SSA Antibodies Research Articles

Sialendoscopy for recurrent parotitis associated with Sjogren syndrome: A case report

Objectives To describe the sialendoscopic findings for a pediatric patient with recurrent bilateral parotitis associated with Sjogren syndrome and to assess the therapeutic potential of sialendoscopy in this patient population. Study design Illustrative case report and literature review. Results A 13-year-old female presented with recurrent bilateral parotitis which was increasing in frequency and affecting her overall health and quality of life. Preoperative imaging demonstrated microcalcifications of her bilateral parotid glands. She was found to have a positive SS-A antibody and was diagnosed with Sjogren syndrome. Sialendoscopy was then performed and findings included thick sludge in Stenson's duct with no salivary stones or duct anomalies identified. The ducts were flushed with saline and steroids. Follow-up has demonstrated no further episodes of recurrent parotitis after 6 months. Conclusions Sjogren syndrome should be considered in all patients with recurrent bilateral parotitis. Sialendoscopy appears to be a potentially valuable diagnostic and therapeutic tool for pediatric patients with Sjogren syndrome.

Primary Sjögren syndrome in a 2-year-old patient: role of the dentist in diagnosis and dental management with a 6-year follow-up

BACKGROUND. Primary Sjögren syndrome is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around 10 years of age. Diagnosis during childhood is difficult, especially because of the diversity of the clinical presentation and difficulty obtaining reliable history data, accounting for a higher frequency of underdiagnosed cases. Differential conditions should be considered, especially the ones that promote xerostomia, such as diabetes, ectodermal dysplasia, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, sarcoidosis, lymphoma, HIV and HTLV infection. Conditions associated with parotid enlargement should also be excluded, including juvenile recurrent parotitis (JRP), sialadenosis, sarcoidosis, lymphoma, infectious parotitis caused by streptococcal and staphylococcal infections, viral infections (paramyxovirus, Epstein-Barr virus, cytomegalovirus, and parvovirus), and diffuse infiltrative lymphocytosis syndrome (associated with HIV infection), and rare congenital conditions, such as polycystic parotid disease. CASE REPORT. A paediatric female patient was referred to our clinic for dental treatment complaining about dry mouth, oral discomfort, and dysphagia. The patient presented five of the required criteria to establish the diagnosis of pSS, including ocular symptoms, oral symptoms, evidence of keratoconjunctivitis sicca, focal sialadenitis confirmed by minor salivary gland biopsy, and evidence of major salivary gland involvement. Our patient did not have positive SS-A and SS-B autoantibodies. According to the literature, about 29% of individuals with pSS can present seronegativity for SS-A (anti-Ro) antibodies and about 33% can present seronegativity for SS-B (anti-La) antibodies. CONCLUSION. To the best of our knowledge, this is the youngest patient reported in the scientific English literature with pSS. Primary Sjögren syndrome has a wide clinical and immunologic spectrum and may progress with increased morbidity. Clinicians must be aware of the development of pSS in such an early age and exclude all possible differential findings to provide early diagnosis and treatment.

Prognostic factors for the clinical severity of keratoconjunctivitis sicca in patients with Sjögren's syndrome

AimsTo determine whether prognostic immunological profiles predict the severity of keratoconjunctivitis sicca (KCS) in patients with Sjögren's syndrome (SS).Methods121 patients diagnosed with SS and followed for at least 1 year...

Lupus, “Rhupus” and “Sjrupus”

To the Editor: We offer the following comments on the recent article by Baer, et al 1. Overlap of systemic lupus erythematosus (SLE) and Sjögren’s syndrome (Sj) certainly does occur and we are pleased that this has now been recognized and characterized. We wish to add our observations suggesting that this overlap, which we refer to as “sjrupus,” responds well to rituximab. Patient 1. A woman who in 2000 at age 28 years developed autoimmunity characterized by sicca syndrome with positive SSA and SSB antibodies. There were lupus-like skin changes of discoid disease without SLE antibodies. Hydroxychloroquine (HCQ) was efficacious for arthralgia and later leflunomide was somewhat efficacious for lachrymal and salivary disease. In July 2005, coincidental with administration of Depo Provera, she developed severe bulky polysynovitis, lupus rashes on the face, malaise, and fatigue. Anti-dsDNA antibodies were off-scale positive along with SM, rheumatoid factor (RF) in IgA, M and G subclasses, SSA … Address correspondence to Dr. Handler; E-mail: rphandler{at}gmail.com

