Salivary Gland Enlargement Research Articles

Sialoadenosis caused by spirocercosis in two dogs from Sri Lanka

AbstractSpirocercosis is a common parasitic disease in dogs in tropical and mediterranean regions of the world caused by the nematode Spirocerca lupi. Some dogs with spirocercosis develop sialoadenosis, a condition identified by non‐inflammatory, painless, bilateral enlargement of the mandibular salivary glands. Development of sialoadenosis in dogs infected with S. lupi occurs as a consequence of neurological stimulation caused by larval migration and oesophageal parasitic nodule formation. This report describes the clinical signs, diagnosis, treatment and the treatment outcome of two dogs diagnosed with sialoadenosis caused by spirocercosis. Phenobarbital therapy is useful to control clinical signs shown by the dogs with sialoadenosis secondary to spirocercosis presented at an early stage of the disease. A more favourable disease outcome is possible when phenobarbital therapy is combined with anti‐parasitic medication effective against S. lupi.

A very rare case of adenomatoid hyperplasia of minor salivary gland in a 55 year old male patient

Adenomatoid hyperplasia of minor salivary glands (AHMSG) is an extremely rare non-neoplastic enlargement of minor salivary glands with uncertain pathogenesis. It is most commonly seen in males around 40–50 years of age. The most common sites are hard or soft palate. Clinically, the lesion presents as a sessile tumor-like nodule which mimics neoplasm with histological findings of benign hyperplasia and hypertrophy of mucous glands. Herein, we report a very rare case of AHMSG in a 55-year-old male patient who presented with asymptomatic swelling over the floor of the mouth since 8 months.

Clinical Characteristics of Distinct Subgroups of Patients with Primary Sjögren's Syndrome Classified by Serological Profiles: A Comparison Study.

Sjögren's syndrome (SS) is an autoimmune disease characterized by heterogeneous clinical presentation and the presence of various autoantibodies. This study aimed to determine the differences in clinical findings according to antibody positivity in patients with primary Sjögren syndrome (pSS) in the Turkish population. A retrospective study was conducted and 402 patients (378 women and 24 men) with pSS were analyzed. The patients were categorized into three subgroups based on serological tests. These were (1) quadruple seropositivity (positive for anti-Sjögren's syndrome-related antigen A antibodies (anti-SSA; anti-Ro) and anti-Sjögren's syndrome-related antigen B antibodies (anti-SSB; anti-La), rheumatoid factor (RF), and antinuclear antibody (ANA); (2) double seropositivity (positive for ANA and anti-SSA/Ro antibodies); and (3) quadruple seronegativity (negative for ANA, RF, anti-SSA/Ro and anti-SSB/La antibodies). The number of quadruple-seropositive patients was 72 (18.6%), double-seropositive 174 (43.2%), and quadruple-seronegative was 85 (21.1%). The age at diagnosis of quadruple-seropositive pSS was 42.4 ± 10.8, which was significantly younger than that of patients with double-seropositive and quadruple-seronegative pSS (p = 0.021, p = 0.112). In terms of organ involvement, salivary gland enlargement, arthralgia, arthritis, Raynaud's phenomenon, lymphadenopathy, cutaneous vasculitis, interstitial lung disease, neurological involvement, autoimmune thyroiditis, renal interstitial disease, anemia, leukopenia, hypergammaglobulinemia, and hypocomplementemia were more common in quadruple-seropositive patients with pSS than in quadruple-seronegative patients (p < 0.0001). The results of this study confirmed the strong impact of immunological markers on the pSS phenotype at the time of diagnosis. Immunological patterns play a central role in the phenotypic expression of the disease, even during the initial diagnostic phase, and can guide physicians in designing personalized treatment plans for patients with pSS.

Clinical and laboratory characteristics of salivary gland ultrasonography-positive patients with primary Sjögren's syndrome.

