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  1. 1. S. HarariS. Harari U.O. di PneumologiaU.O. di Pneumologia Ospedale S. Giuseppe,Ospedale S. Giuseppe, MilanMilan
  2. 2. HistoryHistory A 40-year-old hispanic woman came to the emergency department because of a 1- month history of abdominal pain, increasing abdominal girth and fever She also reported thoracic pain and arthralgia No cough, night sweats, no other abdominal symptoms (nausea, vomiting, diarrhea, melena or changes in bowel habits)
  3. 3. HistoryHistory Metrorrhagia was also present Her medical history was unremarcable She had been healthy previously
  4. 4. Physical ExaminationPhysical Examination General condition was well-preserved; she had normal vital signs Chest and cardiac examinations were normal Abdomen was soft but diffusely tender Gynecological examination was normal
  5. 5. LaboratoryLaboratory Laboratory findings on hospital admission revealed an elevated erythrocyte sedimentation rate (57 mm), an elevated C-reactive protein level (6.22 mg/dL) CA 125 was elevated (118 U/mL) Liver enzymes and direct and indirect bilirubin levels were normal
  6. 6. ECGECG ECG indicated a normal heart rhythm Transthoracic echocardiography showed no abnormality
  7. 7. Chest RadiographyChest Radiography Chest radiography was normal
  8. 8. Abdominal ultrasoundAbdominal ultrasound Mild ascites was present
  9. 9. QuestionQuestion 1) Diagnosis is clear! 2) Other evaluations are needed and abdominal CT is indicated 3) Diagnostic paracentesis is indicated
  10. 10. Abdominal CTAbdominal CT No liver abnormality were present Ascites was present with mild but diffuse soft-tissue thickening involving the mesentery and rare small mesenteric nodules were detected
  11. 11. ParacentesisParacentesis
  12. 12. Ascitic fluidAscitic fluid No neoplastic cells were present
  13. 13. Findings of a standard endoscopic diagnostic evaluation (ie, eosophagogastroduodenoscopy with small-bowel biopsy and ileocolonoscopy and endovaginal ultrasonography) were normal
  14. 14. LaparoscopyLaparoscopy On laparoscopy multiple small white nodules in the peritoneum cavity were present. A moderate amount of ascitic fluid was found in the peritoneal cavity Biopsy of the peritoneal nodules was performed and ascitic fluid was sampled
  15. 15. QuestionQuestion The clinical and macroscopic diagnosis is: 1) Tuberculosis 2) Carcinomatosis 3) Sarcoidosis 4) Fungal infection 5) Other
  16. 16. HistologyHistology Histologic findings consisting of multiple, well-formed, noncaseating granulomas that were composed of aggregates of tightly clustered epithelioid cells with some giant cells without central necrosis Acid-fast stain for mycobacteria and Grocott’s methenamine silver stain for fungi were negative
  17. 17. HistologyHistology Cultures for ordinary bacteria and fungi were negative No malignant cells were found
  18. 18. QuestionQuestion The microscopic diagnosis is: 1) Tuberculosis 2) Carcinomatosis 3) Sarcoidosis 4) Fungal infection 5) Other
  19. 19. DIAGNOSISDIAGNOSIS PeritonealPeritoneal sarcoidosissarcoidosis
  20. 20. SarcoidosisSarcoidosis Sarcoidosis is a systemic disease that can involve almost any organ system Infiltration with noncaseating granulomas is the hallmark of the disease, and it may result in various clinical manifestations Am J Respir Crit Care Med 1999; 160: 736-55
  21. 21. SarcoidosisSarcoidosis Unfortunately, no single test can prove the diagnosis Patients are diagnosed with sarcoidosis when a compatible clinical or radiologic picture is present, along with histologic evidence of noncaseating granulomas, and when other potential causes, such as infections, are excluded Am J Respir Crit Care Med 1999; 160: 736-55
  22. 22. DiscussionDiscussion Sarcoidosis of the serosal surfaces is reported rarely, especially when it is the unique manifestation of the disease “The serous surfaces of the body cavities appear as though almost immune to the disease” Longcope WT, Freiman DG. Medicine, 1952 Peritoneal involvement by sarcoidosis is very infrequent: 20 cases are reported in the english literature
  23. 23. Most of these cases presented with ascites (both bloody and nonbloody), which usually had a benign course, resolving either spontaneously or whith a short course of corticosteroid therapy Subacute abdominal distention by ascites, slight adominal pain and a well-preserved general condition are often presented DiscussionDiscussion
  24. 24. Sarcoidosis may extensively involve the peritoneum, and the lesions may closely simulate carcinomatosis or tuberculous peritonitis Peritoneal sarcoidosis should be considered in the differential diagnosis of peritoneal nodules and ascites DiscussionDiscussion Uthaman IW et al. Seminars in Arthritis and Rheumatism, 2002; 31; 353 Uthaman IW et al. Seminars in Arthritis and Rheumatism, 1999; 28: 351-54
  25. 25. DiscussionDiscussion Other miscellaneous presentations of peritoneal sarcoidosis included an adnexal mass and elevated CA 125, a serum marker for malignant tumors CA 125 is elevated in many malignant and non-malignant conditions (cirrhosis of liver, CHF, DM, pericarditis, sarcoidosissarcoidosis, tuberculosis, endometriosis, menstruation, pregnancy and pelvic inflammatory disease)