Fetal Echocardiographic Assessment of Endocardial Fibroelastosis in Maternal Anti-SSA Antibody-Associated Complete Heart Block

There are few reports describing the features of maternal anti-SSA antibody-associated congenital complete heart block (CCHB) patients developing endocardial fibroelastosis (EFE). The aim of this study was to describe the clinical features and the outcome of patients with CCHB, with or without EFE. Over a 20-year period, 12 consecutive patients diagnosed with maternal anti-SSA antibody-associated CCHB were identified. The maternal anti-SSA antibody levels were measured and fetal echocardiographic findings were reviewed. The ratios of the thickness of the endocardium to that of the whole wall of the left ventricle (LE/W) and right ventricle (RE/W) were measured to investigate the degree of endocardial thickening. A total of 7 patients survived (living group) and were not diagnosed as having EFE. The remaining 5 patients died and were diagnosed with EFE during autopsy (dead group). Fetal echocardiography of the patients showed differences in the thickening and hyperintensity of the endocardium. The RE/W value was significantly higher in the dead group than in the living group. The titers of both maternal anti-52-kDa and anti-60-kDa SSA antibodies were high, but showed no significant differences between the 2 patient groups. EFE was the major negative prognostic factor for CCHB. Myocardial damage, predominantly in the right ventricle, was related to the outcome of CCHB associated with EFE.

Fluorodeoxyglucose positron emission tomography findings in NMDA receptor antibody encephalitis

A 28-year-old woman presented with a seven day his-tory of insomnia, and changes in mental status. She was agitated, confused with visual hallucinations and com-bative behaviour. On day 4, she developed partial com-plex seizure with impairment of awareness and automa-tisms of lip smacking. During the first 2 weeks, she had become increasingly drowsy and confused, her level of conciousness was severely impaired, with no response to painful stimuli, and orofacial-limb dyskinesias appeared. These symptoms and central hypoventilation led to her being sedated on mechanical ventilation for 4 weeks. On day 31 after admission, a cardiac arrhythmia, character-ized by alternating repeated episodes of tachycardia and bradycardia was observed.The serum did not contain anti-nuclear, double-strand DNA, SS-A or thyroid antibodies. Tumor markers were normal. Lumbar pucture on day 8 after the onset of symptoms showed lymphocytic pleocytosis (22/mm

The Benefits of Transplacental Treatment of Isolated Congenital Complete Heart Block Associated with Maternal Anti-Ro / SSA Antibodies: A Review

Isolated congenital complete atrio-ventricular block (CAVB) is associated with the transplacental passage of maternal autoantibodies directed to foetal Ro/SSA ribonucleoproteins. Their interactions most likely trigger the inflammation of the atrio-ventricular node and the myocardium in susceptible foetuses. The inflamed tissues may then heal with fibrosis that may cause heart block, endocardial fibroelastosis, and dilated cardiomyopathy. CAVB, the most common cardiac complication, typically develops between 18 and 24 gestational weeks. Untreated, the condition carries a significant mortality risk as the foetus needs to overcome the sudden drop in ventricular rate, the loss of normal atrial systolic contribution to ventricular filling, and perhaps concomitant myocardial inflammation and fibrosis. The rationale to treat a foetus at the stage of CAVB is primarily to mitigate myocardial inflammation and to augment foetal cardiac output. Maternal dexamethasone administration has been shown to improve incomplete foetal AV block, myocardial dysfunction, and cavity effusions. Beta-sympathomimetics may be useful to increase the foetal heart rate and myocardial contractility. Published data from our institution suggest an improved survival >90% if maternal high-dose dexamethasone was initiated at the time of CAVB detection and maintained during the pregnancy and if a beta-adrenergic drug was added at foetal heart rates below 55 beats/min. Despite the improvement in outcome, there is an ongoing debate about treatment-related risks. In this review, we will appraise the natural history of untreated CAVB, discuss currently available management options, and examine the results and risks of in-utero treatment of antibody-mediated CAVB.

SSA and SSB antibodies are important in the formation of circulating immune complexes in SLE

The authors have earlier demonstrated that the amounts of circulating immune complexes (CIC) are higher in anti-SSA positive as compared to anti-SSA negative systemic lupus erythematosus (SLE) patients. As the...

