Dipartimento di Reumatologia della Regione Basilicata                 Direttore: Dott. Ignazio OlivieriLe manifestazioni m...
Whipple’s disease: definitionA rare, systemic infectious disease caused by thebacterium Tropheryma whipplei.
Whipple’s disease: epidemiology•   No valid estimate of its actual prevalence.•   More than 1000 cases have been reported ...
Whipple’s disease: history              Fenollar F, et al. N Engl J Med 2007;356:55-66.
Whipple’s disease: features              Fenollar F, et al. N Engl J Med 2007;356:55-66.
Whipple’s disease: two stagesThe prodromal stageprotean symptoms, along with chronic nonspecificsymptoms, mainly arthralgi...
Whipple’s disease: two stagesThe steady-state stageweight loss, diarrhea, or both, and occasionallyother manifestations.
Whipple’s disease: two stagesThe average time between the prodromal and thesteady stages is 6 years.
Whipple’s disease: two stagesA more rapid progression may occur in patientsreceiving corticosteroids, DMARDs or anti-TNFαd...
Whipple’s disease: two stages27 patients with Whipple’s disease (WP) 1. patients with immunosuppressive therapy before dia...
Musculoskeletal manifestations• Arthralgia and arthritis• Spondyloarthritis• Myalgia and cramps
Musculoskeletal manifestations• Arthralgia and arthritis• Spondyloarthritis• Myalgia and cramps
Arthralgia and arthritis• Intermittent and migratory• Commonly involving ankles, knees, elbows  shoulders and finger joint...
Musculoskeletal manifestations• Arthralgia and arthritis• Spondyloarthritis• Myalgia and cramps
SpondyloarthritisThe seronegative spondyloarthritides1. Uncomplicated ankylosing spondylitis2. Psoriatic arthritis3. Reite...
Spondyloarthritis18 patients with coexisting Whipple’s disease (WP)and ankylosing spondylitis (AS).Moll and Wright could a...
SpondyloarthritisSubsequently, several cases of coexistingWhipple’s disease and ankylosing spondylitishave been reported. ...
SpondyloarthritisStudies examining patients with Whipple’s disease (WD)for sacroiliitis and ankylosing spondylitis (AS):• ...
SpondyloarthritisStudies on the frequency of HLA-B27 in patients withWhipple’s disease (WD):• Feurle GE, et al. Eur J Clin...
SpondyloarthritisStudies on the frequency of HLA-B27 in patients withWhipple’s disease (WD):• Bai JC, et al. Am J Gastroen...
McCarty DJ, et al. Remitting seronegativesymmetrical synovitis with pitting edema.RS3PE syndrome. JAMA 1985;254:2763-7.•  ...
Altre malattie infiammatorie•   Polimialgia reumatica/arterite di Horton•   Spondiloartriti•   Condrocalcinosi•   Artropat...
Olivieri I, et al. J Rheumatol 2001;28:1294-7. •   No patient with a family history of spondyloarthritis. •   No patients ...
Practical Take Home Messages
Musculoskeletal manifestations    of Whipple’s disease• Among the most common extra-intestinal symptoms• May precede the g...
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Olivieri ignazio malattia di whipple torino gennaio  2011_14° convegno patologia immune e malattie rare
Upcoming SlideShare
Loading in …5
×

Olivieri ignazio malattia di whipple torino gennaio 2011_14° convegno patologia immune e malattie rare

954 views

Published on

Published in: Health & Medicine
  • Be the first to comment

  • Be the first to like this

Olivieri ignazio malattia di whipple torino gennaio 2011_14° convegno patologia immune e malattie rare

