Primary Sjogren's Syndrome Presenting Research Articles

Primary Sjogren's syndrome presenting as autoimmune cytopenia.

Sjogren’s syndrome (SS) is a chronic systemic autoimmune disease, characterized by lymphocytic infiltration of lacrimal and salivary glands. Although extra glandular manifestations are uncommon, they can occur with the musculoskeletal, renal, pulmonary and hematological disease. We report the case of a 35-year-old woman presented to us with persistent unexplained bicytopenia (anemia and thrombocytopenia). Antinuclear antibody and direct Coombs test were positive. Anti-Ro/SSA and anti-La/SSB antibodies were also positive in high titer. The final diagnosis of primary SS with autoimmune cytopenia was made. Cytopenias in SS are rarely reported. Our case illustrates that clinically significant cytopenias may present as an extraglandular manifestation of SS.

Primary Sjogren's Syndrome Presenting as Acute Interstitial Pneumonitis/Hamman-Rich Syndrome.

A previously well, 45-year-old Pakistani lady was admitted to the medical unit on-call of Khyber Teaching Hospital (KTH) Peshawar with a 5-day history of fever, productive cough with copious mucoid sputum, dyspnea, and pleuritic chest pain. She also complained of dry eyes, mouth, and vagina. Her chest X-ray showed diffuse alveolar shadowing and arterial gas analysis confirmed type 1 respiratory failure. Over the next few days, she deteriorated rapidly making an urgent transfer to the medical intensive care unit (MICU) necessary, where she was mechanically ventilated. An HRCT followed by bronchoscopic biopsies made a diagnosis of acute interstitial pneumonitis (AIP), formerly known as Hamman-Rich syndrome. She also turned out to be positive for both anti-SS-A/Ro and anti-SS-B/La antibodies along with a positive Schirmer's test and lower lip biopsy. She received intravenous steroids and supportive care. The patient had a complete recovery after approximately three weeks' stay in the hospital with lung function returning back to normal. This is most probably the first ever case of primary Sjogren syndrome (pSjS) presenting as AIP, recovering completely in less than a month time.

Primary Sjogren's Syndrome Presenting as Osteomalacia

Background: The most common renal disease in Sjogren’s syndrome (SjS) is tubulo-interstitial nephritis, which is responsible for renal tubular acidosis type 1 (RTA-1) in around 20% of patients. Osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to a connective tissue disease such as SjS. Results and conclusion: We report a 47-year-old woman with tubular acidosis induced osteomalacia. The tubular acidosis was a result of renal involvement in SjS. Improvement was obtained with vitamin D, calcium, and high-dose steroid therapy. These findings suggest that evidence for SjS should be sought in adult patients with unexplained osteomalacia and renal tubular acidosis, even in the absence of subjective sicca syndrome. Conversely, in patients with SjS, early investigation and treatment of renal tubular dysfunction may prevent future complications, such as osteomalacia.

Primary Sjogren′s Syndrome presenting with Interstitial lung disease and Myositis

Sjogren's syndrome (SS) is an autoimmune disease, is the most common autoimmune disease following rheumatoid arthritis but remains undiagnosed in more than half. The hallmark histopathological features of SS are periepithelial lymphocytic infiltration of the affected organs. Antibodies to SSA (Ro) and SSB (La) are characteristically associated with SS. The reported frequency of pulmonary involvement in primary SS varies widely, ranging from 9% to 75% and consists of various forms of small airway diseases and interstitial lung diseases (ILD). We are reporting a case of primary SS presenting with ILD and myositis.

Primary Sjogren's syndrome presenting as a case of isolated recurrent unilateral parotitis in a 12 year old

We describe a case of a 12 year old male presenting with recurrent unilateral parotitis who was found on diagnostic workup to have Sjogren's syndrome. We describe the presentation of Sjogren's syndrome in pediatric population and review the workup and diagnostic criteria.

