Cytokines in cerebrospinal fluid of children with West syndrome

In this study we aimed to determine the levels of tumor necrosis factor beta (TNF-b), interleukin (IL) 1-beta (IL-1b), IL-5, IL-10, and interferon gamma (IFN-g ) in CSF from children during the onset of West syndrome (WS). We observed elevated levels of IL-1b and IFN-g correlated to clinical, EEG, therapeutic response, and follow-up suggesting the involvement of immune response in WS. These results suggest that inflammatory and immunologic mediators may play a role in the pathophysiologic mechanisms of infantile spasms. Our findings may explain the perfusion and cognitive disfunctions and actions of adrenocorticotropic hormone (ACTH), corticosteroids, and intravenous immunoglobulin (IVIg) observed in WS. In conclusion, WS results from association of neurophysiological mechanisms and structural abnormalities with participation of cytokines mainly in symptomatic group.

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Clinical, neuroimaging and developmental outcomes of West syndrome

INTERNATIONAL NEUROLOGICAL JOURNAL ◽

10.22141/2224-0713.17.3.2021.231566 ◽

2021 ◽

Vol 17 (3) ◽

pp. 5-11

Author(s):

Kemal Karataş ◽

Hüseyin Tan ◽

Şenay Güven Baysal

Keyword(s):

Adrenocorticotropic Hormone ◽

West Syndrome ◽

Developmental Screening ◽

Positive Contribution ◽

Biochemical Tests ◽

Neurodevelopmental Outcomes ◽

Laboratory Findings ◽

Symptomatic Group ◽

Significant Difference ◽

Treatment Responses

Background. West syndrome is characterized by a hipsarrhytmia pattern on electroencephalography, spasm type seizures, and psychomotor regression triad. In this study, we aimed to document demographic characteristics, clinical and laboratory findings, treatment responses, neurodevelopmental outcomes, and risk factors developed during long-term follow-up with the diagnosis of West syndrome. Materials and methods. The study included patients who were diagnosed with West syndrome between July 2011 and December 2012 in the Department of Pediatric Neurology of Atatürk University. The following data were collected from each patient’s history. Systemic and neurological examinations, cerebral imaging, and electroencephalography were reviewed. Biochemical tests were performed from laboratory tests. The development of each child was assessed using the Denver 2 developmental screening test, the Ankara Developmental Screening Inventory test. Results. The ratio of male/female of our patients was found 2,28/1 and the mean age of the referral was 8.62 ± 7.20 months (median: 8.0). 59 (85.51 %) of the patients were in the symptomatic group and 10 (14.49 %) were in the idiopathic group. The most common factors in the symptomatic group were anomalies of the congenital central nervous system (45.7 %) and hypoxic-ischemic encephalopathy (28.8 %). There was a significant difference between the idiopathic and symptomatic groups in terms of relapse rates, radiological findings, and prognosis rates (p: 0.035/p < 0.001/p < 0.001). Relapse was detected in 43,5 % of the patients. While 83 % of patients treated with adrenocorticotropic hormone, treatment was responded to, 17 % had resistant seizures. There was no statistically significant difference between the treatment responses with adrenocorticotropic hormone and other treatments (p = 0.093). 46 of 55 children (83.6 %) were found to be a developmental delay. Conclusions. Early diagnosis and treatment, the provision of appropriate and convenient treatment in the West syndrome can make a positive contribution to prognosis according to etiology.

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POS0204 AUTOANTIBODIES AND SYSTEMIC LUPUS ERYTHEMATOSUS INDUCED BY ANTI-TUMOUR NECROSIS FACTOR ALPHA THERAPY IN RHEUMATOID ARTHRITIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3255 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 318.1-318

Author(s):

D. Santos Oliveira ◽

A. Martins ◽

F. R. Martins ◽

F. Oliveira Pinheiro ◽

M. Rato ◽

...

