Bilateral Parotid Enlargement Research Articles

Primary Sjogrens Syndrome in a Child.

Primary Sjogrens Syndrome in a Child.

SP0098 Etiology of SICCA Syndrome: the Forgotten Causes

A 54-year old man was referred in 2010 to our outpatient clinic with a 4-year history of dry mouth and eyes and diffuse arthralgias. Review of symptoms was essentially negative....

AB0887 Autologous Stem-Cell Transplantation (ASCT) as Treatment Option in A Child with Severe Systemic Sclerosis

BackgroundClinical presentation of systemic sclerosis in overlap syndrome with severe skin involvement can be very severe while standard treatment could be ineffective with necessity to apply autologous stem-cell transplantation (ASCT).ObjectivesAfter...

Bilateral Parotid Gland Enlargement and Palpable Nephromegaly in Infant Acute Lymphoblastic Leukemia

Acute lymphoblastic leukemia (ALL) in infants below 1 year of age accounts for 2.5% to 5% of childhood ALL. Most children with ALL present with fever, bruising, mucosal bleeding, bone pain, pallor, hepatosplenomegaly, and lymphadenopathy. Common sites of extramedullary involvement at diagnosis include liver, spleen, lymph nodes, brain, and testes. Nephromegaly has also been reported. We present a novel case of bilateral parotid enlargement along with bilateral palpable nephromegaly in a patient with newly diagnosed infant ALL. This unique presentation highlights the importance of considering ALL in the differential diagnosis of parotid enlargement especially when associated with abnormal blood counts.

1174

Introduction: Introduction End organ dysfunction related to infective endocarditis (IE) is a common cause for the referral to the intensive care unit. A thorough history and physical examination remains an integral part of management of patients with IE. Intensivists should maintain a high index of suspicion towards detecting rare manifestations of IE. We present a case of Methicillin sensitive Staphylococcus aureus (MSSA) endocarditis with two such rare complications in the form of bilateral endophthalmitis and parotitis which were diagnosed early and treated promptly. Case presentation A 63 year old male with a past history of mitral valve annuloplasty was hospitalized for fever with rigors of four day duration. His admission vitals were notable for fever (103 F), tachycardia and tachypnea. Physical examination revealed an apical holosystolic murmur and painful erythematous finger nodules (Osler's nodes).Pertinent positive labs included thrombocytopenia (count of 40,000), elevated BUN (60) and creatinine (1.7). Appropriate antibiotics were initiated for concern of IE; blood cultures grew Methicillin sensitive Staphylococcus aureus (MSSA) and subsequent work up with Trans-esophageal echocardiography showed mitral valve vegetation's (> 2 cm) on the anterior leaflet of the mitral valve with concomitant severe mitral regurgitation. Hospital course was complicated by the development of sudden painful bilateral parotid gland enlargement, bilateral decreased visual acuity and confusion. A thorough visual, neck and neurological exam revealed findings of bilateral endophthalmitis which was emergently treated with vitreous aspiration and intravitreal antibiotic instillation leading to an improvement in vision. Vitreal fluid cultures were positive for MSSA. Bilateral Parotid gland enlargement- acute parotitis secondary to disseminated sepsis was successfully treated with antibiotics and hydration. MRI of brain revealed multiple small cerebral infarcts and small areas of intracerebral hemorrhage consistent with septic infarcts and secondary hemorrhagic conversion. While the risks and benefits of emergent mitral valve replacement surgery were being contemplated, unfortunately only four days into the hospital course patient suffered a massive intracerebral hemorrhage and passed away. Discussion The pathogenesis for complications in IE includes embolic, local spread of infection, metastatic infection or immune-mediated damage. Embolization remains a distressingly common complication of IE and can occur even after appropriate therapy. Endogenous endophthalmitis is a rare complication of infective endocarditis that occurs due to direct invasion of ocular tissues (septic emboli) or by changes in vascular endothelium caused by substrates released during infection. The most common cause of the infection is Staphylococcus aureus. Endogenous endophthalmitis due to MSSA infective endocarditis is an extremely rare complication. Intravitreal and systemic antibiotic therapy should be initiated promptly. Acute bilateral parotitis is rare and is associated with mumps and connective tissue disorders; while in our case it seems to be secondary to MSSA bacteremia and septic emboli. Bilateral Endophthalmitis and Parotitis are very rare complications of infective endocarditis with only a few case reports published to date. Our patient is the first case in literature where both manifestations occurred together as a part of embolic phenomenon. Most common pathogen implicated in the setting of endocarditis is Staphylococcus aureus. Conclusion: Infective endocarditis (IE) is associated with a myriad of complications. It is important to recognize rare complications of IE like bilateral endophthalmitis and parotitis and act rapidly to prevent morbidity associated with it.