This study aimed to investigate the clinical and laboratory characteristics of salivary gland ultrasonography (SGUS)-positive patients with primary Sjögren's syndrome (pSS) compared to SGUS-negative patients and to analyse the diagnostic value of SGUS and labial salivary gland biopsy (LSGB) grading in pSS. A retrospective analysis of patients admitted to the Affiliated Hospital of Yangzhou University between May 2019 and November 2023 was conducted. According to the OMERACT scoring system, patients with pSS were divided into an SGUS-negative group (score <2) and an SGUS-positive group (score ≥2). The patient's age, gender, clinical symptoms, laboratory parameters and diagnostic examinations were compared and analysed, and Spearman correlation analysis was used to analyse the correlation between SGUS, LSGB and influencing factors. There was no significant difference in dry mouth, dry eyes, tooth loss, fever, joint pain, fatigue, interstitial lung disease or renal tubular acidosis between the two groups, although there were more patients with salivary gland enlargement in the SGUS-positive group (p < 0.05). In terms of high levels of immunoglobulin G (IgG), high levels of rheumatoid factor (RF), anti-nuclear antibody ≥1:320, anti-Sjögren's syndrome A-52KD and anti-Sjögren's syndrome B, the number of cases in the SGUS-positive group was greater than that in the SGUS-negative group (p < 0.05). LSGB samples were graded per the Chisholm-Mason system with significant differences between multiple groups. SGUS score negatively correlated with age and positively correlated with LSGB grade. This study showed that the SGUS score positively correlated with LSGB grade in pSS patients and negatively correlated with patient age. Thus, SGUS and LSGB are consistent in the diagnosis of pSS to reflect the degree of salivary gland involvement, and patients who are SGUS positive have high RF and IgG levels, a variety of autoantibodies positive and a tendency toward salivary gland enlargement.

Evaluation of submandibular and parotid salivary glands by ultrasonography in patients with diabetes.

Microvascular complications of diabetes mellitus (DM) include oral manifestations and complications, including xerostomia, reduced salivary flow, susceptibility to infection, periodontal disease and salivary gland enlargement. The present study aims to evaluate B-mode ultrasonography (USG) parameters such as size, volume and echogenicity of the submandibular and parotid salivary glands on both sides, shear-wave elastography (SWE) value and colour Doppler properties in patients with DM and healthy control groups. In total, 160 right and left submandibular glands and 160 right and left parotid glands of 80 patients, 40 patients (20 type 1 DM, 20 type 2 DM) and 40 healthy control group, between the ages of 18-70 were examined by USG. Echogenicity, parenchyma internal structure, margin and dimensional measurements (antero-posterior length, supero-inferior length, medio-lateral length and volume) and colour Doppler with 'ML 6-15-D Matrix Array (4-15 MHz)' probe, shear-wave elastography '9L-D (2-8 MHz)' probe was investigated. Statistically significant difference was observed in echogenicity in the right submandibular gland, echogenicity in the right parotid gland, margin characteristics, parenchymal homogeneity and colour Doppler characteristics between the type 1 DM, type 2 DM and control groups (p < .05). It was observed that the size, volume and SWE values of both submandibular and parotid glands were higher in the DM patient group than in the control group. Higher values were observed in type 2 DM compared to type 1 DM in the patient group. USG is an effective imaging technique in investigating the effects of diabetes on the submandibular and parotid salivary glands.

Clinical and serological characteristics of anti-Ro/SS-A and anti-La/SS-B negative primary Sjögren's syndrome: a comparative study.

