OBJECTIVE: To describe a case of autoimmune exocrinopathy in a child at the age of 3 months who presented with the original diagnosis of recurrent parotitis. DESIGN: This a case report of a 9-year-old girl with recurrent parotitis who was later found to have Sj?gren's syndrome. The literature relating to ...

AIMS: To determine the prevalence of plasma cell monotypia in labial salivary gland tissue of patients with and without Sjögren's syndrome, and to evaluate its relation to the development of systemic monoclonal lymphoproliferative disorders. METHODS: A quantitative immunohistological study was performed on labial salivary gland tissue of 45 patients with ...

Nine patients with Sjögren's syndrome were studied in terms of estradiol, testosterone, and dihydrotestosterone in the labial minor salivary glands using the peroxidase-antiperoxidase method. In normal controls in women, estradiol was positive in the epithelial cells of duct, but testosterone and dihydrotestosterone were negative or doubtfully positive by case. Thus, ...

A 38-year-old woman suffering from primary Sjögren's syndrome for 2 years developed angiofollicular hyperplasia (multicentric Castleman's disease). In Sjögren's syndrome (SS) a number of findings indicate the presence of a B-cell hyperactivity that may evolve to a lymphoproliferative disorder. This report adds another pathological event to the complex spectrum of ...

Recently, an enzyme-linked immunosorbent assay (ELISA), using purified recombinant non-fusion proteins, has been introduced to detect and quantify Ro/SS-A and La/SS-B autoantibodies. We compared this method with anti-Ro/SS-A and anti-La/SS-B detection by means of counterimmunoelectrophoresis and immunoblotting in patients with primary Sjögren's syndrome (SS), patients suspected of the syndrome and ...

A unique occurrence of sarcoidosis autoimmune haemolytic anaemia (AIHA), Sjögren's syndrome and idiopathic thrombocytopenic purpura (ITP) in the same patient is reported. Although the occurrence of autoimmune disease with sarcoidosis is well known, a case in which several of these diseases coexist with sarcoidosis is rare. We present a man ...

A 63-year-old woman, in whom a diagnosis of Sjögren's syndrome was initially made, proved to have systemic vasculitis with salivary gland involvement and necrotizing and crescentic glomerulonephritis. Antineutrophil cytoplasmic autoantibodies (ANCA) against myeloperoxidase were positive. ANCA-associated vasculitis should be considered in the differential diagnosis of Sjögren's syndrome. A positive finding ...

Sjögren's syndrome is an autoimmune exocrinopathy with characteristics of both organ-specific and generalized autoimmune disease. The main features are keratoconjunctivitis sicca and xerostomia, but patients may also show signs of extraglandular involvement. Sjögren's syndrome can occur in the absence (primary Sjögren's syndrome) or in the presence of other connective tissue ...

RATIONALE AND OBJECTIVES: The sialographic ductal patterns of the parotid glands in patients with Sj?gren syndrome were compared with those of normal patients by measuring the fractal dimensions. METHODS: Fractal dimensions were estimated using the modified pixel dilation method. RESULTS: The mean fractal dimension was 1.64 +/- 0.06 for the ...

Patients with multisystem disorders frequently present with or eventually manifest cutaneous disease. Many of these patients will also manifest ocular changes. In these disorders, the dermatologist or ophthalmologist may act as primary provider or consultant. Thus, familiarity with these conditions will enable the specialist to provide better care for these ...

OBJECTIVES: To evaluate the prevalence, the incidence and clinical presentation of non-Hodgkin's lymphoma associated with primary Sjögren's syndrome. METHODS: Sixty-two patients with primary Sjögren's syndrome were analyzed retrospectively in an open investigation. RESULTS: Of 62 patients with primary Sjögren's syndrome, 4 of them (6.4%) developed non-Hodgkin's lymphoma (6.9 cases per ...

Dried, freshly produced saliva from 21 patients with xerostomia related to the sicca syndrome [15 with primary Sjögren's syndrome (pSS), 3 with rheumatoid arthritis (RA) and secondary Sjögren's syndrome (sSS), and 3 with Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE)] and 21 age and sex matched controls, was examined ...