Parotid Gland Enlargement in Pediatric HIV Population

To establish the significance of parotid enlargement, state frequency, cytological features and effect of antiretroviral therapy (ART) on parotid enlargement among a pediatric HIV population. A 6 month cross sectional survey that utilised clinical features, serial immunological indices and fine needle aspiration cytology. 287 HIV positive children were seen at special paediatric clinic of the University College Hospital, Ibadan, Nigeria, 114 (39.7%) had oral features and among these 24 (8.4%) had parotid involvement comprising of 10 males and 14 females. The overall mean age was 43.4 + 39.7 months compared with 59.6 + 36.5 months in the parotid enlargement group (p = 0.03). Mean ages of parotid enlargement and non enlargement group was significantly different (p = 0.03). The mode of transmission was vertical in (91.7%), 87.5% was bilateral (87.5%) and (75%) presented as a syndrome state with generalised lymphadenopathy. The predominant cytology was lymphoid hyperplasia (62.5%). ART resulted in marked clinical reduction in all the cases and statistically significant improvement in serum indices of CD4 count, CD4% and viral load (p = 0.001, 0.000 & 0.009 respectively). HIV positive children often present with bilateral parotid enlargement and the syndrome state with classical clinical and cytological features of lymphoid hyperplasia predominated. ART resulted in satisfactory reduction of the swellings in most of the cases with no need for further intervention.

Bilateral Parotid Swelling With Blindness

We report a case of a 32-year-old woman who presented with bilateral parotid gland enlargement. She had no systemic symptoms such as fever, cough, and weight loss. She had bilateral blindness for a long time. The results of a physical examination revealed a bilateral firm, painless mass in the parotid region. A computed tomographic scan showed no clear lymphadenopathy in the neck but showed localized infiltrates and multiple pulmonary nodules with enlargement of the mediastinal and axillary lymph nodes in the chest. The patient underwent a superficial parotidectomy. A histopathologic finding revealed an epithelioid noncaseating granuloma, which is consistent with sarcoidosis. In summary, this current study shows that sarcoidosis should be considered in the differential diagnosis of all painless swelling of the parotid gland, especially in women, which could be an earlier complaint.

An Algorithm Approach to Diagnosing Bilateral Parotid Enlargement

This contemporary review aims to categorize the disease entities that cause bilateral parotid enlargement and to develop a question-based algorithm to improve diagnosis of bilateral parotid masses. A PubMed search for bilateral and parotid showed 818 results. Of these, 68 relevant studies were reviewed to compile a list of disease processes that can cause bilateral parotid enlargement. A total of 22 diseases entities were reviewed. The disease processes were initially grouped into 6 categories based on etiology: sialadenosis, infection, neoplasm, autoimmune, iatrogenic, and miscellaneous. For each lesion, the incidence, history, and physical examination were compiled in a matrix. After reviewing the matrix, it was clear that grouping diseases based on specific history and physical findings limits the differential diagnosis. The most important factors included disease incidence, timing of onset, nodular or diffuse, pain, and overlying skin changes. With this algorithm, the differential diagnosis can be limited from 28 to 7 or fewer likely diagnoses for a given presentation. Implications for Practice Bilateral parotid disease has a wide differential diagnosis with an expanding number of available tests. An algorithm, based solely on data obtained from the history and physical examination in the first patient encounter, may reduce the differential and aid the clinician in deciding on further workup and treatment. Following the algorithm presented here should allow the clinician to arrive at a diagnosis rapidly without ordering unnecessary tests and wasting resources.

