Pulmonary manifestations of Sjgrens syndrome. Introduction. Primary Sjgrens syndrome was originally described in 1. Gougerot 1. Its prevalence is estimated at 0. It is the second most common multisystem autoimmune disease after rheumatoid arthritis, and is characterised by eye and mouth dryness and lymphocytic infiltration of the salivary glands, which are easily accessible and thus facilitate diagnosis. New classification criteria were endorsed by the American College of Rheumatology in 2. Like other autoimmune diseases, it is associated with a specific genetic background, impaired immune response and environmental factors. These factors are believed to trigger an innate immune response leading to activation of glandular cells, followed by activation of B and T lymphocytes within the glands. Mdb Converter Serial Number there. In addition to dryness, clinical presentation of Sjgrens syndrome generally includes asthenia and arthralgia. The disease can extend beyond the exocrine glands, and systemic manifestations including vasculitis, lung, renal or neurological involvement can occur. In 920 of cases, Sjgrens syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease ILD. Comprehensive, uptodate information on HIVAIDS treatment and prevention from the University of California San Francisco. Patients with Sjgrens syndrome also have an increased risk of lymphoma 4. The pulmonary manifestations of Sjgrens syndrome include airway abnormalities, interstitial lung disease ILD and lymphoproliferative disorders table 1. Lung involvement occurs in 92. Subclinical lung disease is even more frequent, including small airway disease and airway inflammation 5. The presence of ILD is associated with impaired respiratory function. Importantly, pulmonary involvement leads to increased risk of mortality 6. Herein, we summarise the literature about Sjgrens syndrome pulmonary manifestations. Pulmonary edema may be classified as increased hydrostatic pressure edema, permeability edema with diffuse alveolar damage DAD, permeability edema without DAD, or. Eosinophilic lung diseases are a diverse group of pulmonary disorders associated with peripheral or tissue eosinophilia. They are classified as eosinophilic lung. To exclude pulmonary manifestations of conditions associated with Sjgrens syndrome, we only focused on pulmonary involvement of primary Sjgrens syndrome. TABLE 1. Thoracic manifestations of Sjgrens syndrome. Background. Epidemiology of Sjgrens syndrome. Sjgrens syndrome is the second most common autoimmune disease after rheumatoid arthritis. Incidence has recently been estimated at between 3. Europe 7. Population based and sample based studies estimate its prevalence at 4. CHF-1c1.jpg' alt='Patchy Opacity And Infiltrates' title='Patchy Opacity And Infiltrates' />Sjgrens syndrome mostly affects middle aged women, with a peak at 5. There is a clear sex ratio, ranging from 9 to 1. Sjgrens syndrome is likely to be associated with other autoimmune diseases such as thyroiditis. Pathophysiology of Sjgrens syndrome. Like other autoimmune diseases, the aetiopathogenesis of Sjgrens syndrome combines environmental factors such as viruses cytomegalovirus, HIV, Human T cell leukaemia virus, and hepatitis C virus 9 or solvents 1. Patchy Opacity And Infiltrates' title='Patchy Opacity And Infiltrates' />Recent studies have sought to characterise the immune response that occurs in Sjgrens syndrome. They confirm that B and T cells play a major role in this immune response, but also highlight the important role of innate immunity. B cell demethylation 1. B cells promote plasma cell secretion of anti Ro SSA and anti La SSB autoantibodies directed to the small cytoplasmic RNP bound peptides SSA 6. D, SSA 5. 2k. D and SSB 4. D 1. 