This study aimed to describe the clinical spectrum of primary Sjögren's syndrome (pSS) patients with anti-Ro/SS-A and anti-La/SS-B negativity. From a single-center study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, those with triple seronegativity anti-Ro/SS-A (anti-Sjögren's-syndrome-related antigen A autoantibody), anti-La/SS-B (anti-Sjögren's-syndrome-related antigen B autoantibody), rheumatoid factor (RF) (-) and antinuclear antibody (ANA)(+)] or [anti-Ro/SS-A(-), anti-La/ SS-B(-), RF(+) and ANA(-)] and quad¬ruple seronegativity [anti-Ro/SS-A(-), anti-La/SS-B(-), RF(-) and ANA(-)] were identified retrospectively. Clinical, serological, and laboratory features were compared. A comparison between triple and quadruple seronegative pSS patients was also performed. We included 184 patients (168 women, 16 men) with a mean age at diagnosis of 50.1±13.1 years. The most common subjective presenting features at the time of the diagnosis were dry mouth (94.5%) and dry eye (91.3 %). ANA positivity was 57.0%, and RF positivity was 30.4%. Salivary gland enlargement, arthritis, Raynaud's phenomenon, vasculitis, interstitial lung disease (ILD), neurological involvement, primary biliary cholangitis (PBC), lymphopenia, and thrombocytopenia were observed in ANA+ and RF+ patients but not in seronegative patients (p<0.0001). Arthritis was observed most frequently in RF-positive patients and secondly in ANA-positive patients, whereas arthritis was not observed in seronegative patients (p<0.0001). Autoimmune thyroiditis was present in 65 patients (35.0%), 84.6% of these patients were ANA positive while 12.3% were ANA negative (p=0.0014), RF positivity was 30.7% while RF negativity was 6.15% (p=0.001), 23.0% were both ANA and RF positive while 12.3% were seronegative (p<0.002). Cryoglobulinemia, renal disease, and lymphoma were not observed in any of the patients. We confirm the strong influence of immunological markers on the phenotype of primary SS at diagnosis.

Diffuse Lymphocytic Infiltration Syndrome (DILS) as a Mode of Revelation of HIV: On a Case

Diffuse Lymphocyte Infiltration Syndrome (DILS) is a rare condition observed in HIV patients, characterized by infiltration of CD8+ T lymphocytes in several organs. In this article, a case of revelation of HIV infection is presented, diagnosed following bilateral parotidomegaly associated with DILS. The patient, aged 60, presented a progressive deterioration in general condition, significant asthenia, weight loss, and local symptoms including bilateral parotid swelling, cervical lymphadenopathy, and intermittent fever. Investigations revealed HIV-1 seropositivity, lymphocytosis with high T-CD8+ levels, salivary gland enlargement on imaging, and benign lymphoepithelial lesions on parotid biopsy. The diagnostic criteria for DILS were met, highlighting the importance of salivary gland involvement in this syndrome. Treatment with HAART resulted in significant regression of symptoms. In summary, DILS represents a multisystem complication of HIV, requiring appropriate management including antiretroviral treatment to improve patient prognosis.

Unusual upper lip swelling: A review and a case report of cheilitis landularis

Cheilitis glandularis (CG) is a rare inflammatory condition characterized by chronic enlargement and inflammation of the minor salivary glands in the lip. This article provides a comprehensive review of CG, including its clinical features, subtypes, and potential predisposing factors. Additionally, a case report is presented to illustrate the diagnostic challenges and management strategies associated with this uncommon disorder. CG primarily affects the lower lip, leading to symptoms such as lip swelling, persistent inflammation, dryness, and ulceration. While the exact etiology remains elusive, factors such as infections, obstructions, and autoimmune processes are believed to play a role in CG development. Although the exact relationship is not well-established, stress and psychological factors may influence immune functioning and contribute to the exacerbation or recurrence of inflammatory conditions, including CG. The case report highlights an unusual upper lip swelling that was diagnosed as CG. This underscores the importance of thorough clinical examination, differential diagnosis, and histopathological evaluation in accurately identifying CG. Timely recognition and appropriate management are essential for optimizing patient outcomes and preventing potential complications. In conclusion, CG presents a diagnostic challenge due to its diverse clinical presentation. Clinicians should maintain a high index of suspicion when encountering lip swelling and consider CG in the differential diagnosis. Further research is needed to elucidate the underlying mechanisms and potential predisposing factors associated with CG, including the role of mental health. Increased awareness and understanding of this condition will facilitate early detection, appropriate management, and improved outcomes for individuals affected by CG.

