PET/CT in Rare Autoimmune Diseases: Clinical Insights
Use of Pet/CT in different scenarios on rare and orphan diseases of autoimmune origin
REVIEW ARTICLE
Sanchez Orduz Liseth1, Acuña Hernandez Mayrlin2, Gerardo H. Cortés Germán3
- Nuclear Medicine Specialist, Nuclear Medicine Department, Hospital Universitario Mayor Méderi, Bogotá, Colombia
- Nuclear Medicine Specialist, Nuclear Medicine Department, Hospital de Cancerología INC, Bogotá, Colombia
- Nuclear Medicine Specialist, Tashadi SLS and Clínica del Country, Bogotá, Colombia
Email: [email protected]
OPEN ACCESS
PUBLISHED: 31 December 2024
CITATION: Sánchez Orduz, L., Acuña Hernandez, M., et al., 2024. Use of PET/CT in different scenarios on rare and orphan diseases of autoimmune origin. Medical Research Archives, (online) 12(12).
https://doi.org/10.18103/mra.v12i12.5994
COPYRIGHT: © 2024 European Society of Medicine. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
DOI https://doi.org/10.18103/mra.v12i12.5994
ISSN 2375-1924
ABSTRACT
Autoimmune diseases are on the rise, likely due to many factors such as pollution, infections, dietary changes, climate modifications, and exposure to xenobiotics. These diseases impact individuals, society, and healthcare systems. Some diseases are well-known, such as type 1 diabetes, while others are orphan or rare diseases.
According to the WHO, rare diseases affect fewer than five people per 10,000 inhabitants, and there are over 7,000 diseases. These conditions generally have low prevalence, are mostly chronically debilitating, and typically lack treatment.
In 2013, the European Society of Nuclear Medicine and the Society of Nuclear Medicine and Molecular Imaging published guidelines for using [¹⁸F]FDG (the main radiotracer) in PET/CT studies of inflammation and infection. The use of this radiotracer has expanded in recent decades.
Our objective was to describe the use of PET/CT and its various radiotracers in rare or orphan autoimmune diseases classified in ICD-11. To achieve this, we followed the methodology outlined by the Joanna Briggs Institute for scoping reviews.
Seventy-seven articles were included in the thematic analysis; 71 were case reports, while the rest were case series and cross-sectional studies. The predominant finding in the PET/CT scans of the different pathologies was an increase in the uptake of various radiotracers, with [¹⁸F]FDG being the most commonly used.
This diagnostic tool provided a comprehensive view of the systemic involvement of multiple conditions, such as Castleman disease and Cogan syndrome. It helped to identify the primary tumor and guide therapies for diseases like acromegaly.
Introduction
According to the World Health Organization (WHO)¹, orphan or rare diseases include around 5,500 diseases that can affect approximately 30 million people in the United States, according to the U.S. Food and Drug Administration (FDA)².
European Commission³, considering rare diseases have a low prevalence of 5 per 10,000 people, are potentially fatal, chronically debilitating, and have a genetic origin.
In the United States, the National Conference of State Legislatures⁴ defines them as diseases affecting fewer than 200,000 Americans, considering them neglected diseases. Their treatments are not profitable due to their cost.
This type of pathology presents a diagnosis, treatment, and follow-up challenge. The natural history of these diseases needs to be better known and studied. Their biology is complex, leading to difficulties in developing drugs, biological products, and devices to treat these conditions.
For this reason, in 1997, INSERM (French National Institute of Health and Medical Research), with subsequent support from the European Commission starting in 2002, created the Orphanet strategy⁵. This strategy includes multiple medical aspects of this type of pathology, including a comprehensive classification for the methodology explained later.
As for autoimmune diseases, the National Institute of Allergy and Infectious Diseases (NIAID)⁶ and the National Cancer Institute (NCI) define these pathologies as those in which antibodies are formed that attack the immune system.