Certain adults and children infected with the human immunodeficiency virus(HIV)-1 developed a disorder termed diffuse infiltrative lymphocytosis syndrome (DILS) that, although having certain similarities to classic Sjögren's syndrome, manifests distinctive clinical, serologic, immunologic, and immunogenetic characteristics. DILS is characterized by circulating CD8 lymphocytosis and apparently antigen-driven CD8 T-cell infiltration of ...

The major mucous membrane and cutaneous manifestation of Sjögren's syndrome (SS) is xerosis. Severe dryness of the eyes, mouth, nasal passage, and vagina produce many annoying symptoms, including photophobia, burning and itching of the eyes, alterations in taste and smell, recurring nonallergic bacterial sinusitis rhinitis, and dysparunia. SS patients frequently ...

Sjögren's syndrome is a chronic autoimmune and inflammatory disease characterized by lymphocyte proliferation that progressively destroys exocrine glands. The lymphocytes can infiltrate other and more vital organs and may at times become malignant. Another name for this disease is autoimmune exocrinopathy. It was first described over 100 years ago both ...

The purpose of this study was to determine the occurrence and clinical value of anticardiolipin antibodies in patients with Sjögren's syndrome. Thirty one patients with primary Sjögren's syndrome (all women, mean (SD) age 48.3 (11.2) years) and 32 patients with secondary Sjögren's syndrome with rheumatoid arthritis (all women, mean (SD) ...

Clinically significant cytopenias are thought to be uncommon in primary Sjögren's syndrome: only a few cases have been reported in the literature. Over a 3-year period we identified haematological abnormalities in 11 of 27 patients with Sjögren's syndrome. Six patients had a positive direct antiglobulin test, including one patient with ...

Many of the ichthyotic disorders have characteristic ocular findings. These disorders include X-linked ichthyosis, lamellar ichthyosis, Sjögren-Larsson syndrome, KID syndrome, Refsum's disease, neutral lipid storage disease, chondrodysplasia punctata, and Richner-Hanhart syndrome. A knowledge of the ocular manifestations may provide a valuable aid to diagnosis in difficult cases. In some cases, ...

Three cases of optic neuropathy associated with primary Sjögren's syndrome are reported. All three patients had clinical manifestations of primary Sjögren's syndrome, although two of the patients did not report sicca symptoms at initial examination. Two patients had focal neurologic signs in addition to optic neuropathy. The differentiation of this ...

The occurrence and composition of intranodal salivary gland tissue (ISGT) in the head and neck region were investigated. ISGT was mainly composed of mature acini, intercalated ducts and intralobular ducts but immature acini (20.8%) and immature small ducts (18.8%) were also observed. The latter resembled salivary gland tissue in an ...

A variant of the actin-modulating protein gelsolin has recently been identified as a component of the amyloid deposits in familial amyloidosis, Finnish type (Meretoja's syndrome), and has been demonstrated immunohistochemically in amyloid deposits in the cornea, and in the skin, kidney, heart, thyroid gland, salivary gland, and rectum of patients ...

One hundred and forty two patients (62 with definite Sjögren's syndrome, 24 with probable Sjögren's syndrome, and 56 in whom Sjögren's syndrome was finally ruled out) were studied. Schirmer's test and rose bengal staining for the diagnosis of keratoconjunctivitis sicca and salivary scintigraphy and a labial biopsy sample for the ...

A 52-year-old woman presented with psoriasis vulgaris, Sjögren's syndrome, and Hashimoto's thyroiditis with a 5-year history. She had a number of immunological abnormalities and typical psoriatic plaques over her entire body. The relationship between psoriasis, Sjögren's syndrome, and Hashimoto's thyroiditis is discussed from the viewpoint of immunology, and similar cases ...

The incidence of lower urinary tract infection in 120 women with rheumatoid arthritis and secondary Sjögren's syndrome was evaluated retrospectively. Thirty one patients (26%) had secondary Sjögren's syndrome. Recurrent urinary tract infection was significantly more common in these patients (11/31) than in patients without Sjögren's syndrome (4/89). Habitual leucocyturia was ...