Granulomatosis with polyangiitis with salivary glands involvement: Presenting a case and describing its clinical, pathophysiological, and therapeutic aspects

Key Clinical MessageGranulomatosis with polyangiitis (GPA), a rare form of small vessel vasculitis, may be manifested by multisystem involvement misleading its definitive diagnosis. The involvement of salivary glands is a very rare characteristic of GPA. Herein, we described a case of GPA with submandibular salivary gland involvement followed by reviewing the literature on similar cases. The case was a 31‐year‐old man, a known case of seronegative peripheral arthritis that referred recently with bilateral enlargement of the parotid and submandibular glands. Pulmonary nodules were also evident in the patient's CT scan. Fine‐needle aspiration under ultrasound guidance indicated the presence of degenerated squamoid cells, giant cells, and inflammatory cells with a priority of neutrophils in the submandibular gland, as well as the presence of a cyst containing fluid without the evidence of malignancy in the parotid gland. The positivity for the Anti‐neutrophil Cytoplasmic Antibody (C‐ANCA) marker was also revealed. The patient was treated with methotrexate, prednisolone, and rituximab which led to a gradual reduction in the size of the glands and the improvement of the patient's clinical symptoms within 1 month after the treatment. Enlargement of salivary glands in the context of inflammatory disorders can raise doubts about the existence of GPA, and therefore imaging evaluation and histopathological assessment with an ANCA test will be necessary to confirm or rule out it.

Современный метод хирургического лечения стриктуры протока околоушной слюнной железы

INTRODUCTION: A modern principle of treatment for obstructive pathologies of salivary glands is saving the gland itself and its functions using minimally invasive methods. This article describes a clinical case of elimination of the stricture of the parotid salivary gland duct and compensation for its expanded section behind the gland with intraoperative visualization using ultrasound. Under US control, the expanded portion was punctured from external access; a conductor was inserted, which passed into the oral cavity through the natural orifice. Through the formed opening, a catheter was installed, which expanded the narrowed area and prevented the reappearance of the stricture. Control examinations in 1, 6 and 12 months showed the functioning of the orifice with release of clear saliva in the satisfactory amount. The patient noted absence of enlargement of the parotid salivary gland and absence of pain at rest and in chewing. Control US in 6 and 12 months showed absence of any signs of stricture and reduction of the orifice diameter to 2 mm. CONCLUSION: The developed method of elimination of stricture of the parotid salivary gland duct can lead to reduction of duration and the scope of surgical intervention, of the risk of re-stricture, postoperative and rehabilitation periods, frequency of using costly equipment, which makes the procedure available.

TUBERCULOUS SIALADENITIS: A CASE SERIES

Objective Describe cases of tuberculous sialadenitis. Study Design The cases were diagnosed in a southern Brazil hospital. Demographics and clinical characteristics were gathered. Results Four cases (two men and two women) with a mean age of 43.2 years were included. The clinical presentation was a painless (n=3) mass in salivary gland (two in parotid and two in submandibular gland), with an evolution time of one to four months. Cutaneous ulceration was observed in one patient. The histological analysis of the resected gland (three cases) yielded a definitive diagnosis of tuberculosis. However, in one case, the diagnosis was based on fine needle aspiration followed by incisional biopsy. All patients were referred to an infectologist. Conclusion Tuberculosis sialadenitis involving major salivary gland is uncommon with a myriad of clinical presentations. In countries in which tuberculosis is endemic, suspicion of this lesion is a prerequisite for major salivary gland enlargement. Describe cases of tuberculous sialadenitis. The cases were diagnosed in a southern Brazil hospital. Demographics and clinical characteristics were gathered. Four cases (two men and two women) with a mean age of 43.2 years were included. The clinical presentation was a painless (n=3) mass in salivary gland (two in parotid and two in submandibular gland), with an evolution time of one to four months. Cutaneous ulceration was observed in one patient. The histological analysis of the resected gland (three cases) yielded a definitive diagnosis of tuberculosis. However, in one case, the diagnosis was based on fine needle aspiration followed by incisional biopsy. All patients were referred to an infectologist. Tuberculosis sialadenitis involving major salivary gland is uncommon with a myriad of clinical presentations. In countries in which tuberculosis is endemic, suspicion of this lesion is a prerequisite for major salivary gland enlargement.