Bilateral parotid enlargement following snake bite: A rare sign

Snakebite is a common medical emergency in India. Unusual complications may occur after viper bite. Bilateral parotid enlargement after viper bite is a rare entity. An 18-year old gentleman presented to our hospital with history of viper bite. On examination he had cellulitis of right lower limb. He developed swelling of both the parotid glands 12 h after admission. He developed coagulopathy, acute renal failure and died within 48 h of hospital admission. Development of parotid swelling after snake bite is associated with poor prognosis. This case is found worth reporting as it is an unusual complication having prognostic value.

IgG4 related interstitial nephritis: A case report and review of literature

IgG4 interstitial nephritis is a recently described entity. A middle-aged gentleman with bilateral parotid enlargement, hepatosplenomegaly and generalized lymphadenopathy was referred to us for evaluation of renal failure. He had trace proteinuria and large kidneys. Kidney biopsy revealed interstitial nephritis with characteristic storiform fibrosis. Immunohistochemistry demonstrated intense staining for IgG4-secreting plasma cells in the interstitium.

Bilateral parotitis as the initial presentation of childhood sarcoidosis

The differential diagnosis of bilateral parotid gland enlargement in children includes infectious, inflammatory, and neoplastic disorders. We present the case of a 13-year-old male who presented with a 5-week history of bilateral parotid swelling. On exam, both parotid glands were nontender, smooth, and diffusely enlarged. He had slightly elevated inflammatory markers, but other lab results were normal. A neck CT revealed symmetric enlargement of the parotid, submandibular, and sublingual glands. A chest CT revealed scattered peripheral pulmonary nodules and bilateral hilar adenopathy. A parotid gland biopsy showed multiple noncaseating granulomas with multinucleated giant cells surrounded by lymphocytes, consistent with the diagnosis of sarcoidosis. Special stains for acid-fast and fungal organisms were negative. Using this illustrative case, we discuss the differential diagnosis of bilateral salivary gland enlargement in children and review the etiology, diagnosis, clinical manifestations, and treatment of pediatric sarcoidosis.

Bilateral multicystic parotid masses in primary Sjögren syndrome

We report the case of a 48-year-old woman who presented with bilateral parotid gland enlargement as the initial manifestation of primary Sjögren syndrome. Magnetic resonance imaging demonstrated multiple areas of low-intensity signal mixed with foci of high-intensity signal, a finding suggestive of Sjögren syndrome. Autoimmune blood tests for Sjögren syndrome were positive, and a sublabial biopsy confirmed the diagnosis. Investigations for other conditions, including human immunodeficiency virus infection, were negative. After 5 years of follow-up, the parotid swellings remained essentially unchanged. We discuss the etiology of multicystic parotid gland pathology, its involvement in Sjögren syndrome, and the risk of lymphoma.

Neurosarcoidosis Affecting the Spinal Cord

Sarcoidosis is a chronic inflammatory disease that involves multiple systems. Complications in the nervous system are rare, especially in Asia. This report describes an unusual case of neurosarcoidosis affecting the spinal cord and other systems in a 53-year-old Taiwanese women patient. The patient presented with subacute onset of right arm radicular pain followed by quadriparesis. Magnetic resonance imaging revealed a hyperintense lesion on T2WI in the cervical spinal cord. The patient also had a history of facial neuropathy, bilateral parotid enlargement, intrathoracic lymphadenopathy, hypercalcemia, nephrocalcinosis, and renal function impairment. Parotid gland biopsy showed granulomatous lesion with multinucleated giant cells. Sarcoidosis was diagnosed after other possibilities were excluded. Oral steroid treatment resulted in initial significant clinical improvement. Awareness of the systemic and neurological presentations of sarcoidosis can prompt the correct diagnosis and treatment.

