About pulmonary angiitis

What is pulmonary angiitis?

Pulmonary angiitis is a term used to describe a collection of disorders with both a vascular (angiitis) and granulomatous components. It means inflammation of blood vessels in the lungs. 

There are five unique clinical syndromes that have been identified, including: 

  • Polyangiitis with eosinophilic granulomatosis (previously known as Churg-Strauss syndrome) 

Eosinophilic granulomatosis with polyangiitis (EGPA), often known as Churg-Strauss syndrome (CSS), is a necrotizing pulmonary vasculitis characterized by tiny to medium vessels. It's also a kind of pulmonary angiitis and granulomatosis, and it's part of the eosinophilic lung disease spectrum. 

  • Bronchocentric granulomatosis is a type of granulomatosis that affects the lungs 

Bronchocentric granulomatosis is an uncommon chronic illness in which granulomas grow in the airways as a result of several traumas. It is part of the eosinophilic lung disease spectrum. 

  • Polyangiitis with granulomatosis

GPA, also known as Wegener granulomatosis, is a multisystem necrotizing non-caseating granulomatous c-ANCA positive vasculitis that affects small to medium-sized arteries, capillaries, and veins, with a preference for the respiratory system and kidneys. 

  • Lymphomatoid granulomatosis is a type of lymphomatoid granulomatosis. 

 Because the lung is one of the most common locations of lymphomatoid granulomatosis involvement, pulmonary symptoms of lymphomatoid granulomatosis are relevant.

  • Granulomatous sarcoid necrotizing 

NSG (necrotizing sarcoid granulomatosis) is a rare systemic disease characterized by the production of sarcoid-like granulomas, vasculitis, and varying degrees of necrosis. It's been lumped in with pulmonary angiitis and granulomatosis on occasion. 

What are the symptoms for pulmonary angiitis?

Most kinds of Pulmonary angiitis have the following signs and symptoms:

1) asthma

2) Constitutional (fever, chills, weight loss, arthralgias/myalgias)

3) System of nerves (mono neuritis multiplex, CNS, cerebral hemorrhage)

4) Cutaneous Sinusitis (purpura, urticaria, subcutaneous nodules, and exanthem)

5) Cardiac arrest (pericarditis, heart failure, coronary vasculitis)

6) G.I. (diarrhea, GI bleeding, colitis, pain)

7) Renal system (proteinuria, hematuria)

The following are the symptoms of the clinical syndromes: 

1) Polyangiitis with eosinophilic granulomatosis (EGPA)

asthma and eosinophilia affect almost every patient. Sinusitis, diarrhea, skin purpura, and/or arthralgias are common extrapulmonary signs and symptoms.

2) Bronchocentric granulomatosis is a type of granulomatosis that affects the bronchial

Tissue eosinophilia affects about one-third to half of the patients, who also have asthma, peripheral eosinophilia, fungal hyphae at biopsy, and positive sputum cultures for Aspergillus species. 

3) Polyangiitis with granulomatosis (GPA)

The following organ symptoms are involved in the presentation:

  • hemoptysis and cough
  • a history of nasal blockage, rhinitis, or epistaxis ranging from subacute to chronic
  • hematuria with proteinuria

Other organ system symptoms are less common, owing to a lower frequency of involvement (musculoskeletal symptoms, ocular symptoms, skin abnormalities). Anorexia, malaise, and Fever are all prevalent systemic symptoms.

 4) Sarcoid Granulomatosis Necrotizing (NSG)

 Although Dyspnea and chest discomfort are common, up to 25% of patients may be asymptomatic. It usually affects people in their forties or fifties.

List of Symptoms

1) asthma

2) Constitutional (fever, chills, weight loss, arthralgias/myalgias)

3) System of nerves (mono neuritis multiplex, CNS, cerebral hemorrhage)

5) Cardiac arrest (pericarditis, heart failure, coronary vasculitis)

6) G.I. (diarrhea, GI bleeding, colitis, pain)

List of Conditions

  1. Hepatitis B and C infections
  2. Cancers of the blood
  3. Rheumatoid arthritis, lupus, and scleroderma are examples of immune system illnesses.
  4. Adverse medication reactions

List of Drugs

  1. cyclophosphamide (CYC)
  2. rituximab (RTX)

What are the causes for pulmonary angiitis?

Pulmonary angiitis is caused by a variety of factors that aren't completely understood. Some types are influenced by a person's genetics. Others are caused by the immune system attacking blood vessel cells. Possible triggers include the following:

  • Hepatitis B and C infections
  • Cancers of the blood
  • Rheumatoid arthritis, lupus, and scleroderma are examples of immune system illnesses.
  • Adverse medication reactions

Anyone can develop pulmonary angiitis. The following are some of the factors that may enhance the risk of certain disorders:

  • Age: Kawasaki illness is most frequent in children under the age of five, while giant cell arteritis occurs seldom before the age of 50.
  • Your family's history: Kawasaki illness, Behcet's disease, and granulomatosis with polyangiitis can all run in families.
  • Choices of lifestyle: Cocaine use raises your chances of getting vasculitis. Tobacco use can increase your chance of Buerger's disease, especially if you're a man under 45.
  • Medications: Medications including hydralazine, allopurinol, minocycline, and propylthiouracil can sometimes cause vasculitis.
  • Infections: Vasculitis is more likely if you have hepatitis B or C.
  • Immune system problems: Vasculitis is more common in people who have immune system problems in which their immune systems wrongly assault their own bodies. 

