Mikulicz Disease Mimicking Intraorbital Tumors.

We report a case of Mikulicz disease (MD), an immunoglobulin G4 (IgG4)-related disease that affects the lacrimal and salivary glands. IgG4-related disease is a relatively new clinical entity and is not commonly encountered by neurosurgeons. MD sometimes mimics intraorbital tumors such as malignant lymphoma but responds well to corticosteroid treatment. Thus it is important to recognize the clinical and radiographic features of MD.

A Case Report of Mikulicz Syndrome.

Mikulicz Syndrome (MS) is a rare chronic condition characterized by the abnormal enlargement of glandular tissue in the head and neck. Patients usually present with enlarged lacrimal and parotid glands. While this can be a benign self-limiting condition, other complex systemic diseases, such as sarcoidosis, may represent other underlying etiologies. We present a case of MS in a patient with a history of Crohn's disease.

Gallium SPECT/CT in evaluation of IgG4-related disease: A case report and literature review.

BACKGROUND: The clinical picture of IgG4-related sclerosing disease (IgG4-RSD) may mimic lymphoma, and should be in the differential diagnosis of patients with this clinical picture. CASE SUMMARY: A 32-year-old female had recurrent swelling of both eyelids for more than 15 years. Examination revealed elastic, firm, swollen lacrimal glands about 2-3 cm in diameter that was not painful. Head and orbits magnetic resonance imaging (MRI) showed mass lesions over the bilateral lacrimal glands, submandibular glands, and left foramen of ovale. The differential diagnosis included lymphoid tissue, inflammatory masses, and lymphoma. Gallium single-photon emission computed tomography/computed tomography (SPECT/CT) showed uptake in the bilateral lacrimal glands, right parotid and bilateral submandibular glands, bilateral perirenal region, mediastinal, prevertebral, paraaortic, lumbar, bilateral pelvic (including internal iliac chain) lymph nodes, anterior aspect of right 3rd rib, and lateral aspect of left 6th rib. CT showed multiple enlarged lymph nodes in the mediastinum, right pulmonary hilum, prevertebral space of the thoracolumbar spine, retroperitoneal paraaortic area, bilateral parailiac areas, and bilateral perirenal spaces. Antinuclear and anti-SSA/SSB antibodies were negative, and the serum IgG4 level was 740 mg/dL (normal, 8-140 mg/dL). Right parotid gland biopsy showed abundant IgG4-positive plasma cells. Mikulicz disease (IgG4-related sclerosing disease) was diagnosed and she received glucocorticoid treatment. Follow-up CT and MRI showed with resolved eyelid swelling and perirenal mass lesions. Follow-up gallium scan was normal. CONCLUSION: Gallium SPECT/CT can be a useful tool for initial and follow-up evaluation of IgG4-RSD.

IgG4-related Mikulicz's disease: ultrasonography of the salivary and lacrimal glands for monitoring the efficacy of corticosteroid therapy.

OBJECTIVES: IgG4-related Mikulicz's disease (IgG4-MD) has been recently established as a subtype of IgG4-related diseases involving the salivary and lacrimal glands, and the diseased glands are characteristically and highly responsive to corticosteroid therapy. We retrospectively evaluated ultrasonography (US) of the salivary and lacrimal glands for monitoring the efficacy of corticosteroid treatment in patients with IgG4-MD. METHODS: US features of the salivary and lacrimal glands were assessed and compared with the serum IgG4 levels in 8 patients with IgG4-MD before and at various stages after initiation of oral corticosteroids. RESULTS: US features of the lacrimal and salivary glands of patients with IgG4-MD were characterised by multiple hypoechoic areas in enlarged glands. The submandibular glands were most frequently involved by the disease, and bilateral glands of the same type were similarly affected exhibiting the same hypoechoic pattern. Alleviations of abnormal gland architecture and size in response to corticosteroid therapy were effectively detected with US. The US findings of the involved glands were proportional to the serum IgG4 level before and during the corticosteroid therapy. CONCLUSIONS: US helps monitor the efficacy of corticosteroid treatment in patients with IgG4-MD.

