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Relapsing polychondritis diagnosed by fusion images of gallium-67 on single photon emission computed tomography/computed tomography
Arthritis & Rheumatology
Abstract
A 64-year-old man has revealed dry cough and severe pain of ear, nose and throat for several weeks. After visiting clinics and hospitals, he was introduced to our hospital and was diagnosed as relapsing polychondritis (RP) based on McAdam's criteria. The involvement in auricular, nasal and tracheal cartilage was clearly shown by fusion images of gallium-67 on single photon emission computed tomography and computed tomography (SPECT/CT) (A, B). High-dose glucocorticoid (GC) therapy markedly improved relevant signs, symptoms and inflammatory reactions as well as gallium uptake in cartilage detected by gallium-SPECT/CT
... It had been previously documented that 18 F FDG PET/CT neither demonstrated sensitivity nor specificity in evaluating therapeutic efficacy [11]. A past study also highlighted 67 Ga visualization as a characteristic feature of RP, capable of guiding biopsy site selection [15]. Regrettably, 67 Ga imaging remains underutilized in China, thereby limiting its application. ...
Background
Relapsing Polychondritis(RP) is a rare rheumatic immune disease. As with most diseases, if intervention is delayed, the patient’s prognosis is worse. Currently, the diagnostic criteria used in clinical practice do not include CT, PET/CT, SPECT/CT and other new imaging examinations that have developed rapidly in recent years. However, these examinations have some special manifestations for RP, which can help clinicians diagnose RP earlier and distinguish it from other diseases.
Case presentation
These five RP patients all had respiratory symptoms such as cough and wheezing as the first symptom, which could not be diagnosed in time according to the previous diagnostic criteria. The clinical data of the five patients are listed in Table 1. The relatively specific manifestations of SPECT/CT examination provided clinicians with very valuable clues to help them advance the diagnosis time.
Conclusions
The application of SPECT/CT bone imaging in early diagnosing RP proves to be effective, enabling clinicians to intervene promptly and enhance the overall well-being and quality of life for individuals affected by this condition.
... Technetium 99m ( 99m Tc) methylene diphosphonate bone scintigraphy can be used for evaluation of costal cartilage involvement, but it is not helpful for assessing other organ involvement [11]. Gallium-67 citrate scintigraphy with SPECT/CT has poor spatial resolution and specificity [12]. Fluorodeoxyglucose (FDG) accumulates in inflammatory conditions and is used in various inflammatory rheumatological diseases such as Takayasu's arteritis [13,14]. ...
Objective:
To evaluate 18F-fluorodeoxyglucose (FDG) PET/CT in the assessment of disease activity, extent of the disease and response to therapy in relapsing polychondritis.
Methods:
Twenty-five patients (9 men, 16 women) with a mean age of 38.2 years (s.d. 13.7; range 18-62), diagnosed to have relapsing polychondritis according to Damiani and Levine's modification of McAdam's criteria, who underwent PET/CT examination were included. Ten patients underwent a second PET/CT examination after therapy or during follow-up. Clinical symptoms and auxiliary examination findings were recorded. PET/CT findings were reviewed and correlated with the clinical symptoms.
Results:
The major symptoms were aural pain (n = 21), nasal pain (n = 10), stridor (n = 5), cough (n = 9), fever (n = 8) and laryngeal tenderness (n = 8). The initial PET/CT was positive in 23/25 patients. PET/CT revealed involvement of auricular (n = 14), nasal (n = 8), laryngeal (n = 7), tracheobronchial (n = 6) and Eustachian (n = 3) cartilages with a mean maximum standardized uptake value (SUVmax) of 4.1 (s.d. 2.5; range 1.7-12.7). Fair correlation of aural/nasal pain/stridor with FDG avidity of cartilage involvement on PET/CT was noted. The key finding was detection of asymptomatic large airway involvement in seven patients (28%). Re-examination PET in 10 patients revealed complete therapeutic response (n = 5), partial response (n = 1), stable disease (n = 1), progressive disease (n = 1) and disease recurrence (n = 2).
Conclusion:
FDG PET/CT is a useful tool for the assessment of the disease activity and extent. It identified activity in clinically inaccessible sites that are of clinical significance. It is also useful in assessing treatment response and finding relapse.
... Recently, the Value of 67 Ga SPECT/CT to diagnose RP was reported. More readily available and lower cost makes 67 Ga SPECT/CT have wide application prospects to diagnose RP. 3 ...
... При проведении дифференциальной диагностики рецидивирующего полихондрита применяют такие методы, как компьютерная томография (КТ) [20], магнитно-резонансная томография (МРТ), сцинтиграфия с технецием-99 или галлием-67, допплерэхокардиография, которые позволяют обнаружить аномалии бронхиального дерева и аорты [4]. ...
... 18 Also not fully validated, several reports suggest that 18F-fluorodeoxyglucose Positron Emission Tomography (18F-FDG PET/CT) could be interesting for therapeutic response monitoring as the uptake may diminish or disappear after therapy. 37 For articular involvement, Tc-99m bone scintigraphy may reveal active sites of inflammation. 38 Cerebral MRI may be useful if central nervous system involvement is suspected. ...
Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). The systematic literature review identified 20 citations, among which no CPGs could be identified. We identified 11 main areas with unmet needs in the field of RP: the diagnosis strategy for RP; the therapeutic management of RP; the management of pregnancy in RP; the management of the disease in specific age groups (for instance in paediatric-onset RP); the evaluation of adherence to treatment; the follow-up of patients with RP, including the frequency of screening for the potential complications and the optimal imaging tools for each involved region; perioperative and anaesthetic management (due to tracheal involvement); risk of neoplasms in RP, including haematological malignancies; the prevention and management of infections; tools for assessment of disease activity and damage; and patient-reported outcomes and quality of life indicators. Patients and physicians should work together within the frame of the ReCONNET network to derive valuable evidence for obtaining literature-informed CPGs.
... 45,46 Imaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI), scintigraphy (with technetium-99m methylene diphosphonate or gallium-67 citrate), and Doppler echocardiography reveal anomalies of the laryngo-tracheo-bronchial tree and aorta. [1][2][3][4][5]47 Numerous studies have shown the value of fluorine-18 fludeoxyglucose positron emission tomography as a new diagnosis modality that may be helpful in early disease recognition, early diagnosis of relapsing polychondritis, as well as detection of subclinical chondritis, evaluation of disease activity, disease extension, site for targeted biopsy, and therapeutic response monitoring. [48][49][50][51][52] Then, the presence of symmetrically distributed high fluorine-18 fludeoxyglucose uptake lesions at two or more cartilages may be a new criterion for the diagnosis. ...
Relapsing polychondritis is a severe systemic immune-mediated disease characterized by an episodic and progressive inflammatory condition with progressive destruction of cartilaginous structures. This disease has for nearly a century kept secrets not yet explained. The real incidence and prevalence of this rare disease are unknown. The multiple clinical presentations and episodic nature of relapsing polychondritis cause a significant diagnosis delay. No guidelines for the management of patients with relapsing polychondritis have been validated to date. The challenges remain, both in the understanding of its pathophysiology and diagnosis, evaluation of its activity and prognosis, and its treatment. Possible solutions involve the sharing of data for relapsing polychondritis from worldwide reference centers. Thus, we would be able to evolve toward a better knowledge of its pathophysiology, the publication of new diagnosis criteria, which will include biological markers and imaging findings, the prediction of life-threatening or organ-threatening situations, and the publication of therapeutic evidence-based guidelines after performing at randomized controlled trials.
The purpose of this study is to evaluate the clinical course and effectiveness of pharmacological therapy in a patient with recurrent polychondritis. Material and methods. Retrospective and prospective analyses of the diagnosis, course and treatment of recurrent polychondritis in the patient were performed. The patient was diagnosed based on the McAdam classification criteria. The patient was assigned laboratory tests: general, biochemical and immunological blood analyses; instrumental methods: X-ray and ultrasound examinations of the vessels, spirometry. The results of the study. The patient was first diagnosed with recurrent polychondritis at the age of 58. The patient complained of pain, swelling and hyperemia of the auricles, a change in the shape of the nose; attacks of suffocation, which were stopped by inhalations of salbutamol, hoarseness of voice; prolonged subfebrility (37.5–38°C), arthralgia. In September 2022, he was examined by an otolaryngologist, then sent to a rheumatologist to determine therapeutic and diagnostic tactics. According to the results of laboratory data: erythrocyte sedimentation rate – 48 mm/hour, C-reactive protein – 30.1 mg/l, rheumatoid factor – 10 units/l, antibodies to cyclic citrulline peptide- 7.0 units, the result of immunoblot is negative; antibodies to myeloperoxidase – 4.15 RU/ml, antibodies to proteinase – 32.2 RU/ml. During spirometry, there was a violation of the function of external respiration of an obstructive type. Ultrasound of the joints: signs of synovitis of the proximal interphalangeal joints of both hands. Based on the McAdam classification criteria, a clinical diagnosis was made: recurrent polychondritis with systemic manifestations: chondritis of the auricles and nasal congestion, seronegative arthritis, bronchoobstructive syndrome, constitutional symptom (fever). The patient was prescribed therapy: prednisone 20 mg/day, methotrexate 25 mg/week. Clinical and laboratory remission was achieved, but subsequently relapses of the disease and the appearance of a herpes infection were observed (antiviral therapy was prescribed). In July 2023, he was sent for examination and treatment at the Nasonova Research Institute of Rheumatology. The patient was submitted to the commission on therapy with genetically engineered biological drugs. Taking into account the often recurrent herpetic infection, the appointment of genetically engineered biological drugs was postponed. It is recommended to continue taking prednisolone at a dose of 20 mg/day, antiviral therapy (valciclovir). Dapsone 100 mg per day was added to the treatment, but his patient independently canceled the drug due to the appearance of severe nausea and insomnia. Subsequently, a rheumatologist with an immunosuppressive purpose prescribed azathiroprine 150 mg/day, continued taking prednisone 20 mg/day, however, exacerbations of the disease were registered. The lack of effect of the therapy was the basis for the patient's re-hospitalization at the V.A. Nasonova Research Institute of Rheumatology. Conclusions. Recurrent polychondritis is a rapidly progressive disease, and in the absence of timely diagnosis and treatment, it has a risk of developing life-threatening conditions.
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