The National Health and Nutrition Examination Survey (NHANES), a study program by the Centers for Disease Control and Prevention (CDC), found that approximately 32% of adults aged 60 or older may have at least four autoantibodies. Globally, an increase in the frequency of autoimmune diseases has been observed, with an estimated annual increase in incidence and prevalence of 19.1% and 12.5%, respectively⁸.
In recent years, there has been a rise in the use of Positron Emission Tomography/Computed Tomography (PET/CT), as it is a non-invasive imaging study used as a diagnostic method in various clinical scenarios: detection, classification, staging, prognosis, treatment planning, evaluation of response to therapy, and surveillance in oncological, cardiovascular, neurological, inflammatory, and infectious disorders, among others⁹.
We did not find a specific list of autoimmune and orphan diseases; therefore, we combined the lists to identify orphan autoimmune diseases.
For this reason, this scoping review aims to describe the different PET/CT tracers used in rare or orphan diseases of autoimmune origin, as defined in the ICD-11 classification.
Methodology
REVIEW QUESTION
What utility and characteristics are reported in the literature regarding using PET/CT with its different tracers in autoimmune orphan diseases?
The databases of available orphan diseases from Orphanet and autoimmune diseases from the Global Autoimmune Institute were cross-referenced, resulting in a list of orphan autoimmune diseases.
The study employed the broad population, concept, and context (PCC) framework indicated by the Joanna Briggs Institute for scoping reviews, as illustrated in Figure 1.
Figure 1. PCC framework in this study
ELIGIBILITY CRITERIA
Articles were deemed eligible for inclusion if they reported case reports, case series, descriptive, or analytical observational studies published without date limitation that included the orphan autoimmune diseases from the cross-referenced list created for this study in which a PET/CT study had been conducted.
During the literature review, the following diseases were excluded due to the quantity and quality of available information, which allows for the execution of systematic reviews: polymyositis, immune-mediated necrotizing myopathy, psoriatic arthritis, psoriasis, sarcoidosis, reactive arthritis, rheumatoid arthritis, Sjögren’s syndrome, systemic lupus erythematosus, acute disseminated encephalomyelitis, multiple sclerosis, myopathies, myositis, myasthenia gravis, connective tissue diseases, Guillain-Barré syndrome, IgG4-related disease, giant cell arteritis, antiphospholipid syndrome, granulomatosis with polyangiitis, autoimmune hepatitis, autoimmune pancreatitis, dermatomyositis, acute disseminated encephalomyelitis, and autoimmune diseases of the nervous system.
SEARCH STRATEGY
Initially, considering the methodology described by the Joanna Briggs Institute for scoping reviews, two researchers conducted a systematic search independently in indexed databases such as MEDLINE, OVID (including Embase), Cochrane Library Epistemonikos, Scielo, LILACS, and JBI Evidence Synthesis and gray literature such as OpenGrey and GreyNet using all the keywords included in the DECS, MESH, and Entry Terms. Annex 1 contains the search methodology.
SOURCE OF EVIDENCE SELECTION
All search results articles were uploaded to Software Mendeley, and duplicates were removed. Subsequently, two independent reviewers assessed the titles and abstracts, selecting the compositions according to the inclusion and exclusion criteria for the review. When there were disagreements between the reviewers, an additional reviewer was consulted, and agreement was reached.
This section’s results are presented as a flow diagram (Figure 2) following the Preferred Reporting Items for Systematic Reviews and Meta-analyses extension for scoping review (PRISMA-ScR) guidelines.
Figure 2. Flow diagram source of evidence
DATA EXTRACTION
For data extraction, two reviewers considered specific details of the articles, such as authors, year of publication, type of study, disease, PET/CT tracer, and main findings.
The items to be assessed by the two reviewers were not disagreeable, and complete information was found in the included articles to evaluate the results.
Results
The results obtained from the literature review are summarized by disease in the tables described below.
