You have 2 free disease views remaining today. Register for more view, it is 100% free →
Riedel's fibrosing thyroiditis
ICD-10 Codes
Related ICD-10:
Description
Riedel's Fibrosing Thyroiditis: A Rare and Chronic Condition
Riedel's fibrosing thyroiditis is a rare and chronic inflammatory disease that affects the thyroid gland, causing compression and fibrosis of adjacent tissues. This condition is characterized by the replacement of normal thyroid parenchyma with dense fibrotic tissue, leading to progressive destruction of the thyroid gland.
Key Features:
- Rare Disease: Riedel's fibrosing thyroiditis is a rare disease, with an estimated incidence of 1.06 cases per 100,000 population.
- Chronic Inflammation: The condition is characterized by chronic inflammation and fibrosis of the thyroid gland.
- Fibrotic Tissue: Normal thyroid parenchyma is replaced by dense fibrotic tissue, leading to compression and destruction of adjacent tissues.
- Hypothyroidism: Classically, Riedel's fibrosing thyroiditis presents as hypothyroidism with a "stone-like, hard-as-wood" non-tender thyroid gland.
Symptoms:
- Hoarseness
- Dyspnea (shortness of breath)
- Dysphagia (difficulty swallowing)
Differential Diagnosis:
- Fibrosing variant of Hashimoto thyroiditis
Treatment and Prognosis:
- Treatment options are limited, and the prognosis is generally poor.
- Surgery may be considered in some cases to relieve compression symptoms.
References:
- [1] Riedel's fibrosing thyroiditis is a rare disease characterized by chronic inflammation and fibrosis of the thyroid gland. (Source: #10)
- The condition is often mistaken for other thyroid diseases due to its low incidence. (Source: #13)
- Riedel's thyroiditis has an estimated incidence of 1.06 cases per 100,000 population. (Source: #15)
Signs and Symptoms
Common Signs and Symptoms of Riedel's Fibrosing Thyroiditis
Riedel's thyroiditis is a rare condition characterized by chronic inflammation and fibrosis of the thyroid gland. The symptoms can vary from person to person, but here are some common signs and symptoms associated with this condition:
- Goiter: A non-painful, rapidly growing thyroid mass that can be hard and fixed in place [6].
- Neck pressure: Patients may experience sensations of suffocation, stridor, or painless neck pressure out of proportion to the size of the goiter [8].
- Dysphagia: Difficulty swallowing due to compression of the esophagus by the enlarged thyroid gland [2].
- Hoarseness: Vocal cord paralysis can occur due to compression of the recurrent laryngeal nerve [7].
- Pain: Some patients may experience pain in the neck or throat, although this is not a common symptom [3].
- Hypothyroidism: As the thyroid gland becomes scarred and inflamed, it can lead to hypothyroidism, with symptoms like cold intolerance, lack of energy, constipation, or dry skin [9].
It's essential to note that Riedel's thyroiditis is a rare condition, and not all patients will experience all of these symptoms. If you suspect you have this condition, consult a healthcare professional for proper diagnosis and treatment.
References: [1] - Not relevant [2] - Context 3 [3] - Context 7 [6] - Context 6 [7] - Context 7 [8] - Context 8 [9] - Context 9
Additional Symptoms
- Goiter
- Hypothyroidism
- Neck pressure
- dysphagia
- hoarseness
- pain
Diagnostic Tests
Diagnostic Challenges of Riedel's Fibrosing Thyroiditis
Riedel's fibrosing thyroiditis, a rare inflammatory disease, poses significant diagnostic challenges due to its rarity and non-specific symptoms. While various tests can suggest the diagnosis, only an open biopsy can confirm it.
- Fine-needle biopsy: Fine-needle aspiration (FNA) has limited value in diagnosing Riedel's thyroiditis, as it may not provide sufficient tissue for histopathological examination [2, 8].
- Open biopsy: An open biopsy is the gold standard for confirming the diagnosis of Riedel's thyroiditis. Histology from the biopsy shows thyroid parenchyma with fibrosis and chronic inflammation [1, 2, 8].
- Thyroid function tests: Thyroid function tests may reveal hypothyroidism or normal thyroid function in patients with Riedel's thyroiditis [10, 12, 13]. However, these tests are not specific for the disease.
- Radioactive iodine uptake test: A diffusely decreased radioactive iodine uptake can be observed in patients with Riedel's thyroiditis [6].
- Ultrasonography: Ultrasonography may display a hypoechoic (dense) appearance of the thyroid gland, but this finding is not specific for Riedel's thyroiditis [9].
Diagnostic Dilemmas
The diagnosis of Riedel's fibrosing thyroiditis can be challenging due to its rarity and non-specific symptoms. A combination of clinical presentation, laboratory tests, and imaging studies may suggest the diagnosis, but only an open biopsy can confirm it.
- Clinical presentation: Patients with Riedel's thyroiditis often present with hypothyroidism, a "stone-like" hard thyroid gland, and compression of adjacent structures [13].
- Laboratory tests: Laboratory tests may reveal elevated levels of inflammatory markers, such as C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR), but these findings are not specific for Riedel's thyroiditis.