Keyword(s):

Lupus Erythematosus ◽

Disease Duration ◽

Seroconversion Rate ◽

Necrosis Factor Alpha ◽

Malar Rash ◽

Tnf Α ◽

Factor Alpha ◽

Necrosis Factor ◽

Over Time

Background:Anti-tumour necrosis factor alpha (anti-TNF-α) therapy is commonly used to treat inflammatory conditions such as rheumatoid arthritis (RA). Autoantibodies namely antinuclear antibodies (ANA) induced by these treatments are well established. However, anti-TNF-α-induced systemic lupus erythematosus (SLE) is rarely described and its incidence is yet unknown.Objectives:This study aimed to determine the prevalence of ANA seroconversion and to characterize the development of SLE induced by anti-TNF-α therapy in patients with RA over time.Methods:An observational retrospective cohort study was conducted with at least one year of follow-up. Patients with diagnosis of RA, according to American College of Rheumatology criteria (ACR), and registered on Rheumatic Diseases Portuguese Register (Reuma.pt) who started their first anti-TNFα between 2003 and 2019 were included. Patients with positive ANA (titer ≥100) and/or positive double-strand DNA (dsDNA) antibodies and/or with a diagnosis of SLE at their first visit were excluded. Demographic, clinical and laboratory data were obtained by consulting Reuma.pt. As there are no recognized criteria for drug-induced SLE, the diagnosis of SLE induced by anti-TNF-α was considered if there is a temporal relationship between clinical manifestations and anti-TNF-α-therapy, the presence of at least 1 serologic ACR criteria (ANA or anti-dsDNA) and at least 1 nonserologic ACR criteria (arthritis, serositis, hematologic disorder or malar rash) [1]. Continuous variables are presented with mean, standard deviation, median, quartile 1 and quartile 3. Categorical variables are presented with absolute and relative frequencies.Results:A total of 211 patients (mean age of 49.9±10.9 years old; 84.4% female) were included with a median follow-up time of 6 [3-14] years. We found a seroconversion rate for ANA of 75.4% (n=159) with median treatment duration of 31 [8.5-70.5] months. The most common titre was 1/100 with diffuse and speckled patterns. ANA seroconversion was higher for etanercept (47.8%, n=76) than with adalimumab (23.9%, n=38), infliximab (13.8%, n=22), golimumab (12.6%, n=20) or certolizumab (1.9%, n=3). SLE induced by anti-TNF-α occurred in two patients (0.9%) with erosive and seropositive (rheumatoid factor and anti-citrullinated protein antibodies) RA previously treated with two conventional synthetic disease-modifying antirheumatic drugs, including methotrexate. The first patient, a female with 66 years old and 17 years of disease duration, developed SLE after 16 months of infliximab, with constitutional symptoms, abrupt worsening of polyarthritis, ANA titer of 1/320 diffuse pattern and positive dsDNA (248 UI/mL) antibodies. The second patient, a woman with 43 years old and 11 years of disease duration, developed SLE after 41 months of adalimumab with malar rash and ANA titer of 1/320 diffuse pattern, positive dsDNA (285 UI/mL), positive anti-histone antibodies and hypocomplementemia. In these two cases, anti-TNF-α therapy was stopped and recovery was spontaneous without treatment. The first patient switched to adalimumab and the second switched to golimumab without recurrence of SLE for more than ten years.Conclusion:We found a high rate of ANA seroconversion induced by anti-TNFα therapy in patients with RA. However, similar to previous literature, only 0.9% of patients developed SLE with mild manifestations without major organ involvement. Although the drug with the highest ANA seroconversion rate was etanercept, those responsible for induced SLE were infliximab and adalimumab. Patients improved after discontinuation of therapy and tolerated an alternative anti-TNF-α drug without recurrence of induced SLE over time. Therefore, ANA and SLE induced by anti-TNF-α should be considered and reported in the follow-up of RA patients. Further research is needed to explore the impact of this adverse event on the outcomes of treatment over time.References:[1]Hochberg MC. Arthritis Rheum. 1997;40(9):1725.Disclosure of Interests:None declared