Table 1. Acromegaly
| Authors | Year of publication | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Bashari et al.¹⁰ | 2020 | Cross-sectional | L-[methyl¹¹C]-methionine or L-[carboxyl¹¹C]methionine | Focal tracer uptake in the lateral sellar and parasellar region |
Disease: Acromegaly
| Authors | Year | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Daniel et al.¹¹ | 2021 | Cross-sectional | [⁶⁸Ga]Ga-DOTA-TATE | A significant inverse relationship between postoperative values and the SUVmax at the sellar region |
| Daya et al.¹² | 2021 | Case report | [⁶⁸Ga]Ga-DOTA-TATE | Receptor activity in the pituitary gland due to physiological somatostatin receptor expression |
| Ahsan et al.¹³ | 2021 | Case report | [⁶⁸Ga]Ga-DOTA-TOC | Hyperplastic left adrenal gland with increased radiotracer uptake compared to the right |
| Alobaid et al.¹⁴ | 2023 | Case report | [⁶⁸Ga]Ga-DOTA-PEPTIDE | Intense focus on the uptake of the pituitary gland |
| Haberbosch et al.¹⁵ | 2023 | Case report | L-[methyl¹¹C]-methionine | Uptake on the left side of the sellar region |
| Chiloire et al.¹⁶ | 2024 | Case report | [¹⁸F]FDG | Excluded the presence of cancer |
| Bakker et al.¹⁷ | 2024 | Case series | [¹⁸F]FET | Suspicious parasellar tracer uptake |
Table 2. Aplastic Anemia, Aplastic Anemia and Systemic Lupus Erythematosus, and Bullous Pemphigoid
Disease: Aplastic Anemia
| Authors | Year | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Matsuki et al.¹⁸ | 2024 | Case report | [¹⁸F]FDG | Uptake of [¹⁸F]FDG pleura and lung |
Disease: Aplastic Anemia AND Systemic Lupus Erythematosus
| Authors | Year | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Dudek et al.¹⁹ | 2024 | Case report | [¹⁸F]FDG | Hypometabolic bone marrow activity |
Disease: Bullous Pemphigoid
| Authors | Year | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Shrestha et al.²⁰ | 2022 | Case report | [¹⁸F]FDG | Left mediastinal lymphadenopathy and lung lesion |
| Grünig et al.²¹ | 2022 | Case report | [¹⁸F]FDG | Multiple small lesions of the skin distant from known primary tumor locations |
Table 3. Chagas Disease
Disease: Chagas Disease
| Authors | Year | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Moll-Bernardes et al.²² | 2020 | Case report | [¹⁸F]FDG AND [⁶⁸Ga]Ga-DOTA-TOC | Increased radiotracer uptake in the mid inferoseptal, mid anteroseptal, and basal inferolateral walls of the left ventricle |
| de Oliveira et al.²³ | 2023 | Cross-sectional | [¹⁸F]FDG AND [⁶⁸Ga]Ga-DOTA-TOC | [¹⁸F]FDG AND [⁶⁸Ga]Ga-DOTA-TOC uptake useful for detection of myocardial inflammation; [⁶⁸Ga]Ga-DOTA-TOC uptake may be associated with malignant arrhythmia |
Table 4. Castleman’s Disease
Disease: Castleman’s disease
| Authors | Year | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Reddy et al.²⁴ | 2018 | Case series | [¹⁸F]FDG | Uptake in cervical, mediastinal, and pelvic lymph nodes; sclerotic bone lesions |
| Liu et al.²⁵ | 2023 | Case report | [¹⁸F]FDG | Uptake in mesenteric lymph node and multiple lung nodules with slight FDG uptake |
| Zhang et al.²⁶ | 2023 | Case report | [¹⁸F]FDG | Uptake in abdominal cavity |
