Granulomatosis with Polyangiitis

Welcome to PT 635 Pathophysiology of Complex Patient Problems This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!

Original Editors - Dalie Camic & Daryl Williams from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

Wegener's granulomatosis is a rare disorder characterized by inflammation in the blood vessels, which in turn decreases blood flow to organs and systems.  Most affected areas include the kidneys, lungs, and upper respiratory tracts.

Prevalence

Wegener's granulomatosis is most common in middle aged adults, but has been seen in children as young as 3 months of age. There is also increased incidence in caucasians versus African-American populations

Characteristics/Clinical Presentation

The signs and symptoms of Wegener's Granulomatosis are not only common signs and symptoms of other diseases or illnesses but are often vague in the beginning presentations of this disease.  The first signs and symptoms include upper respiratory tract symptoms, joint pains, weakness, and tiredness.

Respiratory System

Common involvement occurs in the upper respiratory tract in nearly all patients.  

Symptoms include:  sinus pain, discolored or bloody fluid from nose, and nasal ulcers.  

Common signs include:  rhinorrhea ("runny nose"), common cold symptoms that do NOT respond to usual treatment and become increasingly worse.  Although this these are the most prevelant signs Wegener's Granulomatosis is rarely the cause.

Lungs:  Commonly affected in patients with Wegener's Granulomatosis there are rarely signs and symptoms that present.  Possible S&S can include cough, hemoptysis, shortness of breath, and chest discomfort.

Musculoskeletal system

Although it does not lead to permanent joint damage or deformities, pain in the muscle and joints, along with joint swelling affects two-thirds of individuals with this disease.

-Other signs and symptoms can include develop skin lesions resmembling red or purple raised lesions.  The eyes can be affected causing conjunctivitis, scleritis, episcleritis causing double vision, redness, burrning, and pain.  Symptoms involving the eyes require immediate medical attention.

Associated Co-morbidities

Ischemic heart disease

Glucocorticoid-induced osteoporosis

Medications

In an article published in the Annals of Internal Medicine an analysis of 158 patients with Wegener Granulomatosis was prospectively studied to to analyze the clinical features, pathophysiology, treatment, and prognosis of the disease.

-138 Patients recieved "standard therapy" with daily low cyclophosphamide and glucocorticoids; 8 patients recieved only low-dose cyclophophamide; 6 patients were treated with other cytotoxic agents and glucocorticoids, and 10 patients were treated with only glucocorticoids.

- 91% percent of the patients experienced marked improvement, and 75% achieved complete remission.  

-The use of these medications shown a dramatic improvement by daily treatment with cyclophosphamide and glucocorticoids.  Alternative forms have not yet achieved the high rates of remission induction and maintaenece that have been reported with cyclophophamide treatment.  

-A major concern with the long term follow up of these patients is the toxicity resulting from prolonged cyclophosphamide therapy.

According pubmedhealth, treatment may include medicines that slow down the immune system response.  Some of these medications include:

-Gucocorticoids (Prednisone)

-Azathioprine

-Cyclophosphamide

-Methotrexate

These medications may be used in combination with other medications to help reduce side effects:

-Biphosphonate-to prevenent bone loss caused by prednisone.

-Folic acid

-Trimethoprim-to prevent lung infections.

Diagnostic Tests/Lab Tests/Lab Values

Blood panel

• Antineutrophil cytoplasmic antibodies (ANCA) is a common protein found in patients with WG.

Urinalysis

• Assess kidney function and check for output, blood or proteins in the urine

Biopsy is common and will vary depending on organ involvement. Can include:

• lung
• kidney
• nasal mucosa
• upper airway
• skin

Other tests may be order to further evaluate the patient and confirm diagnosis:

• chest x-ray
• chest CT
• bone marrow aspiration

Etiology/Causes

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Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Recent Related Research (from Pubmed)

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References

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