed. Results: About 35.38% of patients presented in the 6th decade of life with a male-to-female ratio of 1.3:1. About 91.93% of patients had low backache and bone pain, and 96.92% of patients had Anemia. About 63.01% of patients had serum creatinine &gt;2 mg/dL, and 92.30% of patients had A/G ratio reversal. About 64.70% of patients had serum beta-2 microglobulin (≥3.5 μg/mL). About 80.7% had osteolytic lesions, predominantly in the skull and pelvis. About 46.15% of patients had &gt;50% plasma cells on bone marrow aspirate. About 85.71% exhibited hypercellularity, and 8.92% of patients had grade 2 marrow fibrosis. About 76.92% of patients presented with Durie Salmon stage III disease, and 58.82% presented with international staging system (ISS) stage II disease. Conclusion: MM has an inconsistent clinical presentation with multiple system involvement. It should be considered as a differential in patients above 50 years of age presenting with normocytic normochromic Anemia and bony pain. Bone marrow study is important in resource-poor settings where specialized laboratory testing is limited. The Durie and Salmon staging and the ISS can be used for the prognosis with equal efficacy.

The International Association for the Study of Lung Cancer Staging Project for Lung Cancer: Proposals for the Revision of the M Descriptors in the Forthcoming Ninth Edition of the TNM Classification for Lung Cancer

IntroductionThis study analyzed all metastatic categories of the current TNM classification of NSCLC to propose modifications of the M component in the next edition (ninth) of the classification. MethodsA database of 124,581 patients diagnosed between 2011 and 2019 was established; of these, 14,937 with NSCLC in stages IVA to IVB were available for this analysis. Overall survival was calculated using the Kaplan-Meier method, and prognosis was assessed using multivariable-adjusted Cox proportional hazards regression. ResultsThe eighth edition M categories revealed good discrimination in the ninth edition data set. Assessments revealed that an increasing number of metastatic lesions were associated with decreasing prognosis; because this seems to be a continuum and adjustment for confounders was not possible, no specific lesion number was deemed appropriate for stage classification. Among tumors involving multiple metastases, decreasing prognosis was found with an increasing number of organ systems involved. Multiple assessments, including after adjustment for potential confounders, revealed that M1c patients who had metastases to a single extrathoracic organ system were prognostically distinct from M1c patients who had involvement of multiple extrathoracic organ systems. ConclusionsThese data validate the eighth edition M1a and M1b categories, which are recommended to be maintained. We propose the M1c category be divided into M1c1 (involvement of a single extrathoracic organ system) and M1c2 (involvement of multiple extrathoracic organ systems).

Clinical Characteristics of Chlamydia psittaci Infection Diagnosed by Metagenomic Next-Generation Sequencing: A Retrospective Multi-Center Study in Fujian, China.

This study aimed to describe and compare the epidemiological, demographic, clinical, laboratory and radiological characteristics as well as the complications, treatments, and outcomes of these patients. We retrospectively investigated clinical data of patients with C. psittaci infection (psittacosis) in eight Grade IIIA hospitals of Fujian. Metagenomic next-generation sequencing (mNGS) was used identify C. psittaci in clinical samples of all included patients. A total of 74 patients (39 severe/35 non-severe) was diagnosed with psittacosis, 25 (33.8%) of whom had history of poultry exposure. Common symptoms included high fever (98% [37/74]), fatigue (52.7% [39/74]), and dyspnea (51.4% [38/74]). Common manifestations in imaging included consolidation (89.2%), pleural effusion (77.0%), and air bronchogram (66.2%). Common complications included acute respiratory distress syndrome (55.4% [41/74]), type I respiratory failure (52.7% [39/74]), acute liver injury (41.9% [31/74]), and secondary infection (27.0% [20/74]). The in-hospital mortality rate was 8.11% (6/74). C. psittaci infection is represents an underestimated cause of CAP. For SCAP patients with poultry and bird contact history, specimens were encouraged to be sended for mNGS test in time. C. psittaci infection can lead to severe, multiple system involvement, and several complications. mNGS facilitate timely diagnosis of C. psittaci infection.