Antisynthetases syndrome associated with right heart failure

Cardiac involvement is a complication of end stage polymyositis with left heart insufficiency reported to be the most frequent manifestation. We here describe an unusual clinical presentation of antisynthetases syndrome, beginning with right-sided cardiomyopathy associated with right heart failure. A 26 year-old Caucasian male experienced a 6-month clinical course of polyarthritis, fever, sweats, and myalgia. Laboratory studies showed elevated C reactive protein, elevated sedimentation rate, and myolysis associated with anti SSA and anti JO1 antibodies. Electromyography showed a myopathic pattern. Muscle biopsy confirmed the diagnosis of polymyositis. Chest X ray, chest scan, and cardiac echography were normal. One week after hospital admission, the patient developed acute right heart insufficiency, and magnetic resonance imaging showed a right ventricular myocarditis with myocardial inflammatory thickening. Treatment with corticosteroids rapidly improved both symptoms and biological abnormalities.

Sex Differences in a Murine Model of Sjögren's Syndrome

Sex differences in a NOD.H2(h4) murine model of Sjögren's syndrome were analyzed. Compared to males, female NOD.H2(h4) mice have increased severity of sialoadenitis and have a significantly increased percentage of CD4(+) T cells in salivary gland infiltrates. CD4(+) T cells in female infiltrates produce more Th2 and Th17 cytokines than in males, while males have greater Th1 responses. Females also have enhanced B cell responses, with higher levels of SSA and SSB serum antibodies, and B cell activation factor F (BAFF). Thus, sex has a strong impact on the severity of murine Sjögren's syndrome by affecting the immune mechanisms driving the autoimmune inflammation.

Fetal arrhythmia: Prenatal diagnosis and perinatal management

The importance of managing fetal arrhythmia has increased over the past three decades. Although most fetal arrhythmias are benign, some types cause fetal hydrops and can lead to fetal death. With the aim of improving the outcome in such cases, various studies for prenatal diagnosis and perinatal management have been published. Detailed analysis of the type of arrhythmia in utero is possible using M-mode and Doppler echocardiography. In particular, a simultaneous record of Doppler waveform at the superior venous cava and the ascending aorta has become an important and useful method of assessing the interval between atrial and ventricular contractions. Common causes of fetal tachycardia (ventricular heart rate faster than 180 bpm), are paroxysmal supraventricular tachycardia (SVT) with 1:1 atrioventricular (AV) relation and atrial flutter with 2:1 AV relation. Of fetal SVT, short ventriculo-atrial (VA) interval tachycardia due to atrioventricular reentrant tachycardia is more common than long VA interval. Most fetuses with tachycardia are successfully treated in utero by transplacental administration of antiarrhythmic drugs. Digoxin is widely accepted as a first-line antiarrhythmic drug. Sotalol, flecainide and amiodarone are used as second-line drugs when digoxin fails to achieve conversion to sinus rhythm. Fetal bradycardia is diagnosed when the fetal ventricular heart rate is slower than 100 bpm, mainly due to AV block. Approximately half of all cases are caused by associated congenital heart disease, and the remaining cases that have normal cardiac structure are often caused by maternal SS-A antibody. The efficacy of prenatal treatment for fetal AV block is limited compared with treatment for fetal tachycardia. Beta stimulants and steroids have been reported as effective transplacental treatments for fetal AV block. Perinatal management based on prospective clinical study protocol rather than individual experience is crucial for further improvement of outcome in fetuses with tachycardia and bradycardia.

Anti-La negative cutaneous lupus erythematosus in a newborn

A newborn presented with erythematous lesion over face, which appeared soon after birth. Diagnosis of neonatal lupus erythematosus (NLE) was confirmed by positive anti-Ro SSA antibody and skin biopsy. But anti-La SSB antibody was negative. Her hepatic transaminases were high. But no cardiac manifestations were noted.