5. It is noteworthy that anti Ro. The role of T cells in Sjgrens syndrome has been extensively studied and reviewed 1. T cells and T helper 1. T cell activation and cytotoxicity are undeniable. Innate immunity in Sjgrens syndrome is an area of growing interest, with promising results, such as the role of the interferon signature 1. New findings regarding innate immunity also include apoptotic cell clearance defect 1. P2. X7 receptors and inflammasome 2. This immune response, combined with local inflammation, gland infiltration and pathogenic anti Ro. Sjgrens syndrome patients. CctJ-kuS_H8_fexKCGEQ245236/GW610H700' alt='Patchy Opacity And Infiltrates' title='Patchy Opacity And Infiltrates' />Severe acute respiratory syndrome SARS is a viral respiratory disease of zoonotic origin caused by the SARS coronavirus SARSCoV. Between November 2002 and July. SA5-C30-FF7.gif' alt='Patchy Opacity And Infiltrates' title='Patchy Opacity And Infiltrates' />Extrathoracic involvement. While glandular involvement is by far the most frequent manifestation, with sicca symptoms affecting up to 9. Sjgrens syndrome 2. Sicca symptoms mostly concern the mouth xerostomia, periodontal disease, caries, angular cheilitis or endobuccal ulcers are frequently encountered. Eyes are the next most involved organ xerophtalmia is often incapacitating and causes photophobia and impaired night vision, while cornea dryness can be complicated by keratitis, ulcers or even perforations. Other ocular complications such as conjunctivitis, uveitis, scleritis and episcleritis, orbital inflammation, retinal vasculitis or optic neuritis have also been reported 2. The ear, nose and throat area is also affected, with symptoms such as smell and taste disorders, hoarseness and itching of the external ear canal, while parotitis or salivary gland swelling is also common 3. Genital sicca can significantly impair quality of life, especially in women 2. Skin involvement includes xeroderma, hypohydrosis, itching, cutaneous shedding, annular erythema, atypical folliculitis and Raynauds phenomenon. Purpura or leukocytoclastic vasculitis, sometimes mimicking urticaria, can also be encountered, especially in association with cryoglobulinaemia 2. Extraglandular, extrathoracic involvement includes psychological manifestations, such as asthenia 7. Arthralgia andor myalgia are estimated to be present in half the cases of Sjgrens syndrome, and symmetrical non erosive arthritis can be observed. Neurological involvement is common 2. The central nervous system can be affected 2 vasculitis and cranial nerve palsy have been described, as well as transverse myelitis and optic neuritis. The peripheral nervous system is more frequently affected 51. Sjgrens syndrome sensitive neuropathy, mostly dorsal root ganglionopathy 5, and painful small fibre neuropathy 51. Digestive involvement has been reported in Sjgrens syndrome, particularly dysphagia and dyspepsia, probably due to xerostomia 2. Sjgrens syndrome can also be associated with primary biliary sclerosis 2. Renal and urological involvement are not unusual tubulointestitial nephritis, interstitial cystitis, kidney stones and glomerulonephritis have been described, as well as vasculitis and distal renal tubular acidosis 2. Haematological involvement is amongst the more serious complications of Sjgrens syndrome even if polyclonal hypergammaglobulinaemia is mostly asymptomatic, it can evolve towards oligoclonal or monoclonal gammapathy. Cryoglobulinaemia mostly type 2 and 3 can occur and cause vasculitis, which is mostly cutaneous but likely to be responsible for multi organ involvement. Cryoglobulinaemic vasculitis can also affect peripheral nerves. Lymphadenopathy is also frequently reported, possibly associated with autoimmune cytopenias. Finally, the most serious haematological complication is B cell mantle lymphoma, with a frequency that is six to 4. Sjgrens syndrome than in the general population 3. The SSARo antigen consists of two polypeptide components of 5. Da. The clinical associations of anti SSA6. Sjgrens syndrome, systemic lupus erythematosus and foetalmaternal autoimmune syndromes. Anti RoSSA5. 2 autoantibodies are the most prevalent extractable nuclear antigen specificity identified. Their specific diagnostic value is mostly based on the simultaneous presence of other autoantibodies whose diagnostic weight is more relevant. Anti RoSSA5. 2 autoantibodies are expressed in systemic lupus erythematosus 5. Sjgrens syndrome 6. Finally, babies of mothers with SSA antibodies can develop neonatal lupus, with cutaneous involvement andor congenital atrioventricular heart block 3. Classification criteria.