Primary Sjögren Syndrome in Spain

We conducted the current study to characterize the clinical presentation of primary Sjögren syndrome (SS) in a large cohort of Spanish patients and to determine whether epidemiologic, clinical, and analytical features modulate disease expression. Patients were from the GEMESS Study group, which was formed in 2005 and included 12 Spanish reference centers. By March 2007, the database included 1010 consecutive patients, recruited since 1994, both incident and prevalent cases. The cohort included 937 women and 73 men (ratio, 13:1), with a mean age of 53 years at diagnosis and 59 years at inclusion in the registry. Multivariate analysis showed that male patients had a lower frequency of thyroiditis, Raynaud phenomenon, and antinuclear antibodies. Young-onset patients had a low degree of sicca involvement (xerostomia and parotid enlargement) and a high frequency of immunologic markers (anti-Ro/SS-A and low C4 levels). Patients with disease duration of more than 10 years had a higher prevalence of xerophthalmia, parotid enlargement, lung involvement, and peripheral neuropathy in comparison with incident cases. The subset of patients with anti-Ro/La antibodies had the highest prevalence of most systemic, hematologic, and immunologic alterations (higher frequency of Raynaud phenomenon, altered parotid scintigraphy, positive salivary gland biopsy, peripheral neuropathy, thrombocytopenia, and rheumatoid factor). Hypocomplementemia was associated with a higher frequency of vasculitis and lymphoma, and cryoglobulins with a higher frequency of parotid enlargement, vasculitis, and leukopenia.Epidemiologic, clinical, and analytical features have a significant impact on the clinical presentation of primary SS, influencing the results of the main diagnostic tests, the prevalence and diversity of extraglandular involvement, and the frequency of the main immunologic markers. Primary SS should be considered as a systemic autoimmune disease that can express in many guises beyond sicca involvement.

Primary Sjogren's syndrome presenting as a case of sarcoidosis and a suspected pancreatic tumour.

A 45 year old man, previously diagnosed as having sarcoid, presented with signs and symptoms of a pancreatic malignancy. An explorative laparotomy, however, showed only chronic pancreatitis. He was found to have a raised erythrocyte sedimentation rate, normocytic normochromic anaemia, renal insufficiency, hypergammaglobulinaemia, and a strongly positive rheumatoid factor and antinuclear antibody titre. Bilateral hilar lymph node enlargement was noted on chest x ray. Subsequently, the patient complained of xerostomia and keratoconjunctivitis sicca. Large lymphocytic infiltrates and a shift in the relative number of IgA bearing plasma cells in favour of IgG and IgM bearing plasma cells were seen in tissue obtained by sublabial salivary gland biopsy. A transbronchial lung biopsy and review of the biopsies of the pancreas, the lung, liver, and a lymph node all failed to show granulomatous disease. These findings strongly suggested a diagnosis of Sjögren's syndrome instead of sarcoidosis. This case shows the difficulty sometimes encountered in differentiating between sarcoid and systemic Sjögren's syndrome, and the value of a sublabial salivary gland biopsy.

Primary Sjogren's syndrome presenting as oesteomalacia secondary to renal tubular acidosis

International Journal of Clinical PracticeVolume 42, Issue 10 p. 436-438 Case Report Primary Sjogren's syndrome presenting as oesteomalacia secondary to renal tubular acidosis B Pal MRCP, D Med Rehab, Corresponding Author B Pal MRCP, D Med Rehab Senior Registrar n/[email protected] Royal Victoria Infirmary, Newcastle upon TyneAddress correspondence to Dr B Pal, Consultant Physician, Department of Rheumatology, Withington Hospital, West Didsbury, Manchester M20.Search for more papers by this authorI D Griffiths FRCP, I D Griffiths FRCP Consultant Rheumatologist Royal Victoria Infirmary, Newcastle upon TyneSearch for more papers by this author B Pal MRCP, D Med Rehab, Corresponding Author B Pal MRCP, D Med Rehab Senior Registrar n/[email protected] Royal Victoria Infirmary, Newcastle upon TyneAddress correspondence to Dr B Pal, Consultant Physician, Department of Rheumatology, Withington Hospital, West Didsbury, Manchester M20.Search for more papers by this authorI D Griffiths FRCP, I D Griffiths FRCP Consultant Rheumatologist Royal Victoria Infirmary, Newcastle upon TyneSearch for more papers by this author First published: 01 October 1988 https://doi.org/10.1111/j.1742-1241.1988.tb08622.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Volume42, Issue10October 1988Pages 436-438 RelatedInformation