Conclusion
In conclusion, the diagnosis of Riedel's fibrosing thyroiditis requires a combination of clinical presentation, laboratory tests, and imaging studies. However, only an open biopsy can confirm the diagnosis. A high index of suspicion is necessary to diagnose this rare disease, and a multidisciplinary approach may be required to manage patients with Riedel's thyroiditis.
References:
[1] Moloo Z., Vinayak S. (2022). Riedel's thyroiditis as a diagnostic dilemma - A case report and review of the literature.
[2] Shafi AA, Saad NB, AlHarthi B. (2020). Riedel's thyroiditis as a diagnostic dilemma - A case report and review of the literature.
[3] Juraniec O, Pypkowski J, Krymer A, Marszałek A. (2019). A difficult diagnosis: a case report of combined Riedel's disease and fibrosing Hashimoto's thyroiditis.
[4] Isotope tests, such as thyroid scintigraphy, may be used to evaluate thyroid function and structure in patients with Riedel's thyroiditis [14].
Note: The references provided are based on the search results within the context.
Treatment
Treatment Options for Riedel's Fibrosing Thyroiditis
Riedel's fibrosing thyroiditis, a rare inflammatory disease of the thyroid gland, can be challenging to treat. While there is no cure, various drug treatments have been employed to manage symptoms and improve quality of life.
- Corticosteroids: Currently, corticosteroid therapy is the medical treatment of choice for patients with Riedel thyroiditis (RT). Corticosteroids are believed to act by reducing inflammation and inhibiting the actions of fibrinogenic cytokines [11]. A meta-analysis found that corticosteroids were used in 70% of patients with RT, with a median duration of treatment being 3 months [11].
- Rituximab: Rituximab, a monoclonal antibody against the CD20 protein found mainly on B lymphocytes, has gained popularity as a treatment for refractory Riedel's thyroiditis. It is considered after the failure of conventional therapies and has been successful in several case reports [13][17][18][19].
- Tamoxifen: Tamoxifen has been used to treat progressive Riedel thyroiditis (RT) that is not responsive to corticosteroids or surgical decompression, or is contraindicated [4]. However, its effectiveness and safety in this context are still being studied.
- Immunosuppressant medications: Other immunosuppressant medications, such as azathioprine or mycophenolate mofetil, have also been used to treat Riedel's thyroiditis. However, their efficacy and potential side effects require further investigation.
Important Considerations
It is essential to note that the effectiveness of these treatments can vary depending on individual circumstances, and more research is needed to fully understand the best treatment approaches for Riedel's fibrosing thyroiditis. Patients should consult with their healthcare provider to determine the most suitable course of treatment.
References:
[4] Aug 22, 2022 — Tamoxifen use in progressive Riedel thyroiditis. [11] Currently, corticosteroid therapy is the medical treatment of choice for patients with Riedel thyroiditis (RT). [13] A drug gaining popularity in the treatment of refractory Riedel's thyroiditis is Rituximab.
Differential Diagnosis
Riedel's fibrosing thyroiditis, also known as Riedel struma or chronic invasive fibrous thyroiditis, is a rare disease characterized by chronic inflammation and fibrosis of the thyroid gland. When it comes to differential diagnosis, several conditions need to be considered.
- Fibrosing variant of Hashimoto's thyroiditis: This condition shares similarities with Riedel's fibrosing thyroiditis in terms of its fibrotic nature and involvement of the thyroid gland. However, the presence of lymphocytic infiltration and the absence of significant extrathyroidal extension are key distinguishing features [10][11].
- Undifferentiated carcinoma: The differential diagnosis between Riedel's thyroiditis and undifferentiated carcinoma is crucial due to their distinct outcomes. While Riedel's thyroiditis is a benign condition, undifferentiated carcinoma is a malignant tumor that requires prompt treatment [2][3].
- Fibrosing Hashimoto's thyroiditis: This condition can be distinguished from Riedel's fibrosing thyroiditis by the presence of lymphocytic infiltration and the absence of significant extrathyroidal extension. Additionally, fibrosing Hashimoto's thyroiditis is often associated with autoimmune thyroiditis [13].
- IgG4-related thyroid disease: More recently, IgG4-related thyroid disease has been recognized as a distinct entity that includes Riedel's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, and other conditions. This diagnosis requires the presence of IgG4-positive plasma cells and significant fibrosis [12].
It is essential to note that the differential diagnosis of Riedel's fibrosing thyroiditis can be challenging due to its rarity and overlapping features with other thyroid conditions. A definitive diagnosis often requires an open biopsy of the thyroid gland, as mentioned in [9] and [14].
Additional Information
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:14351
- core#notation
- DOID:14351
- oboInOwl#hasDbXref
- UMLS_CUI:C0154162
- oboInOwl#hasExactSynonym
- Riedel fibrosing thyroiditis
- rdf-schema#label
- Riedel's fibrosing thyroiditis
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#NCIthesaurus
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_7166
- relatedICD
- http://example.org/icd10/E06.5
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_8911
Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.
It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.