S. HarariS. Harari

U.O. di PneumologiaU.O. di Pneumologia

Ospedale S. Giuseppe, Ospedale S. Giuseppe, MilanMilan

ItalyItaly

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)

HistoryHistory

Metrorrhagia was also present

Her medical history was unremarcable

She had been healthy previously

Physical Physical ExaminationExamination

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

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

ECGECG

ECG indicated a normal heart rhythm

Transthoracic echocardiography showed no

abnormality

Chest RadiographyChest Radiography

Chest radiography was normal

Abdominal Abdominal ultrasoundultrasound

Mild ascites was present

QuestionQuestion

1)Diagnosis is clear!

2)Other evaluations are needed and abdominal CT is indicated

3)Diagnostic paracentesis is indicated

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

ParacentesisParacentesis

Ascitic fluidAscitic fluid

No neoplastic cells were present

Findings of a standard endoscopic diagnostic evaluation (ie, eosophagogastroduodenoscopy with small-bowel biopsy and ileocolonoscopy and endovaginal ultrasonography) were normal

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

QuestionQuestion

The clinical and macroscopic diagnosis is:

1) Tuberculosis

2) Carcinomatosis

3) Sarcoidosis

4) Fungal infection

5) Other

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

HistologyHistology

Cultures for ordinary bacteria and fungi were negative

No malignant cells were found

QuestionQuestion

The microscopic diagnosis is:

1) Tuberculosis

2) Carcinomatosis

3) Sarcoidosis

4) Fungal infection

5) Other

DIAGNOSISDIAGNOSIS

Peritoneal Peritoneal sarcoidosissarcoidosis

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

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

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

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

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

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)

Other evaluations…Other evaluations…

LaboratoryLaboratory

ACE level and serum calcium were normal

Tuberculin skin test was negative

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

Chest CTChest CT

QuestionQuestion

Pleural effusion is due to:

1) Pleural sarcoidosis

2) Laparoscopy

3)Hepatic disease

4)Cardiac dysfunction

5) Other

Pleural involvementPleural involvement

Pleural effusions are uncommon and are evident in only 1-4% of patients with sarcoidosisIn 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

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

BALBAL

Bronchoscopy findings were normal

BAL fluid showed an elevated lymphocyte number (39%) and a high CD4/CD8 cell ratio (8.2)

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

HistoryHistory

…3-months later…

Hypopigmented lesions

QuestionQuestion

1)Cutaneous sarcoidosis?

2)Adverse event due to antibiotic therapy? (prescribed during hospitalization)

3)Fungal infection?

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 Cutaneous sarcoidosissarcoidosis

“A dermatologic masquerader”

Katta R, Am Fam Physician 2002; 65: 1581-4

Cutaneous Cutaneous sarcoidosissarcoidosis

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

Cutaneous Cutaneous sarcoidosissarcoidosis

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 biopsyMana J et al. Arch Dermatol 1997; 133: 882-8

Cutaneous Cutaneous sarcoidosissarcoidosis

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 performed

English JC et al. J Am Acad Dermatol 2001; 44: 725-43

Baughman et al. Lancet 2003; 361: 1111-8

Common presentations and Common presentations and differential diagnoses of differential diagnoses of cutaneous sarcoidosiscutaneous sarcoidosis

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 erythrodermaKatta R, Am Fam Physician 2002; 65: 1581-4

Cutaneous Cutaneous sarcoidosissarcoidosis

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

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

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

ConclusionsConclusions

Extrapulmonary symptoms appear to be a considerable problem in chronic sarcoidosis Multidisciplinary approch could Multidisciplinary approch could

improve diagnosis, treatment improve diagnosis, treatment and and

knowledge regarding many knowledge regarding many aspects aspects

of this intriguing diseaseof this intriguing disease