Labial salivary gland histopathology has long been considered to be the most disease-specific single test for diagnosis of Sjögren's syndrome. However, the diagnostic yield of widely used grading systems of focal lymphocytic sialoadenitis is rather low. Determination of the percentages IgA-, IgG-, IgM-, kappa-, and lambda-containing plasma cells in minor ...

In this paper the basis of the oral features of Sjögren's syndrome is described and guidelines for proper management of these features are given. The most pronounced oral symptoms are a dry sensation in the mouth and difficulties with swallowing and speech. Furthermore, these patients do not sleep well and ...

A 67-year-old woman with Sjögren's syndrome was found to have left hydronephrosis and stenosis of the left ureter. Exploratory laparotomy disclosed a nodule at the ureteropelvic junction of the left ureter. Histopathological examinations of the biopsied specimen of this nodule showed lymphoid hyperplasia within the ureteral wall. Low dose prednisolone ...

The criteria for a clinical diagnosis of Sjögren's syndrome remain controversial and vary widely from study to study. With respect to the oral component it is considered necessary to use some form of objective test, but many of those available are not suitable for use in a busy clinical situation. ...

Gustatory sweating, or Frey's syndrome, is a fairly common sequela of partial or radical parotidectomy, submaxillary gland surgery, or radical neck dissection. It is caused by an anastomotic communication with facial sweat glands by parasympathetic secretomotor nerve fibers intended for the excised parotid gland; treatments, whether surgical or topical, generally ...

The pulmonary manifestations of collagen vascular diseases span an enormous range of clinical and radiographic findings. The breadth of these abnormalities is as diverse as the underlying diseases themselves. A comprehensive discussion of pulmonary involvement in four of these diseases, the Marfan syndrome, ankylosing spondylitis, Sjögren's syndrome, and relapsing polychondritis, ...

A 53-year-old woman was admitted because of Raynaud's phenomenon, polyarthralgia and polymyalgia. Biopsy specimens of the liver and thyroid gland revealed characteristic findings of primary biliary cirrhosis (PBC) (stage I by Scheuer's classification) and chronic thyroiditis. Her clinical features were also complicated by scleroderma (type I by Barnett's classification) and ...

A safe and simple technique is reported by which primary Sjögren's syndrome can be detected with a relatively high specificity and sensitivity. The method of serial scintigraphy has been used with reasonable success; however, the application of the linearity index as described here produced superior results. In 71 patients investigated, ...

A 61 year old man with dryness of the eyes and mouth, constitutional and musculoskeletal symptoms is described. A diagnosis of fibromyalgia with Sjögren's syndrome was made elsewhere. Further examination, however, disclosed diffuse mast cell infiltration in several organs, including the labial accessory salivary glands.

Sjogren's syndrome is an autoimmune disease with a known predisposition for lymphoma development. Eight of 120 patients with primary Sjogren's syndrome followed at the University of Ioannina over the past 7 years developed non-Hodgkin's lymphoma diagnosed according to the Kiel classification. The lymphomas differed by location and grading. Six were ...

Autoimmune reactions are evoked in hybrid mice after induction of a chronic graft-versus-host reaction by transfer of viable leucocytes from one of the parental strains to non-irradiated F1 recipients. We have previously demonstrated an SLE-like syndrome early in the reaction, with an additional Sjögren's syndrome-like glandular affection occurring later. In ...

We present a case of corneal melting in a patient with severe rheumatoid arthritis and Sjögren's syndrome. The melting appeared in the area of a bacterial corneal ulcer that healed after treatment with antibiotics. No signs of scleritis were present. Repeated melting of two corneal grafts was seen after surgery.

Bilateral cystic parotid glands occur in some cases of human immunodeficiency virus (HIV) infection. This abnormality, which is associated with cervical adenopathy, can be defined by sonography in the superficial gland, as noted in three men. In retrospect, this parotid disease has similarity to the sonographic finding of Sjögren's syndrome, ...

Palindromic rheumatism is a syndrome of intermittent abrupt onset monoarthritis with asymptomatic intercritical periods of variable duration, which commonly evolves into rheumatoid arthritis. Felty's syndrome consists of leucopenia (selective neutropenia) and splenomegaly, usually occurring in longstanding classic rheumatoid arthritis. Felty's syndrome can be confused with the more recently recognised rheumatoid ...