"Quasi-symmetric" FDG avidity of bilateral salivary glands in Mikulicz disease yielding false-positive staging in lung cancer.

A 77-year-old man with lung cancer underwent fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT). Cervical FDG uptake of variable intensity was detected corresponding to enlarged masses in the bilateral parotid and left submandibular regions. We suspected metastases from primary lung cancer or coexisting salivary gland neoplasms. Histopathological examination of the FDG-avid parotid mass revealed Mikulicz disease, an uncommon autoimmune disease involving the salivary glands. We present this case of Mikulicz disease with "quasi-symmetric" FDG avidity in the major salivary glands. We believe that salivary FDG uptake can lead to erroneous interpretation of tumor staging using PET.

Utility of FDG PET/CT in IgG4-related systemic disease.

IgG4-related systemic disease (IgG4-RSD) is an emerging clinical entity about which much remains to be elucidated, in terms of its aetiology, pathogenesis, diagnosis, treatment and outcome. Autoimmune pancreatitis (AIP) and Mikulicz disease (MD) are the two major, well-studied constituents of IgG4-RSD. AIP and MD have common characteristics of forming tumour-mimicking lesions that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous immunoglobulin G4 (IgG4)-positive plasma cells, as well as various multi-organ manifestations of IgG4-RSD. 2-[(18)F]-fluoro-2-deoxy-d-glucose positron-emission tomography/ computed tomography (FDG PET/CT) enables the acquisition of whole-body images and provides functional information about disease activity; as such it has a valuable role in staging extent of disease, guiding biopsy, and monitoring response to treatment. However, FDG PET/CT is likely to be only one component of the management strategy, and clinical, laboratory, imaging and histological findings are crucial in the overall diagnosis of the condition. At present FDG PET/CT does not have a well-established role in the assessment of patients with IgG4-RSD and future prospective studies are required to define the cost-effectiveness and clinical impact in this patient group more accurately.

[The first experience of using rituximab in Mikulicz disease].

The paper describes a case of Mikulicz's disease (MD) in a young woman (aged 19 years) with symmetrical large salivary gland lesion concurrent with the enlarged lacrimal glands. Immunomorphological and molecular studies of parotid gland biopsy specimens revealed the formation of MALT tissue without signs of B-cell clonality of an infiltrate. The diagnosis of lacrimal sac lymphoma was ruled out. MD was diagnosed. The use of rituximab in therapy for MD has first demonstrated a positive result in Russian and worldwide practice.

Sonographic diagnosis for Mikulicz disease.

OBJECTIVE: The aim was to investigate the diagnostic imaging characteristics of Mikulicz disease (MD), especially sonographic ones, and to clarify the differences between them and those in Sjögren syndrome (SS), based on new criteria of MD. STUDY DESIGN: The sonographic and sialographic images, as well as clinical, histopathologic, and serologic findings of 9 patients satisfying the new criteria of MD were analyzed and compared with those in SS. RESULTS: All swollen submandibular glands showed bilateral nodal hypoechoic areas with high vascularization on sonograms and a parenchymal defect on sialograms, whereas parotid glands showed normal or slight change on both images. Nodal areas in submandibular gland sonograms were unclear on computerized tomography and on magnetic resonance imaging, but showed accumulation on gallium scintigraphy. CONCLUSION: Mikulicz disease showed a high rate of bilateral nodal change in submandibular glands, which was completely different from SS. For detection and follow-up of these changes, sonography may be the best imaging modality.

F-18 FDG PET-CT findings in Mikulicz disease and systemic involvement of IgG4-related lesions.

A 72-year-old woman with Mikulicz disease with pathogically proven sclerosing sialadenitis showed systemic abnormal F-18 FDG uptake in the bilateral lacrimal and submandibular glands, pancreas, abdominal aortic wall, and a retroperitoneal fibroid mass on PET/CT scan, with marked elevation of the serum IgG4 level. This case supports Mikulicz disease being included as 1 of the disorders associated with a new clinical entity of systemic IgG4-related plasmacytic syndrome. A whole-body FDG-PET/CT scan can be expected as a useful tool for detecting systemic involvement in systemic IgG4-related plasmacytic syndrome.

