Patient Presentation
A 17-year-old female was hospitalized with a few week history of fatigue, weight loss and general malaise. She developed increasing cough and difficulty breathing that brought her to the local emergency room and a chest radiograph was markedly abnormal with cavitary lesions.

The past medical history was negative. The family history was positive for diabetes, thyroid disease and arthritis. She had no significant travel or recreational history and had not been exposed to any birds or any other non-domestic animals. She denied intentional weight loss or increased exercise.

The review of systems was negative for any gastrointestinal, dermatological or eye problems. She denied any syncope, mental status changes, hemoptysis or changes in her bowels or bladder. She denied any specific problems with her joints or muscles but said she just “felt weak,” and didn’t feel like moving much.

The pertinent physical exam showed an ill-appearing teenager with heart rate of 92 beats/min, respiratory rate of 32/minute, with normal blood pressure and temperature. She was 163 cm (50%) and weight was 65.2 kg (75%) which was down from 68.4 kg a few weeks previously. HEENT was positive for increased tearing but there was no obvious erythema of the eye structures. She had no skin changes including changes to any mucous membranes. She had no obvious nail clubbing nor significant lymphadenopathy. Her cardiac examination was normal as was her abdomen. Her lung examination had decreased breath sounds throughout but no specific wheezing or rales.

The diagnosis of cavitary pulmonary disease was made. The initial differential diagnosis focused on infections (especially tuberculosis, fungal or abscess), congenital lesions and much less likely pulmonary infarction or oncological disease. The work-up was extensive and eventually a diagnosis of granulomatosis with polyangiitis was made based on abnormal chest radiograph, abnormal urinalysis and eventually kidney biopsy and elevated anti-neutrophil cytoplasmic antibodies. She was treated with cyclophosphamide and corticosteroids.

Figure 145 – Chest radiograph AP and lateral (above) show bilateral thick walled cavitary nodules in the lungs. Axial CT with contrast of the chest (below) shows just how thick walled the cavitary nodules are in the left lung. Multiple smaller non-cavitary nodules are seen scattered throughout both lungs.

Discussion
“Granulomatosis with polyangiitis (GPA), previously referred to as Wegner’s granulomatosis, is a necrotizing vasculitis that affects small and medium-sized blood vessels. It is a systemic disease that results in inflammatory, non-caseating granulomatous lesions that may develop in any organ, but are most commonly in the respiratory tract and kidney. GPA is associated with elevated anti-neutrophil cytoplasmic antibodies (ANCA), similar to microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.” The role of ANCA in the disease process is being elucidated. Other ANCA-associated vasculitides including microscopic polyangiitis and eosinohilic granulomatosis with polyangiitis (previously known as Churg-Strauss syndrome).

Most patients with GPA are middle age, and pediatric cases are rare and mainly in the adolescent age range. Diagnosis is made by three of six criteria: “…abnormal urinalysis (hematuria and/or significant proteinuria); granulomatous inflammation on biopsy; nasal sinus inflammation; subglottic, tracheal, or endobronchial stenosis; abnormal chest X-ray; or CT, PR3 ANCA or C-ANCA staining.” ANCA if positive can be helpful but in up to 40% of pediatric patients it may be negative especially if there is localized disease. If needed, biopsy can be performed and the kidney is the preferred site.

There are two disease forms, localized and systemic. Localized disease is in the upper airway (especially the subglottic area (up to ~48% in the pediatric age group and is the most common area affected)) but other areas such as the distal trachea and bronchi can be affected. Systemic disease tends to involve the respiratory and renal systems. Renal disease can progress to renal failure and death. Cardiac, gastrointestinal and neurological systems are involved only very rarely. Localized disease tends to relapse whereas systemic disease is less likely to do so. A variety of immunomodulators, chemotherapeutic agents and steroids are used to treat the disease.

Learning Point
Signs and symptoms of GPA include:

• Systemic
  • Fatigue
  • Fever
  • Arthralgia
  • Myalgia
  • Weight loss
• Dermatological
  • Gangrene
  • Purpura
  • Ulcers
• Pulmonary – affects 50% of patients
  • Cough
  • Dyspnea
  • Wheezing
  • Hemoptysis
  • Stridor
  • Nasoseptal perforation
  • Mass
• Renal
  • Necrotizing glomerulonephritis
  • Hematuria
  • Proteinuria
• Ophthalmological
  • Corneal ulcers
  • Episcleritis
  • Scleritis
  • Masses, granulomatous
  • Retinal thrombosis or vasculitis
  • Visual acuity changes

Questions for Further Discussion
1. What causes cavitary pulmonary lesions?
2. What causes hemoptysis? A review can be found here
3. What causes respiratory failure? A review can be found here
4. What are therapy options for renal replacement? A review can be found here

Related Cases

Disease: Granulomatosis with Polyangiitis | Wegener’s Granulomatosis

Symptom/Presentation: Fatigue | Weight Loss | Respiratory Distress

Specialty: Immunology | Allergy / Pulmonary Diseases

Age: Teenager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Granulomatosis with Polyangiitis and Vasculitis

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To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Filocamo G, Torreggiani S, Agostoni C, Esposito S. Lung involvement in childhood onset granulomatosis with polyangiitis. Pediatr Rheumatol Online J. 2017;15:28. doi:10.1186/s12969-017-0150-8

Lee PY, Adil EA, Irace AL, et al. The presentation and management of granulomatosis with polyangiitis (Wegener’s Granulomatosis) in the pediatric airway. The Laryngoscope. 2017;127(1):233-240. doi:10.1002/lary.26013

Calabrese V, Gallizzi R, Spagnolo A, et al. Granulomatosis with Polyangiitis: A Focus on Differences and Similarities Between Child and Adult Patients. Medicina (Kaunas). 2025;61(3):534. doi:10.3390/medicina61030534

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa