ICD-10: M31.6

Other giant cell arteritis

Additional Information

Description

ICD-10 code M31.6 refers to "Other giant cell arteritis," a specific classification within the broader category of giant cell arteritis (GCA). This condition is characterized by inflammation of the blood vessels, particularly the large and medium-sized arteries, and is most commonly associated with the temporal arteries. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Other Giant Cell Arteritis (M31.6)

Definition and Overview

Giant cell arteritis, also known as temporal arteritis, is a type of vasculitis that primarily affects older adults, particularly those over the age of 50. The term "other giant cell arteritis" encompasses forms of GCA that do not fit into the more commonly recognized categories, such as classic temporal arteritis. This classification is essential for accurate diagnosis and treatment, as it helps differentiate between various presentations of the disease.

Symptoms

The symptoms of other giant cell arteritis can vary widely but often include:

  • Headaches: Severe, persistent headaches are common, often localized to the temples.
  • Visual Disturbances: Patients may experience blurred vision, double vision, or sudden vision loss due to ischemic optic neuropathy.
  • Jaw Claudication: Pain in the jaw while chewing, which can indicate involvement of the arteries supplying the jaw.
  • Fatigue and Malaise: General feelings of unwellness and fatigue are frequently reported.
  • Fever and Night Sweats: Low-grade fever and excessive sweating at night may occur.
  • Weight Loss: Unintentional weight loss can be a significant symptom.

Diagnosis

Diagnosing other giant cell arteritis involves a combination of clinical evaluation and diagnostic tests, including:

  • Clinical Assessment: A thorough history and physical examination focusing on symptoms and signs of systemic inflammation.
  • Laboratory Tests: Blood tests may reveal elevated inflammatory markers, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP).
  • Imaging Studies: Ultrasound of the temporal arteries can help identify characteristic findings, such as the "halo sign," indicating inflammation.
  • Biopsy: Temporal artery biopsy remains the gold standard for diagnosis, revealing the presence of giant cells and inflammation in the arterial wall.

Treatment

The primary treatment for other giant cell arteritis involves the use of corticosteroids to reduce inflammation and prevent complications, particularly vision loss. The treatment regimen typically includes:

  • High-Dose Corticosteroids: Initiated promptly to control symptoms and prevent complications.
  • Tapering: Gradual reduction of steroid dosage based on clinical response and laboratory markers of inflammation.
  • Additional Immunosuppressive Therapy: In some cases, other immunosuppressive agents may be considered if patients do not respond adequately to corticosteroids.

Prognosis and Complications

The prognosis for patients with other giant cell arteritis can vary. Early diagnosis and treatment are crucial to prevent serious complications, such as permanent vision loss. Long-term management may be necessary, as some patients experience relapses or require prolonged corticosteroid therapy.

Conclusion

ICD-10 code M31.6 for other giant cell arteritis encompasses a range of clinical presentations that require careful diagnosis and management. Understanding the symptoms, diagnostic criteria, and treatment options is essential for healthcare providers to ensure optimal patient outcomes. Early intervention is key to preventing complications associated with this potentially serious condition, highlighting the importance of awareness and prompt action in clinical practice.

Clinical Information

Giant cell arteritis (GCA), also known as temporal arteritis, is a significant inflammatory condition primarily affecting the large and medium-sized arteries, particularly those in the head and neck. The clinical presentation, signs, symptoms, and patient characteristics associated with GCA, specifically under the ICD-10 code M31.6 (Other giant cell arteritis), are crucial for accurate diagnosis and management.

Clinical Presentation

Symptoms

The symptoms of giant cell arteritis can vary widely among patients, but common manifestations include:

  • Headache: Often described as a new, severe headache, typically localized to the temples or occipital region. This headache may be persistent and can be accompanied by scalp tenderness[13].
  • Visual Disturbances: Patients may experience blurred vision, double vision, or sudden vision loss, which can occur due to ischemic optic neuropathy or retinal artery occlusion[14].
  • Jaw Claudication: Pain or discomfort in the jaw while chewing, which is a hallmark symptom of GCA, indicating involvement of the arteries supplying the jaw[14].
  • Fatigue and Malaise: General feelings of unwellness, fatigue, and a lack of energy are common complaints[13].
  • Fever and Night Sweats: Low-grade fever and night sweats may also be present, reflecting systemic inflammation[13].

Signs

Upon examination, healthcare providers may observe:

  • Temporal Artery Tenderness: The temporal arteries may be tender to palpation, and in some cases, they may appear swollen or nodular[14].
  • Reduced Pulsation: Diminished or absent pulse in the affected temporal artery can be noted during a physical examination[14].
  • Visual Impairment: Signs of visual loss or changes in visual acuity may be evident during an eye examination, particularly in cases of acute ischemia[14].

Patient Characteristics

Demographics

Giant cell arteritis predominantly affects older adults, with the following characteristics:

  • Age: Most commonly diagnosed in individuals over the age of 50, with a peak incidence in those aged 70 and older[13][14].
  • Gender: GCA is more prevalent in women than men, with a ratio of approximately 2:1 to 3:1[13].
  • Ethnicity: There is a higher incidence of GCA among individuals of Northern European descent compared to other ethnic groups[13].