  1. 1. Dipartimento di Reumatologia della Regione Basilicata Direttore: Dott. Ignazio OlivieriLe manifestazioni muscolo-scheletrichedella malattia di Whipple Ignazio Olivieri
  2. 2. Whipple’s disease: definitionA rare, systemic infectious disease caused by thebacterium Tropheryma whipplei.
  3. 3. Whipple’s disease: epidemiology• No valid estimate of its actual prevalence.• More than 1000 cases have been reported so far.• Frequency <0.1% in postmortem studies.• The typical patient is a middle-aged white man.
  4. 4. Whipple’s disease: history Fenollar F, et al. N Engl J Med 2007;356:55-66.
  5. 5. Whipple’s disease: features Fenollar F, et al. N Engl J Med 2007;356:55-66.
  6. 6. Whipple’s disease: two stagesThe prodromal stageprotean symptoms, along with chronic nonspecificsymptoms, mainly arthralgia and arthritis.
  7. 7. Whipple’s disease: two stagesThe steady-state stageweight loss, diarrhea, or both, and occasionallyother manifestations.
  8. 8. Whipple’s disease: two stagesThe average time between the prodromal and thesteady stages is 6 years.
  9. 9. Whipple’s disease: two stagesA more rapid progression may occur in patientsreceiving corticosteroids, DMARDs or anti-TNFαdrugs.
  10. 10. Whipple’s disease: two stages27 patients with Whipple’s disease (WP) 1. patients with immunosuppressive therapy before diagnosis 2. patients with NSAIDs before diagnosis 3. patients without such therapiesArthritis occurred in the mean 8 years before diagnosis and wasthe first symptoms in 63% of the patients.In patients with immunosuppressives, diarrhoea occurred in themedian 4 months after the start of therapy and diagnosis of WPwas made after another 2 months. Mahnel R, et al. Am J Gastroenterol 2005;100:1167-73.
  11. 11. Musculoskeletal manifestations• Arthralgia and arthritis• Spondyloarthritis• Myalgia and cramps
  12. 12. Musculoskeletal manifestations• Arthralgia and arthritis• Spondyloarthritis• Myalgia and cramps
  13. 13. Arthralgia and arthritis• Intermittent and migratory• Commonly involving ankles, knees, elbows shoulders and finger joints• Polyarthrtis is most common but oligoarthritis may occur• Chronic polyarthritis, which can be erosive and resembling rheumatoid arthritis, is less frequent
  14. 14. Musculoskeletal manifestations• Arthralgia and arthritis• Spondyloarthritis• Myalgia and cramps
  15. 15. SpondyloarthritisThe seronegative spondyloarthritides1. Uncomplicated ankylosing spondylitis2. Psoriatic arthritis3. Reiter’s disease4. Ulcerative colitis5. Crohn’s disease6. Behçet’s disease7. Whipple’s disease Moll JM, et al. Medicine (Baltimore) 1974;53:343-64.
  16. 16. Spondyloarthritis18 patients with coexisting Whipple’s disease (WP)and ankylosing spondylitis (AS).Moll and Wright could agree with the diagnosis ofAS in only 4 of these 18 patients. Kelly JJ, Weisinger BB. Arthritis Rheum 1963;6:615-32.
  17. 17. SpondyloarthritisSubsequently, several cases of coexistingWhipple’s disease and ankylosing spondylitishave been reported. Canoso JJ, et al.. J Rheumatol 1978;5:79-84. Blaison D, et al. Ann Gastroentérol Hépatol 1991;27:51-5.
  18. 18. SpondyloarthritisStudies examining patients with Whipple’s disease (WD)for sacroiliitis and ankylosing spondylitis (AS):• D’Eshougues R, et al. Rev Rhum 1976;43:565-73. 5 patients with sacroiliitis among 18 with WD• Khan MA. J Rheumatol 1982;9:928-9. 1 patient with AS among 6 with WD
  19. 19. SpondyloarthritisStudies on the frequency of HLA-B27 in patients withWhipple’s disease (WD):• Feurle GE, et al. Eur J Clin Invest1979;9:385-9. 4 of 9 patients with WD without sacroiliitis and AS were B27 positive• Dobbins WO III. Arthritis Rheum 1987;30:102-5. 13 (28%) of 48 WD patients typed were positive for the B27 antigen. 12 of the 48 patients had sacroiliitis and only 2 of them were B27 positive
  20. 20. SpondyloarthritisStudies on the frequency of HLA-B27 in patients withWhipple’s disease (WD):• Bai JC, et al. Am J Gastroenterol 1991;86:992-4. 14 Argentinian WD patients typed Frequency of the B27 antigen: 7% in patients with WD and 4% in blood donors
  21. 21. McCarty DJ, et al. Remitting seronegativesymmetrical synovitis with pitting edema.RS3PE syndrome. JAMA 1985;254:2763-7.• Esordio acuto• Sinovite dei polsi, carpi, piccole articolazioni delle mani e guaine dei flessori associata ad edema improntabile sul dorso delle mani• Negatività del fattore reumatoide• Indici di flogosi elevati• Risposta brillante alla terapia steroidea• Risoluzione con la restitutio ad intengrum• HLA B-7 nel 50% dei casi
  22. 22. Altre malattie infiammatorie• Polimialgia reumatica/arterite di Horton• Spondiloartriti• Condrocalcinosi• Artropatia amiloidosica• LES• Connettivite mista• Sindrome di Sjögren• Sclerosi sistemica• Dermatomiosite• Poliarterite nodosa• Malattia di Whipple• Sarcoidosi
  23. 23. Olivieri I, et al. J Rheumatol 2001;28:1294-7. • No patient with a family history of spondyloarthritis. • No patients with clinical manifestations of spondyloarthritis such as peripheral enthesitis, inflammatory spinal pain, chest wall pain or uveitis. • No patient with sacroiliitis on pelvis x-rays • No patient with the HLA-B27 antigen.
  24. 24. Practical Take Home Messages
  25. 25. Musculoskeletal manifestations of Whipple’s disease• Among the most common extra-intestinal symptoms• May precede the gastrointestinal complaints by many years• Intermittent and migratory arthralgia and arthritis.• Polyarthritis in most cases• Tenosinovial involvement recently described• Synovitis with pitting oedema over the hand and foot dorsum may occur• WD is not part of the spondyloarthritis complex

×