| Maqbool et al.²⁷ | 2023 | Case report | [¹⁸F]FDG | Uptake right-sided neck mass and other lymph nodes of the head and neck |
| Yamauchi et al.²⁸ | 2023 | Case report | [¹⁸F]FDG | FDG uptake in idiopathic multicentric Castleman disease lower than in Hodgkin lymphoma |
| Zuo et al.²⁹ | 2024 | Case report | [⁶⁸Ga]Ga-DOTA-TATE / [⁶⁸Ga]Ga-Pentixafor | Positive uptake in retroperitoneal mass |
| Aher P et al.³⁰ | 2024 | Case report | [¹⁸F]FDG | Mixed-density mass with uptake in right cardiogenic region |
| Mashal et al.³¹ | 2024 | Case report | [¹⁸F]FDG | Uptake in supraclavicular, mediastinal, and retroperitoneal lymph nodes; diffuse uptake in spleen and soft-tissue nodules |
| Hu et al.³² | 2024 | Case report | [¹⁸F]FDG | Uptake in lymph nodes, spleen, bones, bone marrow, and nasopharynx associated with multicentric Castleman disease |
Table 5. Castleman’s Disease AND POEMS Syndrome & Cogan’s Syndrome
Disease: Castleman’s disease AND POEMS Syndrome
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Choe et al.³³ | 2024 | Case report | [¹⁸F]FDG | Multiple lymph nodes, L1 sclerotic lesion, edema, hepatosplenomegaly |
Disease: Cogan’s Syndrome
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Balink et al.³⁴ | 2007 | Case report | [¹⁸F]FDG | Uptake in aortic arch wall extending into lateral wall |
| Örsal et al.³⁵ | 2014 | Case report | [¹⁸F]FDG | Uptake in arterial walls and knees |
| Cabezas-Rodríguez et al.³⁶ | 2019 | Case report | [¹⁸F]FDG | Increased metabolic activity in thoracic aorta and subclavian arteries |
| Matsui et al.³⁷ | 2021 | Case report | [¹⁸F]FDG | Uptake in aorta, bilateral carotid, iliac arteries, and celiac artery |
| Hafner et al.³⁸ | 2021 | Case report | [¹⁸F]FDG | Multiple liver abscesses and abdominal aortitis |
| Na et al.³⁹ | 2024 | Case report | [¹⁸F]FDG | Uptake in subclavian arteries, common carotids, aortic arch, thoracic aorta, and coronary |
| Lu et al.⁴⁰ | 2024 | Case report | [¹⁸F]FDG | Uptake in right head/arm vessels, left carotid, and left subclavian artery |
Table 6. Cold Agglutinin Disease & Churg–Strauss Syndrome
Disease: Cold Agglutinin Disease
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Nakamoto et al.⁴¹ | 2019 | Case report | [¹⁸F]FDG | Splenomegaly with diffuse bone marrow uptake |
| Hayashi et al.⁴² | 2023 | Case report | [¹⁸F]FDG | Uptake in vertebral body, iliac bone, and spleen |
Disease: Churg–Strauss Syndrome
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Horiguchi et al.⁴³ | 2014 | Case report | [¹⁸F]FDG | Uptake in mediastinal and hilar lymphadenopathy |
Table 7. Eosinophilic Fasciitis
Disease: Eosinophilic fasciitis
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Narváez et al.⁴⁴ | 2019 | Case report | [¹⁸F]FDG | Diffuse symmetrical uptake in fascia of legs and thighs |
| Barlet et al.⁴⁵ | 2020 | Case report | [¹⁸F]FDG | Uptake in shoulders, wrists, knees, and ankles |
| Song et al.⁴⁶ | 2021 | Case report | [¹⁸F]FDG | Uptake in subcutaneous fat and muscle |
| Chalopin et al.⁴⁷ | 2021 | Case report | [¹⁸F]FDG | Uptake in bone lesions |
| Barlet et al.⁴⁵ | 2021 | Case report | [¹⁸F]FDG | Diffuse uptake of muscular fasciae |
| Laria et al.⁴⁸ | 2022 | Case report | [¹⁸F]FDG | Diffuse uptake in forearm muscles and both lower limbs |