Integrative approach to diagnosis of lymphoproliferative disease during long-term Sjogren’s disease. Clinical observation

During long-term autoimmune processes, most commonly in patients with Sjogren’s disease (SD), lymphoproliferative disorders (LPD) can develop. The risk of mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) with involvement of parotid salivary glands is increased 1,000-fold compared to other immunomorphological variants. Some studies show that the main factors predisposing patients with SD to LPD are long-term enlargement of parotid salivary glands, generalized lymphadenopathy, cryoglobulinemic purpura. Application of an integrative approach to LDP diagnosis in patients with SD, identification of high-risk group for this malignant neoplasm promote its early detection.A clinical observation of a female patients with long-term SD which was diagnosed along with MALT lymphoma using an integrative approach to diagnosis of both diseases is presented.

Sjögren's syndrome.

Sjögren's syndrome (SS) is characterised as keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth) commonly associated with salivary gland enlargement, and is referred to as Primary Sjögren's syndrome. It is known as Secondary Sjögren's syndrome when it occurs in patients, with connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, polyarthritis nodosa, polymyositis, and systemic sclerosis. SS has also been associated with chronic graft-versus-host disease after allogeneic bone marrow transplantation, human immunodeficiency syndrome (AIDS), hepatitis C infection (HCV), chronic biliary cirrhosis, neoplastic and myeloplastic syndromes, fibromyalgia, and chronic fatigue syndrome.

Orofacial manifestations in Brazilian people living with HIV/AIDS under long-term antiretroviral therapy: a cross-sectional study

The aim of this study was to assess the prevalence of orofacial manifestations in a Brazilian cohort of people living with HIV/AIDS (PLWHIV) using long-term combined antiretroviral therapy (cART) and to correlate the presence of these manifestations with clinical and laboratory characteristics. A cross-sectional observational study evaluated 101 Brazilian PLWHIV. Demographic characteristics, medical history, and laboratory data were collected. Physical examination and measurement of stimulated salivary flow were performed. The study included 101 participants who were mainly male (61%) and White (73%), with an average age of 48 years, using long-term cART. The most common oral manifestations were facial lipoatrophy (33%), xerostomia (30%), and salivary gland enlargement (12%). Facial lipoatrophy was linked to a longer duration of cART use (P=.002), whereas hairy leukoplakia was linked to a detectable viral load (P=.031). The salivary flow of <0.7 mL/min was associated with an HIV infection time >20 years (P=.023). People living with HIV/AIDS who use cART often experience facial lipoatrophy, xerostomia, and bilateral enlargement of the parotid glands. Although opportunistic infections and malignant neoplasms are not frequent occurrences, they can still arise.