シェーグレン症候群に合併した耳下腺ＭＡＬＴリンパ腫例

We report a case of MALT (mucosa-associated lymphoid tissue) lymphoma in the parotid gland. MALT lymphoma, an extranodal low-grade lymphoma arising in the stomach, salivary gland or thyroid gland is caused by chronic inflammation such as chronic gastritis, Hashimoto’s disease and Sjogren syndrome. An optimal therapy has not been established for MALT lymphoma of the salivary gland. The patient was a 68-year-old woman with swelling of the left parotid gland. A left parotid tumor was diagnosed by CT and MRI, and was treated surgically. The postoperative pathological diagnosis of the left parotid tumor was MALT lymphoma: there were centrocyte-like cells, plasma cells with ditcher bodies, and a lymphoepithelial lesion. Immunohistologic examination showed that the MALT lymphoma was positive for CD20, CD79α and bcl-2, and negative for CD3, CD5 and CD10. She felt mouth dryness. The left parotid gland was found to have become atrophic by MRI, and SS-A antibodies were noted on blood testing. Further, pathological tissue typical of Sjogren’s syndrome was noted in her salivary gland operatively. So a diagnosis of Sjogren’s syndrome was made, and MALT lymphoma was believed to have developed from Sjogren’s syndrome. There was no evidence of recurrence 12 months after the operation

Primary Sjögren′s syndrome manifesting as multiple cranial neuropathies: MRI findings

We report a case of primary Sjögren's syndrome presenting with multiple cranial nerve palsies and radiological evidence of cranial pachymeningitis and hypophysitis. A 47-year-old woman developed right sensory neural hearing loss followed, 2 months later, by right facial palsy. Cranial magnetic resonance imaging showed features of pachymeningitis and pituitary gland infiltration. The diagnosis of primary Sjögren's syndrome was confirmed by demonstrating positive SS-A and SS-B antibodies and histological evidence of lymphocytic infiltration of the sublabial salivary gland. During the 2-year follow-up, the patient had transient VIth, IXth, Xth, and XIIth cranial nerve palsies. Sjögren's syndrome should be considered in the differential diagnosis of patients presenting with multiple recurrent cranial nerve palsies, even if prominent sicca symptoms are absent.

OC016: Signs of decreased cardiac performance in anti‐Ro52/SSA antibody exposed fetuses with prolonged atrioventricular time intervals

Objectives: Myocardial strain measures fractional change in ventricular wall dimension and allows quantification of myocardial performance. Quintero scoring for twin-twin transfusion syndrome (TTTS) cannot predict those who progress rapidly from stage 1 to intrauterine demise. We used vector-velocity imaging (VVI) which tracks natural myocardial speckles to calculate ventricular strain and determine whether this might aid risk stratification. Methods: We captured VVI images at about 100 Hz from a four chamber view during routine fetal echocardiograms in a pilot study of uncomplicated monochorionic twin pregnancies (MCDA) and ones with TTTS. Ventricular strain was calculated off-line and compared with our singleton reference range. Results: 33 MCDA pregnancies were studied at gestational age 21 + 5 (13 + 3 to 32 + 2) weeks. 20 pregnancies had TTTS (stages 1 = 3; 2 = 1; 3 = 12, 4 = 4) and 13 were uncomplicated. 40/66 scans (61%) were suitable for analysis and compared with reference data from 80 aged matched normal singleton pregnancies. Intra-class correlation was 0.68. Strain did not alter with gestational age and was normal in MCDA twins. Strain was significantly reduced in both recipient ventricles at stages 3 and 4, with a tendency to lower values in donor right ventricles. Reduced strain reflects ventricular response to increased afterload and possibly associated placental factors. Conclusions: As ventricular strain was normal in Quintero stages 1 and 2 it is unlikely to aid early risk stratification in MCDA pregnancies. Previously unreported abnormalities of right ventricular function may exist in the donor and require further investigation.

Long-Term Outcome of Isolated Congenital Complete Atrioventricular Block Pacing Since Neonatal Period Experience at a Single Japanese Institution

Few investigators have examined the outcome of patients with isolated congenital complete atrioventricular block (CCAVB) paced from the neonatal period. The present study follows the clinical course and describes the outcome of patients who have been paced with CCAVB since they were neonates. The medical records of 20 patients with CCAVB paced as neonates between 1981 and 2006 were retrospectively studied and reviewed; 18 were diagnosed in utero and 2 at birth. SS-A antibodies were detected in 9 of 14 (64%) mothers tested. The median age at follow-up was 5.6 years (range, 2 days to 21.9 years). Three (15%) of the early neonates died and 3 (15%) died later: 2 from dilated cardiomyopathy (DCM) and 1 from pneumonia. The total mortality rate was 30%. The cumulative probability of survival at 10 years was 73%. Of the 14 survivors, 4 developed DCM, representing a total morbidity of 30%. The cumulative probability of freedom from DCM at 10 years was 59%. Patients with CCAVB who undergo pacing as neonates have high mortality and high morbidity because of DCM. Left ventricular pacing or resynchronization can benefit patients who develop DCM and left ventricular epicardial pacing is recommended for neonates with CCAVB.