| Amrane et al.⁴⁹ | 2022 | Case report | [¹⁸F]FDG | Uptake in subcutaneous nodules, fascia, and synovial walls of knees |
| Benzaquen et al.⁵⁰ | 2023 | Case report | [¹⁸F]FDG | Generalized hypermetabolism of fasciae and adjacent tissues |
| Fevrier et al.⁵¹ | 2024 | Case report | [¹⁸F]FDG | Uptake of fasciae in upper and lower limbs |
Table 8. Henoch–Schönlein Purpura & Immune Thrombocytopenic Purpura
Disease: Henoch–Schönlein Purpura
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Sabzevari et al.⁵² | 2018 | Case report | [¹⁸F]FDG | Uptake in subclavian, brachiocephalic, abdominal aortic, iliac, and femoral arteries |
| Gultekin et al.⁵³ | 2021 | Case report | [¹⁸F]FDG | Uptake in cavitary nodular lesions and hilar lymphadenomegaly |
Disease: Immune Thrombocytopenic Purpura
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Razanamahery et al.⁵⁴ | 2021 | Case report | [¹⁸F]FDG | Uptake in perinephric fat fibrosis, mediastinal lymph nodes, and low uptake in testis |
| Ren et al.⁵⁵ | 2023 | Case report | [¹⁸F]FDG | Uptake in lymph nodes in numerous regions |
Table 9. Neuromyelitis Optica Spectrum Disorder
Disease: Neuromyelitis Optica Spectrum Disorder
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Alkhaja et al.⁵⁶ | 2021 | Case report | [¹⁸F]FDG | Uptake along entire spinal cord suggesting acute myelitis |
| Ding et al.⁵⁷ | 2021 | Case report | [¹⁸F]FDG | Uptake in cervicothoracic region and rectal wall |
| Fujisawa et al.⁵⁸ | 2023 | Case report | [¹⁸F]Flutemetamol, [¹⁸F]MK6240 (TAU), [¹⁸F]FDG | Brain uptake patterns (frontal, parietal, temporal, cingulate); decreased glucose metabolism in specific cortical regions |
| Vlaicu et al.⁵⁹ | 2023 | Case report | [¹⁸F]FDG | Uptake in pulmonary neoplasm with lymph node and adrenal metastases |
Table 10. Paraneoplastic Pemphigus
Disease: Paraneoplastic pemphigus
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Dhull et al.⁶⁰ | 2016 | Case report | [¹⁸F]FDG | Uptake in left paravertebral region, renal hilum, oral cavity, and lung |
| Lim et al.⁶¹ | 2017 | Case report | [¹⁸F]FDG | Uptake in multiple enlarged lymph nodes |
| Khurana et al.⁶² | 2020 | Case report | [¹⁸F]FDG | Uptake in mediastinal lesion extending to pericardial sinus |
| Chen et al.⁶³ | 2020 | Case report | [¹⁸F]FDG | Uptake in mediastinum, parasternal adenopathy, pleural effusion |
| Liska et al.⁶⁴ | 2022 | Case report | [¹⁸F]FDG | Uptake in left tonsil |
| Daniels et al.⁶⁵ | 2023 | Case report | [¹⁸F]FDG | Uptake in mediastinum |
| Lu et al.⁶⁶ | 2024 | Case report | [¹⁸F]FDG | Uptake in neck lymphadenopathies |
Table 11. Paraneoplastic Pemphigus AND Castleman’s Disease & Paraneoplastic Cerebellar Degeneration
Disease: Paraneoplastic pemphigus AND Castleman’s disease
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Fu et al.⁶⁷ | 2018 | Case report | [¹⁸F]FDG | Uptake in oral lesions and heterogeneous soft tissue mass in retroperitoneum |
| Liu et al.⁶⁸ | 2011 | Case series | [¹⁸F]FDG | Uptake in head of pancreas |
| Fu et al.⁶⁹ | 2018 | Case report | [¹⁸F]FDG | Uptake in retroperitoneum |
| Wang et al.⁷⁰ | 2019 | Case report | [¹⁸F]FDG | Uptake in head of pancreas |
