Tuesday, December 2, 2008 - 3:26PM EST

Introduction to Sjogren's Syndrome

Signs and Symptoms of Sjogren's Syndrome

  • Lymphocytic interstitial pneumonitis - a syndrome characterized by fever, cough, and shortness of breath with infiltrates of dense interstitial accumulations of lymphocytes and plasma cells
  • Pulmonary pseudolymphoma - a benign accumulation of inflammatory lymphoid cells in the lung
  • Alveolitis - inflammation of the air sacs (alveoli) in the lungs where the exchange of oxygen and carbon dioxide takes place
  • Various types of pneumonia, such as nonspecific interstitial pneumonia, organizing pneumonia (noninfectious inflammation of the bronchioles and surrounding lung tissue), usual interstitial pneumonia (changes in lung tissue that are indicative of fibrosis or scarring)
  • Primary lymphoma of the lung
  • Mucosa associated lymphoid tissue (MALT)
  • Lung hypersensitivity
  • Diffuse interstitial amyloidosis (deposit or accumulation of protein in lung tissue) - this is not a common development but is associated with progressive deterioration and a poor prognosis.

Tracheobronchial sicca and interstitial pneumonitis are the most common lung conditions associated with Sjogren's syndrome.

To read more about interstitial lung disease in primary Sjogren's syndrome, please click on the following link: http://www.medifocus.com/abstracts.php?gid=RH011&ID=17714766

Kidney/Bladder Symptoms

Kidney and other genitourinary symptoms are thought to occur in approximately 10% of patients with primary Sjogren's syndrome and are caused by the infiltration of lymphocytes into the kidneys. Clinically significant renal involvement is rare.

Renal conditions which may develop in patients with Sjogren's syndrome include:

  • Chronic interstitial cystitis - a chronic, inflammatory condition of the bladder wall that is a common condition among patients with Sjogren's syndrome. Recent studies have found that many people with Sjogren's syndrome also suffer from chronic interstitial cystitis in the absence of bacterial infection. The symptoms are similar to those of a urinary tract infection, however, urine cultures are negative. The diagnosis is confirmed by cystoscopy and biopsy.
  • Tubular disease such as tubulointerstitial nephritis - an inflammation of the tubules of the kidney and the spaces between the tubules and the glomeruli. Tubular involvement is usually found in younger patients and typically remains subclinical without developing into renal failure.
  • Renal tubular acidosis develops when the kidneys fail to excrete acids into the urine resulting in a build-up of acids in the bloodstream. Without proper treatment, this condition can lead to a variety of other complications including kidney stones and progressive kidney failure.
  • Glomerular diseases such as membranous glomerulonephritis - an inflammation of the glomeruli of the kidneys caused by the accumulation of protein in the bloodstream (proteinuria). Glomerular involvement is a severe manifestation of extraglandular disease involvement of Sjogren's syndrome. It typically appears later in the course of Sjogren's syndrome and is associated with morbidity and mortality. Early diagnosis and treatment is very important.
  • Renal calculi - kidney stones
  • Deterioration of renal function due to certain medications given to treat Sjogren's syndrome such as non-steroidal anti-inflammatory drugs
Gastrointestinal Symptoms

Saliva plays a major role in the initial phases of digestion and reduced amounts or absence of saliva can cause a disruption in the normal function of the gastrointestinal tract. Esophageal dryness is the most common gastrointestinal manifestation of Sjogren's syndrome and is the cause of dysphagia (difficulty swallowing) experienced by many people with Sjogren's syndrome. Other difficulties which patients may experience include:

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