We report a rare case of dry eyes and dry mouth caused by primary amyloidosis. A 66-year-old woman with keratoconjunctivitis sicca and xerostomia died of acute respiratory failure. Shirmer's test, gum test, and sialography indicated Sjögren's syndrome. Lip biopsy revealed amyloid deposition around the salivary ducts. Bence-Jones protein was noted ...

We report a case of Sjögren's syndrome characterized by keratoconjunctivitis sicca, parotid swelling, reduced salivary flow, and abnormalities on nuclear scanning. Two labial gland biopsies showed complete atrophy. Biopsy of an enlarged submandibular gland revealed lymphoid follicles and glandular replacement consistent with the diagnosis of Sjögren's syndrome. This case supports ...

Salivary SPECT and factor analysis in Sjögren's syndrome were performed in 17 patients and 6 volunteers as controls. The ability of SPECT to detect small differences in the level of uptake can be used to separate glands from background even when uptake is reduced as in the patients with Sjögren's ...

A 78-year-old man presented simultaneously with subacute cutaneous lupus erythematosus (SCLE) and Sweet syndrome (neutrophilic dermatosis). He was subsequently investigated for and found to have Sjögren syndrome. We believe that the simultaneous occurrence of the above cutaneous manifestations are very suggestive of and should prompt investigation for Sjögren syndrome.

A patient is described with Sjögren's syndrome and an IgM-kappa gammopathy. With time cryoglobulins, the cause of acute renal failure, could be detected. The kidney biopsy showed a proliferative glomerulonephritis as is frequently seen with mixed cryoglobulinaemia. Plasmapheresis and immunosuppression resulted in long-term improvement of kidney function. The differential diagnosis ...

The histochemical and immunocytochemical findings of biopsied muscle in a 2-year-old girl with Marinesco-Sjögren syndrome are reported. Muscle histology consisted of mild muscular dystrophy, such as that found in limb-girdle or non-Fukuyama congenital muscular dystrophy. By immunocytochemical stain using anti-dystrophin antibody, Duchenne and Becker muscular dystrophies were excluded. In addition ...

Keratoconjunctivitis sicca patients diagnosed on the basis of a history, dry-eye symptoms, and definite clinical signs of keratoconjunctivitis sicca, with the associated symptoms of dry mouth and/or arthritis, had measurements of tear osmolarity, Schirmer tear test without anesthetic, stimulated parotid salivary flow, and serum analysis for the presence of autoantibodies ...

Three patients are presented to illustrate the diagnostic and prognostic value of quantitative immunohistological examination of labial salivary gland biopsy in Sjögren's syndrome. In an obvious case of primary Sjögren's syndrome and in a case of rheumatoid arthritis without clinical and serological evidence of secondary Sjögren's syndrome, quantitative immunohistological examination ...

Sjögren-Larsson syndrome (SLS) is an autosomal recessively inherited disorder characterized by the triad of congenital ichthyosis, spastic diplegia or tetraplegia and mental retardation. SLS is also often associated with a variety of other anomalies, including signs that are of interest to optometrists and ophthalmologists. Two case histories are presented. Both ...

The efficacy of eliminating parotid salivary secretions by retrograde infusion of 10% formalin, 2% chlorhexidine, or 2% or 3% silver nitrate solutions was evaluated in 10 horses. Solutions were kept within the parotid salivary gland for 90 seconds after infusion through parotid duct cannulae and then allowed to drain freely. ...

Keratoconjunctivitis sicca (KCS) is one of the dry eye syndromes characterized by a deficiency of the aqueous layer of the tear film. The disorder may occur as an isolated entity or in association with a variety of local and systemic conditions affecting aqueous production. Often it follows a mild course ...

B cell expression of the germline gene-encoded, kappa IIIb-associated, rheumatoid factor (RF) cross-reactive idiotope (CRI) 17-109 and three VHI associated RF CRIs (G6, G8, H1) was investigated immunocytochemically in labial salivary glands from nine patients with primary and six with secondary Sjögren's syndrome, and in inflamed submandibular salivary glands from ...