| Relvas et al.⁷¹ | 2023 | Case report | [¹⁸F]FDG | Uptake in retroperitoneal lymphadenopathies and lobulated mass |
Disease: Paraneoplastic Cerebellar Degeneration
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Rodríguez Herrera et al.⁷² | 2023 | Case report | [¹⁸F]FDG | Uptake in orbitofrontal hypermetabolism, mesial temporal, and bilateral regions |
| Authors | Year | Type of Study | PET/CT Tracer | Main Findings |
|---|---|---|---|---|
| Takahashi et al.⁷³ | 2024 | Case report | [¹⁸F]FDG / [¹²³I]IMP SPECT | [¹⁸F]FDG uptake in lung tumor and mediastinal lymph nodes; [¹²³I]IMP SPECT shows normal blood flow in the cerebellum |
| Kalantari et al.⁷⁴ | 2024 | Case report | [¹⁸F]FDG | Uptake in the annex |
| Imai et al.⁷⁵ | 2022 | Case report | [¹⁸F]FDG | Uptake in the left neck |
Table 12. POEMS Syndrome
Disease: POEMS Syndrome
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Pan et al.⁷⁶ | 2015 | Cross-sectional | [¹⁸F]FDG | Uptake in solitary and multiple hypermetabolic bone lesions, lymph nodes, hepatomegaly, splenomegaly, CNS, serous cavity effusion, and gynecomastia |
| Allam et al.⁷⁷ | 2022 | Case report | [¹⁸F]FDG | Uptake in axillary and retropectoral lymph nodes and systemic fibrosis involving pleura, mediastinum, and pelvis |
| Genicon et al.⁷⁸ | 2022 | Case report | [¹⁸F]FDG | Uptake in osteolytic lesion in right femur |
| Gültekin et al.⁷⁹ | 2023 | Case report | [¹⁸F]FDG | Diffuse muscle uptake |
| Aderhold et al.⁸⁰ | 2024 | Case report | [¹⁸F]FDG | Uptake in osteosclerotic pelvic, vertebral, and clavicular bone lesions and hilar lymphadenopathy |
Table 13. Polyarteritis Nodosa & Postural Orthostatic Tachycardia Syndrome
Disease: Polyarteritis nodosa
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Kang et al.⁸¹ | 2023 | Case report | [¹⁸F]FDG | Uptake in lower extremities |
| Taimen et al.⁸² | 2024 | Case report | [¹⁸F]FDG | Uptake in peri- and intramuscular arterial structures of lower extremities and liver |
| Makiyama et al.⁸³ | 2024 | Case report | [¹⁸F]FDG | Uptake in nodules (right lower lung), pulmonary artery embolism, and subcutaneous tissue |
| Philip et al.⁸⁴ | 2024 | Case report | [¹⁸F]FDG | Uptake in soft tissues and intramuscular arterial tree |
| Taniguchi et al.⁸⁵ | 2024 | Case report | [¹⁸F]FDG | Uptake in medium-sized vessels |
Disease: Postural Orthostatic Tachycardia Syndrome (POTS)
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Khan et al.⁸⁶ | 2022 | Case report | [⁶⁸Ga]Ga-DOTA-TATE | Uptake associated with contrast in both adrenal glands and calcified thyroid nodules |
Disease: Scleroderma
| Authors | Year | Type | PET/CT | Findings |
|---|---|---|---|---|
| Diaz Menindez et al.⁸⁷ | 2023 | Case report | [¹⁸F]FDG | Uptake in multifocal osseous regions, especially spine and pelvis |
Discussion
Autoimmune diseases are characterized by spontaneous hyperactivity of the immune system, leading to the production of additional antibodies, which often results in inflammation that can affect all organs and tissues of the body, especially lymphoid tissues, joints, skin, muscles, salivary glands, blood vessels, and bone marrow⁸⁸.
