Sarcoidosis in TurkeySarcoidosis in Turkey

Dr.Göksel KITERDr.Göksel KITER

Pamukkale University, Medical Pamukkale University, Medical Faculty Faculty

Pulmonary Medicine DepartmentPulmonary Medicine Department

Main topicsMain topics

Overview to the studies on Turkish Overview to the studies on Turkish Sarcoidosis casesSarcoidosis cases

““Sarcoidosis” study preformed by Turkish Sarcoidosis” study preformed by Turkish Thoracic SocietyThoracic Society

The results obtained from that studyThe results obtained from that study

Lessons that we’ve learned in that processLessons that we’ve learned in that process

ACCESSACCESS

Case control studyCase control study Aimed to evaluate the etiological aspectAimed to evaluate the etiological aspect November 1996-June 1999November 1996-June 1999 10 centers; 720 patients and 720 controls10 centers; 720 patients and 720 controls

Turkish Sarcoidosis Case Turkish Sarcoidosis Case SeriesSeries

Gürkan ÖU, Çelik G, Kumbasar Ö ve ark. Sarcoidosis in Turkey:1954-2000. Ann Saudi Med Gürkan ÖU, Çelik G, Kumbasar Ö ve ark. Sarcoidosis in Turkey:1954-2000. Ann Saudi Med 2004 2004

Tabak L, Kılıçarslan Z, Kıyan E ve ark. 147 sarkoidoz hastasının klinik özellikleri. Solunum Tabak L, Kılıçarslan Z, Kıyan E ve ark. 147 sarkoidoz hastasının klinik özellikleri. Solunum 20012001

Sağlam L, Kaynar H, Akgün M ve ark. The retrospective analysis of our patients with Sağlam L, Kaynar H, Akgün M ve ark. The retrospective analysis of our patients with sarcoidosis. 2001sarcoidosis. 2001

Aytemur ZA, Erdinç M, Erdinç E, ve ark. Sarkoidozda evrelere göre klinik özellikler ve tanısal Aytemur ZA, Erdinç M, Erdinç E, ve ark. Sarkoidozda evrelere göre klinik özellikler ve tanısal yaklaşım. Tüberküloz ve Toraks 2003yaklaşım. Tüberküloz ve Toraks 2003

Yalnız E, Kömürcüoğlu A, Polat GE, ve ark. Sarkoidozda klinik, radyolojik, laboratuarla ilgili Yalnız E, Kömürcüoğlu A, Polat GE, ve ark. Sarkoidozda klinik, radyolojik, laboratuarla ilgili parametreler ve tanı yöntemleri. Toraks Dergisi 2003parametreler ve tanı yöntemleri. Toraks Dergisi 2003

Erbaycu AE, Uçar ZZ, Çakan A, ve ark. Seasonal clustering of sarcoidosis in spring in Turkey. Erbaycu AE, Uçar ZZ, Çakan A, ve ark. Seasonal clustering of sarcoidosis in spring in Turkey. Turkish Respiratory Journal, 2004.Turkish Respiratory Journal, 2004.

Baran A, Özşeker F, Güneylioğlu D, ve ark. Sarkoidoz: Yedi yıllık deneyim. Toraks Dergisi Baran A, Özşeker F, Güneylioğlu D, ve ark. Sarkoidoz: Yedi yıllık deneyim. Toraks Dergisi 20042004

Demirkök SS, Başaranoğlu M, Akıncı ED, Karayel T. Analysis of 275 patients with sarcoidosis Demirkök SS, Başaranoğlu M, Akıncı ED, Karayel T. Analysis of 275 patients with sarcoidosis over a 38 year period; a single-instutition experience. Respiratory Medicine 2006over a 38 year period; a single-instutition experience. Respiratory Medicine 2006

1954-20001954-20001327 patients from 29 1327 patients from 29 seriesseries 1990-2000, Istanbul 1990-2000, Istanbul

Medical Faculty; 147 Medical Faculty; 147 casescases1991-2002, Erzurum, 1991-2002, Erzurum,

Atatürk University; 25 Atatürk University; 25 casescases 1990-1998, Izmir, Ege 1990-1998, Izmir, Ege

University; 77 casesUniversity; 77 cases1995-2001, Izmir Chest 1995-2001, Izmir Chest Hospital; 73 casesHospital; 73 cases

1994-2003, Izmir Chest 1994-2003, Izmir Chest hospital; 86 caseshospital; 86 cases

1994-2000, Istanbul 1994-2000, Istanbul Sureyyapasa Chest Sureyyapasa Chest Hospital; 70 casesHospital; 70 cases 1966-2004, Istanbul 1966-2004, Istanbul