A 21-Year-Old Woman with Thrombotic Thrombocytopenic Purpura as Initial Symptom of Systemic Lupus Erythematosus and a Literature Review.

We describe a 21-year-old woman with systemic lupus erythematosus (SLE) who first presented with an thrombotic thrombocytopenic purpura (TTP), and compare the clinical manifestations and prognosis between SLE patients with thrombotic thrombocytopenic purpura in the reported literature. At the time of admission, she was suffering from petechia, purpura and had neurological symptoms. Laboratory findings demonstrated haemolytic anaemia, thrombocytopaenia and high levels of fragmentocytes. Serological test results were highly positive for antinuclear antibodies (ANA) with a particle fluorescent pattern, and also detected antibodies against Smith antibody, RNP antibody and SSA antibody but was negative for dsDNA. Direct Coombs-test was positive and D-dimer was negtive. The activity of ADAMTS13 was significantly reduced even after 4 times plasmapheresis. TTP as presenting sign in the patient with SLE was diagnosed. After 5 times plasmapheresis treatments and immunosuppressive therapy she recovered and abnormal laboratory tests were gradually returned to normal. With a follow-up of 20 months, she had a normal life. Compared with other reports, TTP can be differentiated from other thrombotic microangiopathic syndromes by its normal levels of prothrombin time, partially activated thromboplastin time (APTT), fibrinogen and direct Coombs-test. But TTP might also be a complication of SLE and the manifestations of TTP are similar to those in SLE. The detection of the fragmentation of peripheral red blood cells helped the early diagnosis of TTP. Coomb's test might be positive when patient with TTP had SLE. ADAMTS13 and its inhibitory antibody had an important role in the pathogenesis of TTP. Plasma exchange combined with corticosteroid and cyclophsphamide should be used as early as possible in TTP patient with SLE. The prognosis was related to the treatment time and methods including plasmapheresis.

Periorbital Cutaneous Neonatal Lupus

The authors report 3 infant girls with a similar periorbital eruption. A 5-month-old infant girl presented with a 3-month history of a photosensitive facial eruption that had failed to respond to topical 1% hydrocortisone cream. The otherwise healthy infant was the product of a term pregnancy. The 25-year-old mother was in good health except for occasional arthralgias. She had 2 other healthy children. Physical examination revealed a well-developed, well-nourished infant with annular, polycyclic, erythematous scaly plaques involving the cheeks and periorbital region (Figure 1). Potassium hydroxide scraping from the face was negative for dermatophyte and yeast. Laboratory studies revealed normal complete blood cell count, normal liver function tests, strongly positive anti-SSA antibody at 118 units (>80 units, strongly positive), and a negative anti-SSB antibody. Cardiac examination and electrocardiogram (ECG) were also normal. Laboratory evaluation of the mother revealed a positive anti-SSA at 158 units and mild anemia, but anti-SSB, anti-Smith, U1RNP, and anti-Scl-70 antibodies were all negative. Within 4 months the facial eruption cleared completely with the use of desonide cream 0.05% applied twice a day and sun protection. A 6-month-old girl was referred for dermatitis that began on the trunk and face at about 2 months of age. Although the truncal component resolved after 2 months, the facial dermatitis persisted. The infant was in good health and was the first-born child. The mother was known to have Sjögren syndrome. Physical examination revealed the characteristic erythematous, annular, scaling, polycyclic plaques along the forehead, periorbital cheeks, and eyelids (Figure 2). Laboratory evaluation of the infant revealed normal complete blood cell count, liver function tests, and chemistry profile. Anti-SSA antibody was positive at >6 units (reference, <1 unit) and anti-SSB antibody was positive at 2.84 units (reference, <1 unit). U1RNP antibody was negative. Cardiac examination and ECG were normal. The skin abnormalities cleared completely in 6 weeks with the topical application of tacrolimus 0.03% ointment and sun protection. A 5-month-old girl presented with a 2-month history of a persistent facial dermatitis. The infant was in good health and was the product of a healthy first pregnancy and delivery. The mother was in good health. Physical examination of the infant revealed erythematous, annular, polycyclic periorbital patches (Figure 3). Laboratory evaluation revealed positive SSA and SSB antibodies (units unavailable) and normal complete blood cell count, liver function tests, and chemistry profile. Cardiac examination and ECG were normal. The mother's laboratory results were also positive for anti-SSA and anti-SSB antibodies (units unavailable). The infant's facial eruption resolved without specific treatment.