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Association of biological antirheumatic therapy with risk for type 2 diabetes: a retrospective cohort study in incident rheumatoid arthritis

BMJ Open ◽

10.1136/bmjopen-2020-042246 ◽

2021 ◽

Vol 11 (6) ◽

pp. e042246

Author(s):

Sanjoy K Paul ◽

Olga Montvida ◽

Jennie H Best ◽

Sara Gale ◽

Attila Pethö-Schramm ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Type 2 Diabetes ◽

T Cell ◽

Disease Modifying Antirheumatic Drugs ◽

Antirheumatic Therapy ◽

Treatment Groups ◽

Significant Difference ◽

Necrosis Factor

ObjectiveTo explore possible associations of treatment with biological disease-modifying antirheumatic drugs (bDMARDs), including T-cell-based and interleukin-6 inhibition (IL-6i)-based therapies, and the risk for type 2 diabetes mellitus (T2DM) in patients with rheumatoid arthritis (RA).Study design, setting and participantsFive treatment groups were selected from a United States Electronic Medical Records database of 283 756 patients with RA (mean follow-up, 5 years): never received bDMARD (No bDMARD, n=125 337), tumour necrosis factor inhibitors (TNFi, n=34 873), IL-6i (n=1884), T-cell inhibitors (n=5935) and IL-6i+T cell inhibitor abatacept (n=1213). Probability and risk for T2DM were estimated with adjustment for relevant confounders.ResultsIn the cohort of 169 242 patients with a mean 4.5 years of follow-up and a mean 641 200 person years of follow-up, the adjusted probability of developing T2DM was significantly lower in the IL-6i (probability, 1%; 95% CI 0.6 to 2.0), T-cell inhibitor (probability, 3%; 95% CI 2.3 to 3.3) and IL-6i+T cell inhibitor (probability, 2%; 95% CI 0.1 to 2.9) groups than in the No bDMARD (probability, 5%; 95% CI 4.6 to 4.9) and TNFi (probability, 4%; 95% CI 3.7 to 4.7) groups. Compared with No bDMARD, the IL-6i and IL-6i+T cell inhibitor groups had 37% (95% CI of HR 0.42 to 0.96) and 34% (95% CI of HR 0.46 to 0.93) significantly lower risk for T2DM, respectively; there was no significant difference in risk in the TNFi (HR 0.99; 95% CI 0.93 to 1.06) and T-cell inhibitor (HR 0.96; 95% CI 0.82 to 1.12) groups.ConclusionsTreatment with IL-6i, with or without T-cell inhibitors, was associated with reduced risk for T2DM compared with TNFi or No bDMARDs; a less pronounced association was observed for the T-cell inhibitor abatacept.

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POS1021 THE PsABio STUDY IN ITALY: A REAL-WORLD COMPARISON OF THE PERSISTENCE, EFFECTIVENESS AND SAFETY OF USTEKINUMAB AND TUMOUR NECROSIS FACTOR INHIBITORS IN PATIENTS WITH PSORIATIC ARTHRITIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.81 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 778-779

Author(s):

E. Gremese ◽

F. Ciccia ◽

C. Selmi ◽

G. Cuomo ◽

R. Foti ◽

...

Keyword(s):