What are the treatments for pulmonary angiitis?

Treatment of life-threatening pulmonary angiitis and irreparable damage to organs, notably the kidneys, needs quick diagnosis. Once the diagnosis is done, the following therapies are followed:

Induction therapy
  • Immunosuppressive therapy with corticosteroids and cytotoxic immunosuppressive drugs, with rising intensity dependent on disease severity, is required for pulmonary angiitis. 
  • Patients with severe DAH or RPGN should receive high-dose "pulse" steroids in combination with either cyclophosphamide (CYC) or rituximab (RTX).
  • Patients with acute renal failure and blood creatinine levels more than 2.5 mg/dL may be evaluated for plasmapheresis.
  • In addition to pulse steroids, two drugs to consider for induction are IV rituximab or cyclophosphamide (CYC), both of which may be taken IV in pulse dosing or orally in "continuous" dosing.
  • Though CYC was long thought to be the first line, new statistics suggest that RTX is at least as effective as CYC. We feel that since RTX has less long-term concerns about cancer risk, it should be the preferred induction agent.
  • The goal of first induction treatment is to achieve remission, and it should be followed by maintenance therapy.

What are the risk factors for pulmonary angiitis?

The term ‘vasculitis’ refers to the inflammation of the blood vessels in the body. Angiitis is a synonym for vasculitis. Our body has three different types of blood vessels- the arteries, veins, and capillaries. Pulmonary angiitis, therefore, refers to inflammation of these blood vessels specifically in the lung. 

The following can determine your susceptibility to pulmonary angiitis:

  • Age - People of any age can develop the disease, so age is not a factor 
  • Long-term immune system linked diseases - Presence of autoimmune diseases such as lupus and rheumatoid arthritis, or malignancy
  • Infectious diseases 
  • Connective tissue diseases - Presence of disease that affects the tissues connecting different body parts such as lupus, scleroderma, Churg-Strauss syndrome 
  • Genetic disposition - Pulmonary angiitis can be hereditary and passed on through generations 
  • Lifestyle choices - Certain habits like smoking can also contribute to the disease 
  • Medication - Allergies or sensitivity to specific medicines can also trigger immune reactions that lead to inflammation of blood vessels in the lung. Either proactively avoid such medications or inform your doctor about known allergies 
  • Existing respiratory conditions - Including sinusitis, rhinitis, and asthma 

Above are the most common risk factors for pulmonary angiitis. Track the above factors and visit a doctor to schedule an imaging appointment with the radiologist if you are facing discomfort associated with the lungs. 

Is there a cure/medications for pulmonary angiitis?

While pulmonary angiitis is a long-term disease and there is no cure for it, an early diagnosis can help decide the best course of treatment and minimize lung damage. Diagnosis will require tests such as:

  • Blood test 
  • Urine test 
  • Imaging such as chest x-ray or CT scan 
  • Bronchoscopy 
  • Lung biopsy 

Often, doctors find it difficult to arrive at the pulmonary angiitis diagnosis since its test results and symptoms can mimic those of other diseases such as infections, auto-immune disorders, drug allergies, and malignancies. After testing and diagnosis, the following course of treatment is typically adopted: 

  • The doctor will prescribe a long-term steroid medication (oral corticosteroids like prednisone) to help reduce the inflammation 
  • This will be complemented with immunosuppressive therapy (drugs like methotrexate, cyclophosphamide, and tocilizumab), the intensity of which is determined by the severity of the disease and its damage
  • The goal of treatment is to get the patient into remission (reduce/resolve symptoms) 
  • Once remission is achieved, the doctor will slowly stop medication or alter dosage to help with maintenance 
  • Regular check-ups will continue to monitor side effects, pulmonary angiitis flare-ups, infection, or complications. 

List of conditions:

  • inflammation of blood vessels in the lungs 
  • Blood vessels narrow or close causing organ damage 
  • inflammation can also cause blood vessels to bulge leading to aneurysms

List of symptoms:

  • Cold-like effects
  • Fever
  • Tiredness 
  • Weight loss 
  • Shortness of breath 
  • Cough
  • Spitting of blood
  • Chest pain 
  • Hypertension 

List of drugs:

  • Oral steroids like Prednisone to regulate inflammation 
  • Immunosuppresants like Methotrexate, Cyclophosphamide or Tocilizumab to stop immune system’s attack on blood vessels and prevent further inflammation

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