Correlation of anti-60000 SSA antibody and anti-52000 SSA antibody with systemic lupus erythematosus and Sjögren's syndrome

To investigate the correlation of anti-60 000 SSA antibody and anti-52 000 SSA antibody with systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). Western blotting against purified 60 000 SSA antigenic polypeptide and 52 000 SSA antigenic polypeptide were done to detect the anti-60 000 SSA and anti-52 000 SSA antibodies in 59 serum samples positive in anti-SSA antibodies, among which 44 samples were from SLE patients and 15 samples from SS patients. There was no significant difference in positive rate of anti-60 000 SSA antibodies between the SLE and pSS patients (P > 0.05). But the sole positive rate of anti-60 000 SSA antibodies of the SLA patients was 39.47% (15/38), significantly higher than that of the primary SS (pSS) patients ((6.67%, 1/15, P < 0.05). The positive rate of anti-52 000 SSA antibodies of the pSS patients was 93.33% (14/15), significantly higher than that of the SLE patients (23/38, 60.53%, P < 0.05). There was no significant differences in positive rates of anti-60 000 SSA antibodies and anti-52 000 SSA antibodies between the secondary SS (sSS) and pSS patients (P > 0.05). The pSS patients mainly present anti-52 000 antibodies and with a very low sole anti-60 000 SSA antibody positive rate. Sole positivity of anti-52 000 SSA antibodies maybe correlated with pSS and is not a strong implication for the diagnosis of sSS. Sole positive anti-60000 SSA antibodies rate can be seen mainly in SLE.

ATYPICAL WEGENERʼS GRANULOMATOSIS PRESENTING AS LACRIMAL GLAND ENLARGEMENT AND OPHTHALMIC VENOUS OUTFLOW OBSTRUCTION CAUSING SECONDARY GLAUCOMA IN A MAN WITH A PRIOR PAROTIDECTOMY.

Purpose of Study Report occurrence of Wegener9s granulomatosis presenting as ophthalmic venous outflow obstruction causing secondary glaucoma in a man with a prior parotidectomy. Methods Used Retrospective case study. Summary of Results A 52-year-old man with diabetes mellitus presented with a red, painful eye. The patient denied dry eyes or dry mouth and otherwise felt well. There was no weight loss, fever, or night sweats. A year earlier, he had had left parotidectomy, which showed nonspecific chronic inflammation. On examination, the intraocular pressure was elevated, and there was episcleral injection, suggesting orbital or ophthalmic venous obstruction causing glaucoma. Magnetic resonance imaging of the brain and orbits showed prominence of the left lacrimal gland and subtle signs of venous congestion bilaterally. Biopsy showed a benign lymphoepithelial lesion with prominent chronic inflammation between acini and ducts, composed of lymphocytes, plasma cells, and eosinophils without granulomata. Later the patient complained of sinus congestion. CT of the sinuses showed septal deviation to the left and enlarged inferior turbinates. There were no intranasal lesions. A Schirmer9s test showed no xerophthalmia, and the oral mucosa was normal. The antiproteinase 3 antibody was > 6 (positive), suggestive of Wegener9s granulomatosis. The antinuclear antibody was weakly positive at 1:40, speckled; the anti-smRNP antibody was 9 (equivocal). The rheumatoid factor was negative. The anti-dsDNA, antimyeloperoxidase, anti-Smith, and SS-A (anti-Ro) and SS-B (anti-La) antibodies were negative; the angiotensin-converting enzyme was within normal limits. Despite therapy with prednisone, the patient developed uveitis and scleritis in both eyes and ultimately responded to therapy with oral cyclophosphamide. The patient was well and chose to stop therapy after 3 years. Conclusions Systemic vasculitis may present with tumor-like lesions.1 Retro-orbital pseudotumors, scleritis, and unilateral parotid enlargement are known Wegener9s manifestations.2,3 The present patient had unilateral parotitis, acute glaucoma secondary to orbital venous outflow obstruction, lacrimal enlargement, and, later, uveitis reflecting regional inflammation. The anti-proteinase 3 antibody suggested Wegener9s granulomatosis. 1. Medicine 2000;79:349-59. 2. Mayo Clin Proc 1990;65:1110-7. 3. Laryngoscope 2004;114:1730-3.