Adverse Event ◽

Psoriatic Arthritis ◽

Disease Activity ◽

Tumour Necrosis Factor ◽

Tumour Necrosis ◽

Research Support ◽

Treatment Persistence ◽

Low Disease Activity ◽

Necrosis Factor

Background:There are still unmet needs in the treatment of psoriatic arthritis (PsA), including in terms of treatment persistence, which is a function of effectiveness, safety and patient satisfaction. Ustekinumab (UST) was the first new biologic drug to be developed for the treatment of PsA after tumour necrosis factor inhibitors (TNFi).Objectives:To compare treatment persistence, effectiveness and safety of UST and TNFi in Italian patients within the PsABio cohort.Methods:PsABio (NCT02627768) is an observational study of 1st/2nd/3rd-line UST or TNFi treatment in PsA in 8 European countries. The current analysis set includes 222 eligible patients treated in 15 Italian centres, followed to Month 12 (±3 months). Treatment persistence/risk of stopping was analysed using Kaplan−Meier (KM) and Cox regression analysis. Proportions of patients reaching minimal disease activity (MDA)/very low disease activity (VLDA) and clinical Disease Activity Index for PsA (cDAPSA) low disease activity (LDA)/remission were analysed using logistic regression, including propensity score (PS) adjustment for imbalanced baseline covariates, and non-response imputation of effectiveness endpoints if treatment was stopped/switched before 1 year. Last observation carried forward data are reported.Results:Of patients starting UST and TNFi, 75/101 (74.3%) and 77/121 (63.6%), respectively, persisted with treatment at 1 year. The observed mean persistence was 410 days for UST and 363 days for TNFi. KM curves and PS-adjusted hazard ratios confirmed significantly higher persistence (hazard ratio [95% confidence interval (CI)]) for UST versus TNFi overall (0.46 [0.26; 0.82]; Figure 1). Persistence was also higher for UST than TNFi in patients receiving monotherapy without methotrexate (0.31 [0.15; 0.63]), in females (0.41 [0.20; 0.83]), and in patients with body mass index (BMI) <25 kg/m2 (0.34 [0.14; 0.87]) or >30 kg/m2 (0.19 [0.06; 0.54]). There was no significant difference in persistence between treatments in patients with BMI 25−30 kg/m2. While patients receiving 1st- and 3rd-line UST or TNFi showed similar risk of discontinuation (0.60 [0.27; 1.29] and 0.36 [0.10; 1.25], respectively), patients receiving 2nd-line UST showed better persistence than those receiving 2nd-line TNFi (0.33 [0.13; 0.87]). Other factors added to the PS-adjusted Cox model did not show significant effects. In patients with available follow-up data, the mean (standard deviation) baseline cDAPSA was 26.3 (15.4) for UST and 23.5 (12.3) for TNFi; at 1-year follow-up, 43.5% of UST- and 43.6% of TNFi-treated patients reached cDAPSA LDA/remission. MDA was reached in 24.2% of UST- and 28.0% of TNFi-treated patients, and VLDA in 12.5% of UST- and 10.2% of TNFi-treated patients. After PS adjustment (stoppers/switchers as non-responders), odds ratios (95% CI) at 1 year did not differ significantly between UST and TNFi groups for reaching cDAPSA LDA/remission (1.08 [0.54; 2.15]), MDA (0.96 [0.45; 2.05]) or VLDA (0.98 [0.35; 2.76]). In total, 23 (20.4%) patients reported ≥1 treatment emergent adverse event with UST and 30 (22.2%) with TNFi; 6 (5.3%) and 10 (7.4%) patients, respectively, discontinued treatment because of an adverse event.Conclusion:In the Italian PsABio cohort, UST had better overall persistence compared with TNFi, as well as in specific subgroups: females, patients on monotherapy without methotrexate, with BMI <25 or >30 kg/m2, and patients receiving UST as 2nd-line treatment. At 1 year, both treatments showed similar effectiveness, as measured by cDAPSA responses and MDA/VLDA achievement.Acknowledgements:This study was funded by Janssen. Contributing author: Prof. Piercarlo Sarzi-Puttini, ASST Fatebenefratelli-Sacco, University of Milan, ItalyDisclosure of Interests:Elisa Gremese: None declared, Francesco Ciccia Speakers bureau: AbbVie, Abiogen, Bristol-Myers Squibb, Celgene, Janssen, Lilly, Novartis, Pfizer, Roche, Consultant of: Celgene, Janssen, Lilly, Novartis, Pfizer, Roche, Grant/research support from: Celgene, Janssen, Novartis, Pfizer, Roche, Carlo Selmi Speakers bureau: AbbVie, Alfa-Wassermann, Amgen, Biogen, Celgene, Eli Lilly, Gilead, Janssen, MSD, Novartis, Pfizer, Sanofi-Genzyme, Consultant of: AbbVie, Alfa-Wassermann, Amgen, Biogen, Celgene, Eli Lilly, Gilead, Janssen, MSD, Novartis, Pfizer, Sanofi-Genzyme, Grant/research support from: AbbVie, Amgen, Janssen, Pfizer, Giovanna CUOMO: None declared, Rosario Foti Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Lilly, MSD, Janssen, Roche, Sanofi, Consultant of: AbbVie, Bristol-Myers Squibb, Celgene, Lilly, MSD, Janssen, Roche, Sanofi, Marco Matucci Cerinic Speakers bureau: Actelion, Biogen, Janssen, Lilly, Consultant of: Chemomab, Grant/research support from: MSD, Fabrizio Conti Consultant of: AbbVie, Bristol-Myers Squibb, Galapagos, Lilly, Pfizer, Enrico Fusaro Speakers bureau: AbbVie, Amgen, Lilly, Grant/research support from: AbbVie, Pfizer, Giuliana Guggino Speakers bureau: AbbVie, Celgene, Novartis, Pfizer, Sandoz, Grant/research support from: Celgene, Pfizer, Florenzo Iannone Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Lilly, MSD, Novartis, Pfizer, Sanofi, UCB, Consultant of: AbbVie, Bristol-Myers Squibb, Celgene, Lilly, MSD, Novartis, Pfizer, Sanofi, UCB, Andrea Delle Sedie: None declared, Roberto Perricone: None declared, Luca Idolazzi Speakers bureau: AbbVie, Eli Lilly, Janssen, MSD, Novartis, Sandoz, Paolo Moscato: None declared, Elke Theander Employee of: Janssen, Wim Noel Employee of: Janssen, Paul Bergmans Shareholder of: Johnson & Johnson, Employee of: Janssen, Silvia Marelli Employee of: Janssen, Laure Gossec Consultant of: AbbVie, Amgen, Bristol-Myers Squibb, Biogen, Celgene, Galapagos, Gilead, Janssen, Lilly, Novartis, Pfizer, Samsung Bioepis, Sanofi-Aventis, UCB, Grant/research support from: Amgen, Galapagos, Janssen, Lilly, Pfizer, Sandoz, Sanofi, Josef S. Smolen Speakers bureau: AbbVie, Amgen, AstraZeneca, Astro, Bristol-Myers Squibb, Celgene, Celltrion, Chugai, Gilead, ILTOO, Janssen, Lilly, MSD, Novartis- Sandoz, Pfizer, Roche, Samsung, Sanofi, UCB, Grant/research support from: AbbVie, AstraZeneca, Lilly, Novartis, Roche.