Diffuse periairway thickening in patients with Mikulicz disease.

A 70-year-old woman presented with dry mouth, bilateral swelling of the eyelids, and abnormal taste and smell sensations that had persisted for 3 years. She was diagnosed with Mikulicz disease and presented with dyspnea on exertion afterwards. Chest computed tomography and magnetic resonance imaging showed wall thickening of the trachea and bilateral bronchus. Transbronchial needle aspiration showed lymphoproliferative lesion in the tracheobronchus. The patient was treated with corticosteroid, which improved all of her clinical symptoms, computed tomography, and magnetic resonance findings. In this case, we presented a rare condition of coexistent Mikulicz disease with tracheobroncial wall thickening caused by lymphoproliferation without lesions in small airways or lung.

Mikulicz syndrome and disease: 2 case reports highlighting the difference.

We present 2 case reports highlighting the essential differences between Mikulicz syndrome and Mukulicz disease. A review of the literature appears to show some confusion over the terminology and we therefore also present an historical account of the evolution of the two terms and review their relative merits and relationship with other disorders. In conclusion, we would not advocate the use of the term Mikulicz syndrome and suggest that Mikulicz disease be replaced by the title 'Benign Lymphoepithelial Lesion'.

Evaluation of ultrasound in the diagnosis of pathologic processes in the parotid gland.

The values of ultrasound in differentiation of pathologic processes in the parotid gland were studied in 61 patients. The ultrasound findings were compared with operative and histologic findings. In 28 patients with chronic inflammatory processes, the ultrasound findings were in complete correlation with the histologic findings, showing enlarged parotid glands, homogenous glandular epithelium, and small, multiple cysts. In 31 patients with a tumor of the parotid gland, ultrasound scanning detected the presence of the tumor. In determining the location of the tumor, the method is highly specific when it is localized in the superficial lobe (100%), and it is less specific when it is localized in the deep lobe of the parotid gland. Also, it is highly specific in determination of tumor size (96%). In two cases with retention cysts, the ultrasound findings also correlated with the histologic and operative findings. The results obtained show that ultrasound is a reliable diagnostic method for detection of pathologic changes in the parotid gland.

[Diagnosis of periorbital diseases by means of ultrasound]. / Dijagnostika paraorbitalnih oboljenja ultrazvukom.

The ophthalmology was one of the first medical branches which used ultrasound in diagnostical purposes. The structure of the eye, with the large vitreous body, through which the ultrasound waves pass without attenuation, is highly appreciated in ultrasound examination, thus the diagnostical results of ocular diseases are extremely good. On the base of our rich ophthalmological experience we left the scope of the eye and the orbit, and made ultrasound examinations of the head and the face. The echograms of two patients presented original patterns: one was malignant melanoma of the skin with typical echograms performed by A and B scannings, and the other case was the Mikulicz disease with typical echograms. Original echograms of these patients can be considered as a contribution to ultrasound diagnosis of paraorbital diseases.

Appearance of Ga-67 citrate scanning in a patient with Mikulicz's syndrome associated with non-Hodgkin's lymphoma. A case report.

The case of a patient with Mikulicz's syndrome and associated non-Hodgkin's lymphoma is described. Description of the Ga-67 scan in Mikulicz's syndrome is presented.

Manifestations of parotid gland enlargement: radiographic, pathologic, and clinical correlations. Part I: The autoimmune pseudosialectasias.

Histologic, clinical, and radiographic presentations of the autoimmune salivary gland diseases are reviewed. The punctate and globular sialographic changes observed actually reflect penetration of contrast material through the uniquely diseased glandular ducts and not sialectasis, as was previously thought. "Pseudosialectasis" is suggested as a more accurate term. The progressive cavitary and destructive patterns seen on sialography appear to reflect complications of secondary infection rather than the specific pathology of these diseases. Conditions causing recurrent enlargement of the parotid gland or development of a multinodular gland include chronic sialadenitis, the sialoses, the granulomatous diseases, primary neoplasms, and metastatic tumors. Although they appear similar clinically, many of these diseases can be differentiated sialographically, and such a radiographic approach is presented.