Comorbidities

Patients with GCA often have associated conditions, including:

  • Polymyalgia Rheumatica (PMR): A significant proportion of GCA patients also have PMR, characterized by muscle pain and stiffness, particularly in the shoulders and hips[14].
  • Cardiovascular Risk Factors: Patients may have a history of hypertension, hyperlipidemia, or other cardiovascular diseases, which can complicate the clinical picture[14].

Conclusion

Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with giant cell arteritis under the ICD-10 code M31.6 is essential for timely diagnosis and treatment. Early recognition of symptoms such as headache, jaw claudication, and visual disturbances, along with awareness of the demographic profile of affected patients, can significantly impact patient outcomes. Given the potential for serious complications, including vision loss, prompt medical intervention is critical in managing this condition effectively.

Treatment Guidelines

Giant cell arteritis (GCA), classified under ICD-10 code M31.6, encompasses a group of inflammatory conditions affecting the large and medium-sized arteries, primarily the temporal arteries. This condition can lead to serious complications, including vision loss and stroke, making timely diagnosis and treatment crucial. Below, we explore the standard treatment approaches for GCA, focusing on pharmacological interventions, monitoring, and management of complications.

Pharmacological Treatment

Corticosteroids

The cornerstone of treatment for giant cell arteritis is the use of corticosteroids. High-dose oral prednisone is typically initiated immediately upon diagnosis to reduce inflammation and prevent complications. The standard starting dose is often around 40-60 mg per day, which may be adjusted based on the patient's response and side effects. The goal is to taper the dose gradually over time, usually within 6-12 months, depending on the patient's clinical status and laboratory markers of inflammation[1][2].

Additional Immunosuppressive Agents

In cases where patients experience relapses or have significant side effects from corticosteroids, additional immunosuppressive agents may be considered. Medications such as methotrexate or azathioprine can be used as steroid-sparing agents to help manage the disease while minimizing corticosteroid exposure[3]. Tocilizumab, an interleukin-6 receptor antagonist, has also been shown to be effective in treating GCA and may be used in conjunction with corticosteroids or as a monotherapy in patients who cannot tolerate steroids[4].

Monitoring and Follow-Up

Regular Assessments

Patients diagnosed with GCA require regular follow-up appointments to monitor disease activity and treatment response. This includes assessing symptoms, conducting physical examinations, and performing laboratory tests such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels to evaluate inflammation[5].

Screening for Complications

Due to the risk of complications such as vision loss and cerebrovascular events, patients should be monitored closely for any new neurological symptoms or visual disturbances. Prompt intervention is critical if these symptoms arise, as they may indicate an impending or existing complication[6].

Management of Complications

Vision Loss

In cases where patients experience sudden vision loss, immediate treatment with high-dose intravenous corticosteroids may be warranted to attempt to salvage vision. This is often accompanied by a thorough evaluation by an ophthalmologist[7].

Cardiovascular Risk Management

Patients with GCA often have comorbid conditions that increase their cardiovascular risk. Therefore, managing these risk factors—such as hypertension, hyperlipidemia, and diabetes—is essential in the overall treatment plan[8].

Conclusion

The management of giant cell arteritis (ICD-10 code M31.6) primarily revolves around the use of corticosteroids, with additional immunosuppressive therapies as needed. Regular monitoring for disease activity and complications is vital to ensure optimal patient outcomes. As research continues to evolve, treatment protocols may adapt, emphasizing the importance of personalized care in managing this complex condition. For patients experiencing significant side effects or relapses, consultation with a rheumatologist or specialist in vasculitis is recommended to tailor the treatment approach effectively.

Diagnostic Criteria

Giant cell arteritis (GCA), also known as temporal arteritis, is a type of vasculitis that primarily affects the large and medium-sized arteries, particularly those in the head and neck. The ICD-10 code M31.6 specifically refers to "Other giant cell arteritis," which encompasses forms of GCA that do not fit into the more commonly recognized categories, such as classic temporal arteritis.

Diagnostic Criteria for Giant Cell Arteritis

The diagnosis of giant cell arteritis typically involves a combination of clinical evaluation, laboratory tests, and imaging studies. The following criteria are commonly used:

1. Clinical Symptoms

  • Headache: Often described as a new, severe headache, particularly in the temporal region.
  • Scalp Tenderness: Patients may experience tenderness over the scalp, especially over the temporal arteries.
  • Jaw Claudication: Pain in the jaw during chewing, which can indicate involvement of the arteries supplying the jaw.
  • Visual Disturbances: Symptoms such as blurred vision or sudden vision loss can occur due to ischemia of the optic nerve.

2. Laboratory Findings

  • Elevated Erythrocyte Sedimentation Rate (ESR): A significantly elevated ESR is a common finding in GCA, indicating inflammation.
  • Elevated C-reactive Protein (CRP): Similar to ESR, CRP levels are often elevated in inflammatory conditions, including GCA.
  • Anemia: Mild anemia may be present due to chronic inflammation.