Breast lymphoma in Sjögren’s syndrome complicated by acute monocular blindness

A 69-year-old hypertensive woman presented with eye and mouth dryness, bilateral parotid gland enlargement, associated with anasarca and proteinuria. Family history was notable for malignancies including breast, nasopharyngeal and colon cancers. Physical exam disclosed hypertension, bilaterally enlarged, firm, non-tender parotid glands, fine bibasilar crackles and bipedal edema. Anti Ro/Sjögren's syndrome antigen A antibody was positive, with negative tests for anti La/Sjögren's syndrome antigen B and anti-nuclear antibody (ANA). Chest radiographs showed basal infiltrates. Sjögren's syndrome associated with glomerulonephritis and interstitial lung disease was diagnosed, and she received pulse methylprednisololone followed by oral prednisone with dramatic improvement. Two months later, while on prednisone 5 mg/day, she returned to the clinic with an enlarging fixed non-tender right breast mass. She underwent modified radical mastectomy of the right breast, and pathologic report revealed diffuse, small cell, non-Hodgkin's lymphoma of the breast; axillary lymph nodes were negative for tumor. She opted for alternative therapy and did not return to the clinic until 7 months later when she developed sudden monocular blindness in the right eye with no other systemic manifestations. Magnetic resonance imaging (MRI) revealed swelling and enhancement of intracanalicular and pre-chiasmatic segments of the right optic nerve and right side of the optic chiasm. Considerations were Devic's disease versus metastases. She received pulse methylprednisolone therapy (1 g/day for 3 days) with partial recovery of vision. She is scheduled for lymphoma chemotherapy to include rituximab.

Rosai–Dorfman disease with factor XII deficiency

A 17-year-old female patient presented with chronic symmetrical oligoarthritis of both knees and ankles, xerostomia, xerophthalmia, multiple bilateral lymphadenopathies in the cervical region, and bilateral parotid enlargement with the histological finding of chronic sialoadenitis. She had been already given methotrexate, chloroquine, and corticosteroids with the diagnosis of rheumatoid arthritis (RA) before referral to our outpatient clinic. Because her complaints and the lumps did not remit and she could be classified as neither RA nor primary Sjögren's syndrome (SS) according to 1987 ACR RA criteria or European preliminary criteria for SS, lymph node biopsy was repeated and revealed the diagnosis of Rosai-Dorfman disease (RDD) with the histological findings of histiocytes, phagocyting lymphocytes in enlarged sinuses, and mature plasma cells infiltrating the pulpa. All the medications were stopped after the pathological diagnosis of RDD and consulting with the Division of Hematology. She was reevaluated with magnetic resonance imaging, which showed dense infiltrative areas around knee and ankle joints, and computed tomography that showed a soft tissue mass surrounding the descending aorta and upper part of the abdominal aorta. Activated partial thromboplastin time was found to be prolonged in prebiopsy examinations, and factor XII deficiency was detected after detailed hematological evaluation. The symptoms of joint involvement were relieved with nonsteroidal antiinflammatory drugs. She has been followed-up without medication without obvious clinical or laboratory change. We herein report a patient with RDD mimicking RA and SS. We consider that RDD should be kept in mind especially in patients with resistant symptoms to conventional therapies, younger disease onset, and predominant parotid and lymph node enlargement.

A 41-year-old Female Presenting with Sicca Syndrome

Objective: To describe a case of Sicca syndrome, review the literature on and discuss the diagnostic criteria for Sjögren Syndrome.
 Methods: 
 Design: Case report
 Setting: Private Clinic 
 Patients: One
 Results: A 41-year-old female presenting with throat dryness, dry eyes, bilateral parotid enlargement, vaginal dryness and multiple joint pains previously seen by four specialists over three decades was finally diagnosed with Sjögren syndrome.
 Conclusion: Sjögren syndrome presents uncommonly. As the presentation and clinical course should be taken as a whole, and no single test can be considered as a gold standard for its diagnosis, the disease entity may actually be under diagnosed, misdiagnosed or frequently present as diagnostic dilemmas. A comprehensive history and physical examination beyond the usual focus of the otorhinolaryngologic evaluation is the key for the astute clinician. Our experience may indicate a need for further evaluation of the EU revised criteria for Sjögren syndrome classification in the local setting.
 
 Keywords: sicca syndrome, sjogren syndrome, keratoconjunctivitis sicca, stomatitis sicca, xerostomia, dysparenuia 

Heerfordt Syndrome with Unilateral Facial Nerve Palsy: A Rare Presentation of Sarcoidosis

Heerfordt syndrome is rare and is characterized by fever, uveitis, parotid gland enlargement, and facial nerve palsy. We hereby present a case of Heerfordt syndrome with unilateral facial nerve palsy as a presentation of sarcoidosis. A 29-year-old male patient from Sri Lanka presented with eye redness OU, blurred vision OD, fever, headache, night sweat, fatigue, and weight loss (5 kg over 1 month). Examination revealed mild anterior uveitis OU, mild vitritis OD, fundus whitish lesions OU, left otalgia, taste disorders, bilateral parotid gland enlargement, and left facial nerve palsy. Work-up for infection or tumour was negative. Chest computed tomography and transbronchial lymph node biopsy set the diagnosis of sarcoidosis. The patient recovered completely within 2 months under therapy with prednisone and azathioprine. One year after onset of treatment, no recurrence was noted. Heerfordt syndrome is a rare manifestation of neurosarcoidosis and has to be included in the differential diagnosis of facial nerve palsy.

Diffuse Infiltrative Lymphocytosis Syndrome (DILS)

AbstractDiffuse infiltrative lymphocytosis syndrome (DILS) is characterised by a persistent CD8+ lymphocytosis and lymphocytic infiltration of various organs. The reported prevalence varies between 0.85–3%, and appears to be more common in Africans. Patients with DILS tend to have higher CD4+ cell counts and survive longer than those patients without DILS. Most patients present with bilateral parotid gland enlargement and features of the Sicca syndrome. Extraglandular involvement is common with the lungs being the most common site, followed by peripheral neuropathy and liver involvement. DILS is a benign presentation in most patients with few complications. Therapeutic trials are lacking although there are isolated reports of good response to antiretroviral and steroid therapy. With the high incidence of HIV in our population it is likely that DILS is under diagnosed probably due to our ignorance of this disease. Awareness of its various presentations may bring to light undiscovered patients with DILS.

Correspondence between clinical improvement and proteomic changes of the salivary peptide complex in a child with primary Sjögren syndrome

The aberrant induction of salivary/lacrimal proteins is considered to be crucial in the pathogenesis of sicca-symptoms related to primary Sjögren syndrome (SS). We report the case of an 11-year-old boy who was admitted to hospital due to recurrent bilateral parotid gland enlargement and keratoconjunctivitis, which were diagnosed as primary SS upon a combination of laboratory and instrumental tests. The proteomic analysis of the salivary peptide complex in the patient's salivary fluid near diagnosis and after 6 months of pharmacological therapy revealed quantitative and mostly qualitative differences. This observation reveals that clinical and functional changes of the salivary glands driven by non-steroidal antinflammatory drugs might be reflected in different proteomic patterns of the salivary fluid.

Severe Pulmonary Hypertension in Pediatric Primary Sjögren Syndrome

A 9-year-old girl with a history of xerostomia and recurrent bilateral parotid gland enlargement presented with purpura of the lower limbs and exertional dyspnea. She had hyperglobulinemia, positive ANA, SSA, and SSB and an abnormal Shirmer test leading to a diagnosis of primary Sjögren syndrome. Cardiologic and imaging investigations revealed severe isolated pulmonary hypertension and ruled out pulmonary fibrosis. Prednisolone and cyclophosphamide, together with anticoagulant and vasodilatory drugs therapy, induced a significant improvement of exertional dyspnea and lowered pulmonary artery pressure. This case and reports from the literature suggest that immune mechanisms, not just vasospasm, can be factors in some pulmonary hypertension.