dioxide, and methane). It results in symptoms including abdominal pain, bloating, ßatulence, diarrhea, nausea, and acid reßux. As milk products are a common source of calcium and vitamin D (which is supplemented in milk), osteoporosis may be accelerated in these individuals.

Pancreatitis and sclerosing cholangitis (PSC) [59Ð62]ÑPrimary sclerosing cholangitis is a chronic progressive disorder of unknown etiology that is characterized by inßammation, Þbrosis, and stricturing of medium-sized and largesized ducts in the intrahepatic and extrahepatic biliary tree. The great majority of cases have underlying ulcerative colitis, but the syndrome has also been reported in increased frequency in SS patients. A variety of immunosuppressive and anti-inßammatory agents have been studied in patients with PSC, including ursodeoxycholic acid, steroids, cyclosporine, methotrexate, azathioprine and 6-mercaptopurine, tacrolimus, D-penicillamine, and more recently etanercept (that failed to show beneÞt). Unfortunately, none has been shown conclusively proven to alter the natural history of this disorder. Initial small studies with each of these agents looked promising but subsequent larger studies failed to conÞrm the initial enthusiasm. Among the agents listed above, a result from recent randomized trials of high-dose ursodeoxycholic acid has suggested promise.

18.7Urologic

Painful bladder syndrome (PBS)/interstitial cystitis (IC) has been reported in SS and the patients have increased urinary frequency and urgency [63Ð65]. Large volumes of ßuid consumed due to dry mouth compound the problems of interstitial cystitis.

Amitriptyline is commonly prescribed for relief of PBS/IC symptoms. However, its anticholinergic side effects increase dryness and thus it is poorly tolerated in SS patients. However, only a minority of patients in the amitriptyline group experienced greater than 30% decrease in symptom score, suggesting that beneÞts are modest.

Pentosan polysulfate sodiumÑPentosan polysulfate sodium (PPS) is the only oral medication approved by the United States Food and Drug Administration (FDA) for treatment of IC. The approved dose is 100 mg three times daily, although off-label treatment using 200 mg twice daily is clinically common. The medication is a protein that is supposed to be Þltered by the kidneys and appear in the urine so that it can reconstitute the deÞcient glycosaminoglycan (GAG) layer over the urothelium. In fact, only a tiny proportion of the drug is absorbed by the gastrointestinal tract and excreted in the urine. If the patient is scheduled for surgery, it is worth noting that this medication can lead to altered platelet function [66] and may contribute to thrombocytopenia [67].

Other therapies of interstitial cystitis that have been reported include

¥Intravesical heparin and lidocaine

¥Intravesical dimethyl sulfoxide (DMSO)

¥Hydrodistension, however, there are risks of hydrodistension that includes bleeding (from ruptured vessels) and, rarely, rupture of the bladder wall

18.8Therapeutic Management of Obstetrical/Gynecological Manifestations

18.8.1 Vaginal Dryness [68–72]

A gynecologic exam is useful to rule out other causes of painful intercourse and other causes of vaginal dryness.

When it does occur as part of SjšgrenÕs syndrome, the spouse or partner needs to be reassured that this is a ÒphysiologicalÓ problem and not related to a failure of sexual arousal. The SjšgrenÕs patient currently has many more options regarding safe and effective vaginal lubrication than ever before.

Lubricants such as Maxilube R and Astroglide R have slightly different characteristics when compared with KY Brand R jelly or Surgilube R and yet share the common characteristics of being water soluble and non-irritating.