3. Imaging Studies

  • Ultrasound: Temporal artery ultrasound can reveal characteristic findings such as the "halo sign," which indicates wall edema.
  • Magnetic Resonance Angiography (MRA): This imaging technique can help visualize the involvement of larger vessels and assess for complications.

4. Histopathological Examination

  • Temporal Artery Biopsy: A definitive diagnosis can often be made through a biopsy of the temporal artery, which typically shows giant cell infiltration and inflammation of the arterial wall.

5. Exclusion of Other Conditions

  • It is crucial to rule out other causes of similar symptoms, such as infections or other types of vasculitis, to confirm the diagnosis of GCA.

Conclusion

The diagnosis of giant cell arteritis, particularly under the ICD-10 code M31.6, relies on a combination of clinical symptoms, laboratory tests, imaging studies, and, when necessary, histopathological examination. Early diagnosis and treatment are critical to prevent complications, especially vision loss, which can occur if the condition is not promptly addressed. If you have further questions or need more specific information regarding the diagnostic process or treatment options, feel free to ask!

Approximate Synonyms

ICD-10 code M31.6 refers to "Other giant cell arteritis," a specific classification within the broader category of systemic connective tissue disorders. This condition is primarily characterized by inflammation of the blood vessels, particularly affecting the large and medium-sized arteries. Below are alternative names and related terms associated with M31.6:

Alternative Names for Other Giant Cell Arteritis

  1. Atypical Giant Cell Arteritis: This term is often used to describe cases that do not fit the classic presentation of giant cell arteritis but still exhibit similar inflammatory characteristics.

  2. Non-Temporal Giant Cell Arteritis: This designation highlights cases where the inflammation occurs in arteries other than the temporal arteries, which are commonly affected in classic giant cell arteritis.

  3. Giant Cell Vasculitis: A broader term that encompasses various forms of vasculitis associated with giant cell inflammation, including M31.6.

  4. Giant Cell Arteritis, Unspecified: This term may be used in clinical settings when the specific type of giant cell arteritis is not clearly defined.

  1. Temporal Arteritis: While this specifically refers to giant cell arteritis affecting the temporal arteries, it is often mentioned in discussions about giant cell arteritis as a whole.

  2. Vasculitis: A general term for inflammation of blood vessels, which includes giant cell arteritis as one of its subtypes.

  3. Systemic Giant Cell Arteritis: This term may be used to describe cases that involve systemic symptoms or multiple vascular territories.

  4. Polymyalgia Rheumatica: Although distinct, this condition is often associated with giant cell arteritis and may share similar inflammatory pathways.

  5. Cranial Arteritis: A term that can refer to giant cell arteritis affecting cranial arteries, including the temporal arteries.

Understanding these alternative names and related terms can help in the accurate diagnosis and treatment of patients with giant cell arteritis, particularly in distinguishing between different forms of the disease and its manifestations.

Related Information

Description

Clinical Information

  • Severe headache often described as new
  • Blurred or double vision may occur
  • Jaw claudication while chewing
  • General fatigue and malaise common
  • Low-grade fever and night sweats present
  • Temporal artery tenderness on palpation
  • Reduced or absent pulse in temporal arteries
  • Visual impairment due to ischemia
  • Mostly affects individuals over 50 years old
  • More prevalent in women than men
  • Higher incidence among Northern Europeans
  • Often associated with polymyalgia rheumatica

Treatment Guidelines

  • Treat GCA with high-dose corticosteroids
  • Initiate 40-60 mg prednisone daily
  • Taper steroids over 6-12 months
  • Use immunosuppressive agents for relapses or side effects
  • Consider methotrexate or azathioprine as steroid-sparing agents
  • Monitor ESR and CRP levels regularly
  • Screen for complications such as vision loss and stroke
  • Treat sudden vision loss with high-dose IV corticosteroids
  • Manage cardiovascular risk factors
  • Evaluate patients by an ophthalmologist for vision loss

Diagnostic Criteria

  • New severe headache
  • Scalp tenderness over temporal arteries
  • Jaw pain during chewing (claudication)
  • Visual disturbances such as blurred vision
  • Elevated Erythrocyte Sedimentation Rate (ESR)
  • Elevated C-reactive Protein (CRP)
  • Mild anemia due to chronic inflammation
  • Temporal artery ultrasound 'halo sign'
  • Magnetic Resonance Angiography (MRA) of larger vessels
  • Giant cell infiltration in temporal artery biopsy

Approximate Synonyms

  • Atypical Giant Cell Arteritis
  • Non-Temporal Giant Cell Arteritis
  • Giant Cell Vasculitis
  • Giant Cell Arteritis Unspecified
  • Temporal Arteritis
  • Vasculitis
  • Systemic Giant Cell Arteritis
  • Polymyalgia Rheumatica
  • Cranial Arteritis

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