As established in the results section, multiple autoimmune diseases are considered within the spectrum of orphan diseases. Therefore, from the perspective of molecular diagnostic studies, this review aimed to compile the available findings in the literature so that readers can become familiar with these types of pathologies and find a diagnostic aid for these conditions in these studies.
As for the pathophysiology, inflammation is the host’s initial defense against pathogens and other triggering stimuli. It plays an essential role in tissue repair and eliminating harmful pathogens. However, an inadequate response can damage normal cells adjacent to affected tissue. In many autoimmune diseases, sterile inflammation occurs.
Molecular imaging allows visualization, characterization, and measurement of biological processes at molecular and cellular levels, with PET/CT being the most widely used modality in clinical practice⁸⁹.
The European Association of Nuclear Medicine (EANM), in conjunction with the Society of Nuclear Medicine and Molecular Imaging (SNMMI), published guidelines in 2013 on the use of [¹⁸F]FDG in inflammation and infection. In 2018, along with the PET Interest Group (GIP) and endorsed by the American Society of Nuclear Cardiology, guidelines were published for PET/CT in diagnosing and following up patients with suspected large vessel vasculitis and polymyalgia rheumatica. Over the last 10 years, PET/CT use has rapidly evolved and is now considered the most utilized imaging study in nuclear medicine for diagnosing and treating inflammatory disorders⁹⁰.
[¹⁸F]FDG is the most commonly used PET tracer; as a glucose analog, it is taken up by cells with high energy demand, where it accumulates and allows detection via PET imaging. Inflammatory processes exhibit increased FDG uptake due to glucose transporter expression (GLUT1, GLUT3) and increased metabolic activity in inflammatory cells.
This is supported by the review findings, where increased FDG uptake was the predominant observation across diseases. SUVmax values greater than 4 were reported in Castleman disease, Wegener’s granulomatosis, POEMS syndrome, and eosinophilic fasciitis in some cases.
PET/CT also helped guide diagnosis by identifying primary tumors or metastatic lesions in conditions such as paraneoplastic pemphigus.
Cogan syndrome, a rare disease of unknown origin, often presents with systemic vasculitis. PET/CT facilitated diagnosis by revealing increased metabolic activity in major vessels like the aorta and subclavian arteries.
Some cases showed diagnostic challenges—for example, distinguishing idiopathic multicentric Castleman disease from Hodgkin lymphoma, where FDG uptake patterns differ significantly.
Patients with chronic autoimmune/inflammatory diseases have a higher risk of malignancies. For example:
- 2–4× risk for esophageal and pancreatic cancer
- ~2× risk of lymphoma in rheumatoid arthritis
- 3–6× in systemic lupus erythematosus
- 9–18× in Sjögren syndrome
- 7× in dermatomyositis/polymyositis
PET/CT is useful in differentiating inflammation from malignancy using metrics like spleen/liver SUVmax ratio.
In diseases like acromegaly, Chagas disease, and Castleman disease, tracers such as ⁶⁸Ga-DOTATATE/DOTATOC show increased uptake due to somatostatin receptor overexpression.
Other tracers include amino acid–based tracers like ¹¹C-methionine and ¹⁸F-FET, useful for detecting residual or central nervous system lesions.
In neuromyelitis optica, tracers like ¹⁸F-flutemetamol and ¹⁸F-MK6240 (TAU) detect amyloid and tau deposition.
[⁶⁸Ga]-Pentixafor may indicate CXCR4 expression in diseases like Castleman disease.
Overall, PET/CT provides a comprehensive view of systemic inflammation and helps guide diagnosis, treatment, and follow-up in autoimmune orphan diseases.
Conflict of Interest
The authors have no conflicts of interest to declare.
Funding Statement
None.
Acknowledgements
None.
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