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Efficacy of long‐term adrenocorticotropic hormone therapy for West syndrome: A retrospective multicenter case series

Epilepsia Open ◽

10.1002/epi4.12497 ◽

2021 ◽

Author(s):

Shimpei Baba ◽

Tohru Okanishi ◽

Yoichiro Homma ◽

Takeshi Yoshida ◽

Tomohide Goto ◽

...

Keyword(s):

Hormone Therapy ◽

Adrenocorticotropic Hormone ◽

Case Series ◽

West Syndrome

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Computed Tomography and Bronchoscopy in Endobronchial Tuberculosis

Canadian Respiratory Journal ◽

10.1155/2003/496296 ◽

2003 ◽

Vol 10 (8) ◽

pp. 445-448 ◽

Cited By ~ 11

Author(s):

Halil Yanardag ◽

Cüneyt Tetikkurt ◽

Seza Tetikkurt ◽

Sabriye Demirci ◽

Tuncer Karayel

Keyword(s):

Computed Tomography ◽

Retrospective Study ◽

Therapeutic Response ◽

Histopathological Examination ◽

Computed Tomographic ◽

Before And After ◽

Endobronchial Tuberculosis ◽

Age Range

BACKGROUND: The therapeutic response to endobronchial tuberculosis is usually evaluated by bronchoscopy. Currently, there are no published studies investigating the use of computed tomography for the evaluation of therapeutic response in endobronchial tuberculosis.OBJECTIVE: A retrospective study was performed to evaluate the bronchoscopic and computed tomographic features of endobronchial tuberculosis before and after treatment. The aim of this study was to investigate the usefulness of computed tomography for the assessment of treatment.METHODS: The clinical, pathological and bronchoscopic features of endobronchial tuberculosis were evaluated in 55 patients. The age range of the patients was 21 to 52 years. Computed tomography and bronchoscopy were performed before and after treatment.RESULTS: Diagnosis of tuberculosis was confirmed by culture and histopathological examination. Bronchoscopic examination revealed 89 endobronchial lesions of various types in 55 patients. The exudative type was the most common. Follow-up bronchoscopy revealed that exudative-, ulcerative- and granular-type lesions healed completely. Computed tomography performed after treatment correlated well with the follow-up bronchoscopic findings.CONCLUSION: The results suggest that follow-up computed tomography is useful for the evaluation of therapeutic response and complications associated with endobronchial tuberculosis, and may replace bronchoscopy.

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Follow-up results of 702 patients receiving tumor necrosis factor-alpha antagonists and evaluation of risk of tuberculosis

Rheumatology International ◽

10.1007/s00296-009-1170-6 ◽

2009 ◽

Vol 30 (11) ◽

pp. 1459-1463 ◽

Cited By ~ 14

Author(s):

Tulin Cagatay ◽

Munevver Aydın ◽

Sule Sunmez ◽

Penbe Cagatay ◽

Ziya Gulbaran ◽

...

Keyword(s):

Tumor Necrosis Factor ◽

Tumor Necrosis Factor Alpha ◽

Tumor Necrosis ◽

Necrosis Factor Alpha ◽

Factor Alpha ◽

Necrosis Factor

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The SPOSIB SB2 Sicilian Cohort: Safety and Effectiveness of Infliximab Biosimilar SB2 in Inflammatory Bowel Diseases, Including Multiple Switches

Inflammatory Bowel Diseases ◽

10.1093/ibd/izaa036 ◽

2020 ◽

Cited By ~ 5

Author(s):

Fabio Salvatore Macaluso ◽

Walter Fries ◽

Anna Viola ◽

Andrea Centritto ◽

Maria Cappello ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Bowel Disease ◽

Tumor Necrosis ◽

Prospective Observational Study ◽

Cumulative Number ◽

Serious Adverse Events ◽

Bowel Diseases ◽

Inflammatory Bowel ◽

Necrosis Factor

Abstract Background No data on the recently introduced infliximab (IFX) biosimilar SB2 in inflammatory bowel disease (IBD) are available. Methods The Sicilian Prospective Observational Study of Patients With IBD Treated With Infliximab Biosimilar SB2 is a multicenter, observational, prospective study performed among the cohort of the Sicilian Network for Inflammatory Bowel Disease. All consecutive IBD patients starting the IFX biosimilar SB2 from its introduction in Sicily (March 2018) to September 2019 (18 months) were enrolled. Results Two hundred seventy-six patients (Crohn disease: 49.3%, ulcerative colitis: 50.7%) were included: 127 (46.0%) were naïve to IFX and naïve to anti-tumor necrosis factor medications (anti-TNFs), 65 (23.5%) were naïve to IFX and previously exposed to anti-TNFs, 17 (6.2%) were switched from an IFX originator to SB2, 43 (15.6%) were switched from the biosimilar CT-P13 to SB2, and 24 (8.7%) were multiply switched (from IFX originator to CT-P13 to SB2). The cumulative number of infusions of SB2 was 1798, and the total follow-up time was 182.7 patient-years. Sixty-seven serious adverse events occurred in 57 patients (20.7%; incidence rate: 36.7 per 100 patient-year), and 31 of these events caused the withdrawal of the drug. The effectiveness after 8 weeks of treatment was evaluated in patients naïve to IFX (n = 192): 110 patients (57.3%) had steroid-free remission, while 56 patients had no response (29.2%). At the end of follow-up, 72 patients (26.1%) interrupted the treatment, without significant differences in treatment persistency estimations between the five groups (log-rank P = 0.15). Conclusions The safety and effectiveness of SB2 seem to be overall similar to those reported for the IFX originator and CT-P13.