Retrospective Analysis of Canadian Adults with Tuberous Sclerosis Complex.

Our study goal was to characterize the relative frequencies of molecular and phenotypic traits of tuberous sclerosis complex (TSC) in a Canadian adult population. Previous studies have sought to identify TSC-related genotypic and phenotypic trends in pediatric cohorts, but little is known about clinical manifestations and severity when it presents in adults. We conducted a retrospective chart review of adult patients seen at the TSC clinic at the University Health Network genetics clinics (Toronto, Ontario) to compare trends in the relative frequency of TSC manifestations with genotype. Fifty-one patients were eligible for this study. Eight patients had a pathogenic/likely pathogenic variant in the tuberous sclerosis complex 1 (TSC1) gene, 18 had a tuberous sclerosis complex 2 (TSC2) pathogenic/likely pathogenic variant, 6 patients had multiple variants identified in TSC1/TSC2 or TSC2/PKD1, 11 had no mutation identified (NMI) and 8 had no genetic testing done. Patients with a pathogenic/likely pathogenic variant in TSC2 presented with an increased involvement of multiple systems and a higher frequency of TSC-related manifestations relative to the other mutation groups. Previous studies comparing the wide phenotypic variability with TSC genotype have mainly comprised pediatric cohorts. With a focus on adults, we found trends to be similar across previous literature. An informed multidisciplinary approach should be taken to ensure proper surveillance and management of adults with TSC until a correlation between genotype and phenotype, especially past infancy, is better understood.

IgG4-related disease - a clinical case

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease affecting one or more organs. Pathomorphologically, a dense infiltrate of lymphocytes and IgG4 plasmocytes, and subsequent fibrosis is detected. Despite the many hypotheses of genetic predisposition, molecular mimicry and autoimmune nature of the disease, the etiology still remains unclear. Both innate and acquired immunity are believed to play a key role in disease pathogenesis. The involvement of occupational risk factors is also discussed. The possible involvement of multiple organs and systems (most often lacrimal and salivary glands, kidneys, lungs, aorta, pancreas, hepatobiliary duct, lymph nodes, as well as retroperitoneal fibrosis) is the reason for the multidisciplinary approach in this disease. In pulmonary involvement, clinical manifestations are nonspecific (cough, dyspnea, chest or back pain, hemoptysis, low-grade fever, weight loss) or patients are asymptomatic and pulmonary changes are an incidental finding on imaging. Up to 60% of cases of autoimmune pancreatitis represent a pancreatic manifestation of IgG4-related disease, with the majority of patients being elderly men. The most common differential diagnosis is a malignant process due to the tumor-like changes of the affected organ. Treatment with corticosteroids, immunosuppressors and symptomatic agents in most cases has a good effect, although relapses of the disease are also observed. We present a clinical case of a 72-year-old patient whose first complaints were nonspecific, for which a chest computed tomography was performed with visualization of four solid nodules in the right lung. After antibiotic therapy, without effect and deterioration of the patient's condition, video-assisted thoracoscopy, biopsy, histology and immunohistochemistry were performed. A diagnosis of IgG4-RD with pleural and lung involvement was confirmed. Anemic syndrome, increased values ​​of amylase, lipase, and acute phase parameters were found from the laboratory tests. Through endoscopic retrograde cholangio-pancreatography, autoimmune pancreatitis was diagnosed in the course of IgG4-RD. A plastic stent was placed on the common bile duct. Treatment with Prednisolone 40 mg/day was started with a subsequent dose reduction. Follow-up computed tomography of the lung after 9 months demonstrated a reduction of the described changes in the lungs, but with persistent lymphadenopathy and fibrotic changes. Azathioprine 100 mg daily was added to prednisone therapy. Against the background of the combined treatment, an improvement of the clinical condition and laboratory parameters was established.