IgG4 related disease: a novel association with hypertrophic osteoarthropathy

IgG4 related disease (IgG4-RD) is a recently recognized disease that has several associated clinical phenotypes including autoimmune pancreatitis, sclerosing cholangitis, major salivary gland enlargement (Mikulicz disease), orbital pseudotumor, retroperitoneal fibrosis, and inflammatory vascular disease manifesting as aortitis or coronary artery calcification. Histopathology of affected organs shows lymphoplasmacytic infiltrations with a predominance of IgG4-positive plasma cells in the affected tissue and an increased number of eosinophils. Serum IgG4 levels are elevated in two-thirds of patients, and IgE is also often elevated. We report here the first known associated case of presumed IgG4-RD with hypertrophic osteoarthropathy. Hypertrophic osteoarthropathy (HOA) is frequently a paraneoplastic disease of bone, but HOA can be associated with chronic infection, chronic pulmonary disease, cardiac shunts, and cirrhosis. HOA is a clinical diagnosis consisting of arthralgia, digital clubbing, and periostosis of tubular bones seen on imaging with or without synovial effusion. Our 35 yo male LatinX patient presented with typical clinical symptoms of HOA that were confirmed on imaging. During the negative work up for malignancy, disproportionate-for-age coronary artery calcification was identified, which prompted an evaluation for IgG4-RD. The patient also had lung disease (peribronchial thickening), atopic dermatitis, subtle clubbing, elevated IgE, elevated IgG4 (9× normal), and eosinophilia. The patient's symptoms and inflammatory markers improved on prednisone and methotrexate, but a decision to treat with rituximab was made after having to discontinue methotrexate for transaminitis. Rituximab has been used in patients with IgG4-RD with some success, and our patient continues to improve in his skin, respiratory, and bone symptoms with reduction in IgG4, inflammatory markers, and bone turnover markers (alkaline phosphatase).

The clinical and immunological characteristics related to salivary gland enlargement in primary Sjögren’s syndrome: a retrospective cross-sectional study

Salivary gland enlargement (SGE) is one of the common manifestations in primary Sjögren's syndrome (pSS) patients who are first referred to the hospital of stomatology. Whether the characteristics of the pSS patients with SGE differ from those of the ones without SGE remains unclear. Therefore, this study aimed at investigating the clinical and immunological characteristics related to SGE in pSS, to provide a comprehensive understanding of the clinical phenotype of pSS with SGE. In this retrospective cross-sectional study, medical records of patients diagnosed with pSS from 2016 to 2021 were evaluated. The included patients were divided into the SGE and non-SGE groups. Patient data including general clinical data, radiographic and B-ultrasound examination data, and immunological data were extracted. Intergroup differences were analyzed using the chi-square test, Fisher's exact test, and non-parametric tests with SPSS 23.0. Binary logistic regression analysis was further performed to determine the factors related to SGE in pSS. Two hundred and three patients with pSS were included, including 126 and 77 patients with and without SGE, respectively. Univariate analysis showed that compared to the non-SGE group, the SGE group was younger, had dry eye symptom for a longer duration, and had a higher proportion of patients with severe conditions on salivary gland radiography (P<0.05). Regarding immunological indicators, the levels of anti-Ro52, anti-SSA (Ro60), and anti-SSB antibodies; immunoglobulin (Ig)G; IgA; and rheumatoid factor (RF) and erythrocyte sedimentation rate (ESR) were significantly higher in the SGE group (P<0.05). Binary logistic regression analysis showed that younger age and high anti-Ro52 levels were independent factors related to SGE in pSS. SGE is highly consistent with increased immunological indicators, reflecting disease activity. pSS patients with SGE were younger than those without. Special attention should be paid to the changes in the anti-Ro52 level since it is an independent factor related to SGE in pSS.

Current concepts of Kimura disease: pathophysiology and evolution of treatment.

Kimura disease (KD) is a chronic inflammatory disorder that frequently involves the subcutaneous tissue of the head and neck regions. It often manifests as regional lymphadenopathy or salivary gland enlargement in the affected area. The histologic architecture of the lymph nodes in KD patients is preserved, while the affected tissues show follicular hyperplasia and eosinophilic infiltration. No single modality has been adopted as an optimal treatment for KD. This article concentrates on the fundamental features of KD and reviews current approaches to its treatment.

The clinical phenotype of primary Sjögren's syndrome patients with lymphadenopathy.