Cerrahpasa Medical Cerrahpasa Medical Faculty; 275 casesFaculty; 275 cases

Gürkan Tabak Sağlam Aytemur Yalnız Erbaycu Baran Demirkök

N 1327 147 25 77 73 86 70 275

M/F 1,8 2,1 2,1 2,3 2,7 5,1 3,1 2,2

Age 30-48 39±13 38±13 43 45±13 45±16 44±12 40±13

Nonsmoker %70 %71,5

Gürkan Tabak Sağlam Aytemur Yalnız Erbaycu Baran Demirkök

EN 10 29 38 26 15 11 29

Skin 1,6 5 3 5 19 26

Eye 5 10 0 5 3 8 7 11

Peripheral LAP 9 1 11 4 68 6 19

HM 5 4 6 7

SM 3,5 3 3 6

Heart 2 3 1

Nervous 1 2 3,6 5 3

Renal 1,3

Joint 2 0 8 27

Parotid gland 4 0 1,4 7

Turkish Thoracic Society Turkish Thoracic Society Clinical Problems Working Clinical Problems Working

GroupGroup““Sarcoidosis” studySarcoidosis” study

Aim of the studyAim of the study

To find-out the epidemiological features of To find-out the epidemiological features of sarcoidosis in Turkeysarcoidosis in Turkey

To evaluate the pulmonary involvementTo evaluate the pulmonary involvement

To evaluate the utility of diagnostic approachsTo evaluate the utility of diagnostic approachs

To assess the extra-pulmonary involvementTo assess the extra-pulmonary involvement

To make a comment on geographical disturbanceTo make a comment on geographical disturbance

1 June 2004 – 31 May 20061 June 2004 – 31 May 2006

12 cities, 19 centers, 24 12 cities, 19 centers, 24 colleaguescolleagues

293 new patients 293 new patients

5,0 2,8 6,8

7,0 3,1

4,6

4,2

9,1

10,6

0,9

2,6

Incidence 4 in 100 000

Female / Male = 2,08Female / Male = 2,08

Mean age: 44 ± 13

1 in 3 cases was above 50 age

♀ Mean 47 ± 13

♂ Mean 37 ± 12

p<0,001

Smoking historySmoking history

74%

21%

5%

NonsmokerCurent smokerEx-smoker

SymptomsSymptomsSymptom N %

Cough 156 53.2

Dyspnea 118 40.3

Sputum 40 13.7

Hemopthysis 7 2.4

Chest pain 66 22.5

PULMONARY (Total) 216 73.7

Fatigue 113 38.6

Weight loss 53 18.1

Fever 39 13.3

Night sweating 7 2.3

CONSTITUTIONAL (Total) 148 50.5

Symptom N %

Erythema nodosum 63 21.5

Joint pain 61 20.8

Back pain 48 16.4

Skin lesion 47 16

Joint swelling 17 5.8

Myalgia 14 4.8

Ocular findings 9 3.1

Head ache 9 3.1

Mass in neck 8 2.7

Bone pain 6 2

Sweating 6 2

Dyspepsia 5 1.7

Paresthesia 5 1.7

Diarrhea 4 1.4

Difficulty in movements 3 1

Dry mouth 3 1

Palpitation 2 0.7

Dizziness 2 0.7

Subcutaneous mass 1 0.3

Dry eye 1 0.3

Other 12 4

EXTRAPULMONARY (total) 181 61.8

Extrapulmonary Symptom N %

Erythema nodosum 63 21.5

Joint pain 61 20.8

Back pain 48 16.4

Skin lesion 47 16

Joint swelling 17 5.8

Myalgia 14 4.8

Ocular findings 9 3.1

Head ache 9 3.1

Mass in neck 8 2.7

Bone pain 6 2

Sweating 6 2

Sign N %

Erythema nodosum 50 17,1

Inspiratory crackles 36 12,3

Peripheral LAM 33 11,3

Splenomegaly 12 4,1

Hepatomegaly 11 3,8

Wheezing 10 3,4

Parotis gland enlargement 4 1,4

Cyanosis 1 0,3

Radiological finding N %

Hilar lymphadenomegaly 263 89.8

Bilateral 231 78.8

Right 26 8.9

Left 6 2.0

Nodular involvement 98 33.4

Paratracheal lymphadenomegaly

75 25.6

Reticular infiltration 31 10.6

Pleurisy 5 1.7

Pneumothorax 7 2.4

Honey combing 2 0.7

Normal 14 4.8

Radiological stageRadiological stage

Stage n %

Stage 0 14 4.8

Stage I 152 51.9

Stage II 110 37.5

Stage III 15 5.1

Stage IV 2 0.7

CT findings N %

Mediastinal lymphadenomegaly 270 92.2

Nodules 143 48.8

Reticular infiltration 62 21.2

Ground-glass apperence 50 17.1

Alveolar sarcoidosis 13 4.4

Pleural calsification 13 4.4

Calsified lymphadenomegaly 12 4.1

Bronchiectases 9 3.1

Diffuse fibrosis 8 2.7

Pleurisy 8 2.7

Air-trapping 7 2.4

Honey combing 5 1.7

Cavity 1 0.3

CT not performed 7 2.4

Parameter Mean ± SD %pred.