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Median 10 years follow-up of patients with covert Cushing’s syndrome: a case series

Journal of Medical Case Reports ◽

10.1186/s13256-021-03046-3 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Farzad Najafipour ◽

Amir Bahrami ◽

Mitra Niafar ◽

Jalil Houshyar ◽

Monireh Halimi ◽

...

Keyword(s):

Cushing’S Syndrome ◽

Adrenocorticotropic Hormone ◽

Hormone Secretion ◽

Cushing's Syndrome ◽

Whole Body ◽

Whole Body Scan ◽

Body Scan ◽

Posterior Aspect ◽

Ectopic Adrenocorticotropic Hormone

Abstract Background Ectopic adrenocorticotropic hormone secretion syndrome occurs in 10% of all patients with adrenocorticotropic-hormone-dependent hypercortisolism. It is usually associated with overt malignancies or with occult and indolent tumors. This study aims to confirm the source of ectopic adrenocorticotropic hormone in four patients with ectopic Cushing’s syndrome over time. Case presentation A 38-year-old Iranian man with Cushing’s syndrome underwent bilateral adrenalectomy since the source of ectopic adrenocorticotropic hormone secretion was not localized and pituitary imaging was normal. A whole-body scan revealed a right-lung tumoral mass with mediastinal lymph node metastasis. The mass was assumed a lung carcinoid tumor with mediastinal adenopathy. Right-lung mid-zone lobectomy and mediastinal lymphadenectomy were done. In a 47-year-old Iranian man with Cushing’s syndrome, whole-body computed tomography scan revealed a pulmonary nodule in the posterior segment of the left lower lobe of the lung. The third case was a 25-year-old Iranian man who presented with symptoms and signs of Cushing’s syndrome. Pituitary magnetic resonance imaging revealed a microadenoma 5 × 9 mm. Whole-body scan showed abnormal focal somatostatin receptors analog avid lesion in the posterior aspect of inferior third of right lung, highly suggestive of ectopic adrenocorticotropic-hormone-producing tumor. The last case was a 43-year-old Iranian woman with Marfan syndrome with a history of mitral and aortic valve replacement and chronic dissection of the aorta, who presented with symptoms and signs of Cushing’s syndrome. She underwent bilateral adrenalectomy 1 year later owing to failure to locate ectopic adrenocorticotropic hormone syndrome. Whole-body scan showed abnormally increased radiotracer uptake in the midline of the skull base and posterior aspect of the middle zone of left hemithorax and bed of left lobe of thyroid. Conclusion The clinical spectrum of ectopic adrenocorticotropic hormone secretion syndrome is wide, and distinguishing Cushing’s disease from ectopic adrenocorticotropic hormone secretion syndrome is difficult. Initial failure to identify a tumor is common. Pulmonary carcinoid or occult source of ectopic adrenocorticotropic hormone secretion syndrome is usually the cause. In occult cases of ectopic adrenocorticotropic hormone in which the tumor cannot be localized, serial follow-up with serial computed tomography, magnetic resonance imaging, or scintigraphy is recommended for several years until the tumor can be localized and treated.

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