Previous cohort studies have shown that around 10% of patients with primary Sjögren's syndrome (pSS) develop lymphadenopathy during their disease course. However, no studies have described their clinical phenotype. The present study aims to describe the clinical manifestations and laboratory findings of pSS patients presenting long-standing lymphadenopathy. From a total of 1234 consecutive pSS patients fulfilling the 2016 ACR-EULAR criteria, those with stable lymphadenopathy unrelated to lymphoma were identified (lymphadenopathy group). Their clinical data were collected and compared with 2 control groups: a) the remaining unmatched pSS patients without lymphadenopathy (unmatched non-lymphadenopathy group) and b) pSS patients without lymphadenopathy matched for age, sex, and disease duration, in an approximately 1:1 ratio (matched non-lymphadenopathy group). One hundred and sixty-five (13.37%) patients presented persistent, stable lymphadenopathy. They were characterised by younger age at both pSS onset and diagnosis, and by shorter disease duration. Compared to the unmatched nonlymphadenopathy group, patients with lymphadenopathy had more frequently salivary gland enlargement (p<0.001), higher focus score at first salivary gland biopsy (p=0.017), palpable purpura (p<0.001), peripheral nervous system involvement (p=0.012), glomerulonephritis (p<0.001), and leukopenia (p<0.001), while the results of the matched comparison were similar. Regarding the serological profile, the comparison with the unmatched group demonstrated higher frequency of ANA (p=0.013), anti-Ro/SSA (p=0.001), and anti-La/SSB (p<0.001) positivity for the lymphadenopathy group, while in the matched comparison only higher rates of anti-Ro/SSA positivity (p=0.002) remained statistically significant. pSS patients presenting non-lymphoma related stable lymphadenopathy constitute a subgroup of younger individuals with B-cell hyperactivation.

Difficult-to-treat rhinosinusitis in IgG4-related disease

Introduction: IgG4-related disease is a rare, immune disease with hyper-IgG4-gamma-globulinemia and fibro-inflammatory lesions that can occur in any organ. The diagnosis is made on the basis of clinical and histopathological criteria. In otolaryngological practice, the IgG4-related disease should be taken into account in the differential diagnosis of patients with salivary or lacrimal gland enlargement, with eyelid edema and chronic rhinosinusitis. Systemic glucocorticosteroids are the first line of treatment and can be combined with other immunosuppressants or biological treatment. The prognosis is favorable, but delay in diagnosis and therapy can lead to multiple organ failure and premature death. Case study: The paper presents a case report of patient initially treated for allergic rhinitis with asthma and eyelid edema, accompanied by rhinosinusitis, and history of numerous comorbidities (Hashimotos disease, parotid tumor, idopathic hepatitis, anemia and albinism). Imaging studies confirmed enlargement of the salivary and lacrimal glands with the upper eyelids edema and rhinosinusitis. The patient was qualified for sinus surgery. Postoperative histopathological examination enabled the diagnosis of IgG4- -related disease. For the next 5 years, the patient received prednisone and azathioprine, then prednisone and methotrexate, inhaled and nasal steroids, under constant specialist care, including otolaryngologist. Eyelid oedema resolved, asthma and allergic rhinitis were well controlled. Despite treatment, sinus symptoms gradually worsened, accompanied by headaches. The mucocele of the left frontal sinus was diagnosed and qualified for sinus reoperation. In the postoperative period, despite intensive treatment, mucocele of the right frontal sinus developed. Finally, another radical surgery on the frontal sinuses was performed (Draf III). The patient also showed other signs of progression of IgG4-related disease, including a twofold increase in serum IgG4 concentration. The lack of full control of the disease, manifested by difficult to treat rhinosinusitis, became the basis for qualification of the patient, now 44 years old, to biological treatment with rituximab.

P40 Dry eyes and a dry mouth: is it Sjögren’s syndrome?

P40 Dry eyes and a dry mouth: is it Sjögren’s syndrome?