  26. 26. Other evaluations…Other evaluations…
  27. 27. LaboratoryLaboratory ACE level and serum calcium were normal Tuberculin skin test was negative
  28. 28. PFTPFT Mild restrictive pattern with decreased diffusing capacity (VC 2.27L 76%, FVC 2.27L 75%, FEV1 1.99L 77%, FEV1/SVC 88%, TLC 3.08L 67%, RV 0.81L 55%, DLCO 62%, DLCO/VA 114%) ABG and 6-MWT were normal
  29. 29. Chest CTChest CT
  30. 30. QuestionQuestion Pleural effusion is due to: 1) Pleural sarcoidosis 2) Laparoscopy 3) Hepatic disease 4) Cardiac dysfunction 5) Other
  31. 31. Pleural involvementPleural involvement Pleural effusions are uncommon and are evident in only 1-4% of patients with sarcoidosis In most reported cases, the pleural effusion was an incidental finding on the physical examination or on the chest radiographs No specific radiological features of the effusion suggest that it is related to sarcoidosis
  32. 32. Pleural involvementPleural involvement The concurrent findings of intrathoracic lymphadenopathy or the parenchymal disease may suggest the cause Pleural thickening may be seen in association with pleural effusion and is usually confined to the lower lobes Lynch JP et al. Clin Chest Med 1997; 18: 755-85
  33. 33. BALBAL Bronchoscopy findings were normal BAL fluid showed an elevated lymphocyte number (39%) and a high CD4/CD8 cell ratio (8.2)
  34. 34. ManagementManagement Oral prednisone was started (1 mg/kg) 1 months after the discharge, she was doing well with no abdominal complaints and fever Steroid therapy was tapered
  35. 35. HistoryHistory …3-months later…
  36. 36. Hypopigmented lesions
  37. 37. QuestionQuestion 1) Cutaneous sarcoidosis? 2) Adverse event due to antibiotic therapy? (prescribed during hospitalization) 3) Fungal infection?
  38. 38. Cutaneous sarcoidosis is known as one of the “great imitators” in dermatology Involvement may be mild or severe, self- limited or chronic, and limited or wide- ranging in extent Correctly diagnosing sarcoidosis may be a challenge Cutaneous sarcoidosisCutaneous sarcoidosis “A dermatologic masquerader” Katta R, Am Fam Physician 2002; 65: 1581-4
  39. 39. Cutaneous sarcoidosisCutaneous sarcoidosis Cutaneous involvement occurs in 20 to 35% of patients with systemic sarcoidosis and may occur without systemic involvement Recognition of cutaneous lesions is important because they provide a visible clue to the diagnosis and are an easily accessible source of tissue for histologic examination Newman LS et al. N Engl J Med 1997; 336; 1224-34
  40. 40. Cutaneous sarcoidosisCutaneous sarcoidosis Most authors divide lesions of cutaneous sarcoidosis into nonspecific and specific types Although nonspecific lesions occur in association with systemic sarcoidosis, no granulomas are found on biopsy Specific lesions display noncaseating granulomas on biopsy Mana J et al. Arch Dermatol 1997; 133: 882-8
  41. 41. Cutaneous sarcoidosisCutaneous sarcoidosis Despite this same histologic appearance, clinical appearance of the specific lesions may be markedly variable The term “specific” is misleading because the clinical appearance of such lesions is usually not specific for sarcoidosis, and the correct diagnosis is often reached only after skin biopsy is performedEnglish JC et al. J Am Acad Dermatol 2001; 44: 725-43 Baughman et al. Lancet 2003; 361: 1111-8
  42. 42. Common presentations andCommon presentations and differential diagnoses of cutaneousdifferential diagnoses of cutaneous sarcoidosissarcoidosis Papules: Granulomatous rosacea, Acne, Benign appendageal tumors Plaques: Psoriasis, Lichen planus, Nummular eczema, Discoid lupus erythematosus, Granuloma annulare, Cutaneous T-cell lymphoma, Kaposi’s sarcoma, Secondary syphilis Lupus pernio: Scar, Discoid lupus erythematous Erythema nodosum: cellulitis, Furunculosis, Other inflammatory panniculitis Other: scarring and nonscarring alopecia, ulcerative sarcoidosis, hypopigmented patches, ichthyosis of the lower legs, subcutaneous nodules and erythroderma Katta R, Am Fam Physician 2002; 65: 1581-4
  43. 43. Cutaneous sarcoidosisCutaneous sarcoidosis It is thought that specific skin lesions do not have prognostic significance and do not correlate with the presence of systemic disease Although cutaneous involvement may occur at any stage of the disease, it is most often present at the onset
  44. 44. ConclusionsConclusions Sarcoidosis is a diagnosis of exclusion, supported by the finding of negative cultures and non-caseating epithelioid granulomas The management of sarcoidosis is generally coordinated by a pulmonary physician However, sarcoidosis can involve any organ
  45. 45. ConclusionsConclusions Sarcoidosis patients not only suffer from symptoms related to the lung, but they may also suffer from a wide spectrum of other symptoms These symptoms include persistent fatigue, arthralgias, muscle pain, weight loss, skin lesions, eye problems and neurological as well as cardiological problems
  46. 46. ConclusionsConclusions Extrapulmonary symptoms appear to be a considerable problem in chronic sarcoidosis Multidisciplinary approch couldMultidisciplinary approch could improve diagnosis, treatment andimprove diagnosis, treatment and knowledge regarding many aspectsknowledge regarding many aspects of this intriguing diseaseof this intriguing disease