A general term for a large group of diseases, vasculitis is a condition that causes inflammation and narrowing of the blood vessels (arteries, veins and capillaries) that carry blood to and from the heart and the body’s organs. Also called angiitis or arteritis, vasculitis can cause alterations in the walls of the blood vessels such as weakening, scarring, narrowing and thickening that cause them to close entirely. These changes can restrict blood flow, resulting in organ and tissue damage. The condition can be either acute and short-term or chronic and long-term, affecting different parts of the body, causing a wide range of symptoms. In certain cases, the organs in the body may be affected (when there is lack of nutrients and oxygen-rich blood), resulting in serious organ damage and sometimes death. Symptoms of the condition depend on the type of vasculitis a person suffers from and the part of the organ system affected. If left untreated, the condition can be life-threatening causing stretching and bulging of blood vessels (called an aneurysm). The blood vessels may also burst open, causing severe bleeding. Treatment options for this condition depend on what factors are causing vasculitis and which organs get affected. Rheumatologists, neurologists and other specialists providing treatment need to correctly document the same in the patients’ medical records. Opting for medical billing services from an established medical billing company can help simplify the documentation process.

As mentioned above, vasculitis occurs when the body’s immune system mistakenly attacks its blood vessels. However, the exact causes behind this immune system attack are not fully known. The condition can occur either on its own, or may be linked to certain other factors like immune system disorders, infections (hepatitis B or C), allergic reactions to medications, and certain types of cancers (such as leukemia and lymphoma). It can also occur due to certain rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE/ lupus) or Sjogrens syndrome.

Types of Vasculitis

Generally, physicians group vasculitis according to the size of the blood vessels affected. Most types of vasculitis are rare and include –

  • Large blood vessel – This includes conditions like polymyalgia rheumatica, Takayasu’s arteritis, and temporal arteritis.
  • Medium vessel – This includes Buerger’s disease, cutaneous vasculitis, Kawasaki disease, and polyarteritis nodosa.
  • Small vessel – This includes Behcet’s syndrome, granulomatosis with polyangiitis, Churg-Strauss syndrome, cutaneous vasculitis, golfer’s vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, and cryoglobulinemia.

Signs and Symptoms

The symptoms associated with the condition may occur once or several times over several years. The condition affects people of all age groups. The signs and symptoms vary and can range from mild to life-threatening depending on the type of vasculitis, the specific affected area and the severity of the condition. For some people, symptoms may be few and develop slowly, over months, while at other times, the signs and symptoms start quickly, over days or weeks. Some of the common symptoms are loss of appetite, fatigue, fever, weight loss, night sweats, rashes, nerve problems (such as numbness or weakness) and general aches and pains. There are other signs and symptoms that are related only to certain types of vasculitis and these include –

  • Weakness and numbness or tingling sensation
  • Skin changes
  • Scalp tenderness
  • Nerve pain and nasal allergies
  • Mouth and genital ulcers
  • Joint pain
  • Jaw pain
  • Headaches
  • Eye inflammation
  • Blurred or double vision
  • Acne-like skin lesions
  • Abdominal pain
  • Blood in the urine

Diagnosis and Treatment of Vasculitis

Diagnosis of this condition may generally begin with a medical history analysis and physical examination. Patients need to undergo tests to look for inflammation or to rule out other conditions. These may include – blood tests, urine tests (to check for kidney damage), imaging tests, heart tests and biopsy. Blood tests may be performed to look for signs of inflammation and these may include – Erythrocyte sedimentation rate (ESR) test, C-reactive protein (CRP) test, a complete blood count (CBC) test and platelet count test. Imaging tests for vasculitis include X-rays, ultrasound, computerized tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET). An echocardiogram will be performed to test the functioning of the heart. Biopsy may be performed wherein a sample of tissue from your blood vessel or organ will be taken to check for signs of inflammation or damage.

Treatment for the condition depends on the relative causes and the specific organs affected. If vasculitis is the result of an allergic reaction, it does not require any specific treatment and may go away on its own. However, if specific organs like lungs, brain or kidneys are involved, treatment is required. Treatment modalities for this condition focus on controlling the inflammation levels and resolving any underlying diseases that triggered the symptoms. Treatment involves two phases – first stopping the inflammation and then preventing relapse (maintenance therapy). Medications prescribed by physicians include corticosteroid drugs such as prednisone or methylprednisolone (Medrol) that help control inflammation. Other medications that may be prescribed with corticosteroids include methotrexate (Trexall), azathioprine (Imuran, Azasan), mycophenolate (Cellcept) or cyclophosphamide. Biologic therapies such as rituximab (Rituxan) or tocilizumab (Actemra) may be recommended, depending on the type of vasculitis. If the condition causes a balloon-like bulge (aneurysm) to form in the wall of a blood vessel – a surgery may be required. Blocked arteries also may require surgical treatment. Once the vasculitis is under control, physicians will slowly stop the medications. However, regular checkups may be required to manage vasculitis flare-ups, and other related complications.

Medical billing and coding can be challenging for this auto-immune disorder as it involves using several codes. ICD-10 diagnosis codes for vasculitis include –

  • L95 Vasculitis limited to skin, not elsewhere classified
  • L95.0 Livedoid vasculitis
  • L95.1 Erythema elevatum diutinum
  • L95.8 Other vasculitis limited to the skin
  • L95.9 Vasculitis limited to the skin, unspecified

Vasculitis complications may depend on the type and severity of the condition. Certain factors like smoking, chronic hepatitis B or C infections, and some types of autoimmune conditions such as rheumatoid arthritis, scleroderma or lupus can increase the risk of this condition. Apart from undergoing appropriate treatment, following certain lifestyle habits like eating a healthy diet, getting routine vaccinations and exercising regularly can help better manage vasculitis in the long run.

With the high specificity involved in ICD-10 codes, specialists treating vasculitis need to correctly document the diagnosis and other treatment procedures performed. Partnering with an experienced medical coding company is a great option for physicians to ensure accurate and timely medical coding and claim submissions.