FVC 3198 ± 1179 92.2 ± 42.8

FEV1 2546 ± 946 86.1 ± 19.0

FEV1/FVC 81.3 ± 11.1

MMEF 2.7 ± 1.4 71.0 ± 28.8

DLco 20.5 ± 8.5 82.6 ± 21.3

DLco/VA 4.4 ± 1.3 95.5 ± 23.0

PFT was performed in 257 patients, CO diffusion test was performed in 161 patients

Gurkan OU, Celik G, Kumbasar O et al. Gurkan OU, Celik G, Kumbasar O et al. Sarcoidosis in Turkey:1954-2000. Ann Saudi Sarcoidosis in Turkey:1954-2000. Ann Saudi

Med 2004 Med 2004

Bronchoscopic findings N %

Mucosal hyperemia 46 19.8

External bronchial compression 39 16.8

Nodular lesion 14 6.0

Mucosal infiltration 14 6.0

Bronchoconstriction 2 0.9

Intrabronchial mass 1 0.4

No abnormal finding 86 37.1

Bronchoscopy not performed 62 21.1

BALF analysesBALF analyses

In 79 sarcoidosis patients (26.9%)In 79 sarcoidosis patients (26.9%) Lymphocytic plus neutrofilic alveolitis Lymphocytic plus neutrofilic alveolitis

46.8%46.8% Lone lymphocytic alveolitis 34.2% Lone lymphocytic alveolitis 34.2% Lone neutrophilic alveolitis 5.1%Lone neutrophilic alveolitis 5.1% Mixt alveolitis 5.1%Mixt alveolitis 5.1% No alveolitis 8.9% No alveolitis 8.9%

Only Neutrophilic plus lymphositic Only Neutrophilic plus lymphositic alveolitis was found associated with only alveolitis was found associated with only being recently active smoker (p=0.03). being recently active smoker (p=0.03).

Demirkök SS, Başaranoğlu M, Akıncı ED, Demirkök SS, Başaranoğlu M, Akıncı ED, Karayel T. Analysis of 275 patients with Karayel T. Analysis of 275 patients with

sarcoidosis over a 38 year period; a single-sarcoidosis over a 38 year period; a single-instutition experience. Respiratory Medicine instutition experience. Respiratory Medicine

20062006

Sampling method N % Success rate

Transbronchial biopsy 127 43.3 48.8

Bronchial mucosa biopsy 95 32.4 52.6

Mediastinoscopy 90 30.7 100.0

Transbronchial needle aspiration

68 23.2 44.1

Skin biopsy 28 9.6 75.0

Peripheral lymph node biopsy

18 6.1 94.4

Scalen lymph node biopsy 12 4.1 83.3

Open lung biopsy 9 3.1 88.9

Mediastinotomy 5 1.7 100.0

VATS 3 1.0 100.0

Transthoracic biopsy 2 0.7 100.0

ACCESSACCESS LiteratureLiterature

%% %%

LungLung 99,099,0 95,095,0 9090

Erythema nodosumErythema nodosum 22,022,0 8,28,2 3-253-25

Skin except Erythema nodosumSkin except Erythema nodosum 16,416,4 15,915,9 10-3510-35

Peripheral lymph nodePeripheral lymph node 13,813,8 15,215,2 1515

JointJoint 6,86,8 0,50,5 25-3925-39

Eye*Eye* 5,95,9 11,811,8 25-6025-60

Neurolojic systemNeurolojic system 3,33,3 4,64,6 5-125-12

HeartHeart 3,33,3 2,32,3 55

Saliva gland and parotisSaliva gland and parotis 14,314,3 3,93,9 1010

Tyroid glandTyroid gland 2,62,6 00 44

Upper air wayUpper air way 1,11,1 3,03,0 99

RenalRenal 0,90,9 0,70,7 44* More

in above age 40

Older patients were mostly living at sea-side cities; p=0,006

GEOGRAPHIC DIFFERENCESGEOGRAPHIC DIFFERENCES

ConclusionConclusion Turkish sarcoidosis cases, especially females, were older Turkish sarcoidosis cases, especially females, were older

than the literature. Female/male=2than the literature. Female/male=2

Smokers were in 20%; similar to published dataSmokers were in 20%; similar to published data

Pulmonary symptoms were frequent (Cough the most)Pulmonary symptoms were frequent (Cough the most)

Constitutional symptoms in half of the patients (fever the Constitutional symptoms in half of the patients (fever the most)most)

Lower eye and neurological involvement ratiosLower eye and neurological involvement ratios

Elderly patients in the areas near to the seaElderly patients in the areas near to the sea

Histopathological confirmation rate was high (%90,4); Histopathological confirmation rate was high (%90,4); bronchoscopy was yielded in half of the patients, bronchoscopy was yielded in half of the patients, mediastinoscopy was used not very rarely.mediastinoscopy was used not very rarely.

LESSONS WE’VE LEARNEDLESSONS WE’VE LEARNED It should be forced to include patients It should be forced to include patients

representing all geographic areas.representing all geographic areas. All physicians should be attend to the study.All physicians should be attend to the study. 2.5 fold attendence to ILD study.2.5 fold attendence to ILD study. Internet is a useful tool.Internet is a useful tool. Method must be described more precisely.Method must be described more precisely. That study design is worked very-well. That study design is worked very-well.

Valuable epidemiologic data have been Valuable epidemiologic data have been obtained.obtained.

A data base has been created for further A data base has been created for further studies on treatment and follow-up.studies on treatment and follow-up.