Granulomatosis with Polyangiitis (Wegener Granulomatosis)

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Image Source: The New England Journal of Medicine©

A 42-year-old man with a four-year history of rhinosinusitis came to the outpatient department with complaints of a 3-month history of shortness of breath, fever and progressive cough. Patined denied the use of cocaine. Upon inspection, a saddle-nose deformity (Panel A) was observed. Examination of the nose revealed crusting and inflamed nasal mucosa. On auscultation of the lungs, bilateral wheezes and rales were heard.

A computed tomographic (CT) scan of the face and chest was conducted (Panel B), which showed an enlarged nasal cavity secondary to extensive bony destruction of the midface and numerous nodules in the lungs, respectively. A diagnosis of granulomatosis with polyangiitis was suspected which was further backed up by the positive proteinase 3 antineutrophil cytoplasmic antibody (ANCA) in the serum.

Treatment was initiated with immunosuppressive drugs.

Substantial clinical recovery was noticed on the 6-month follow-up. A repeat CT scan was conducted which proved the improvement as the lung nodules were resolved and the facial findings remained stable.

Granulomatosis with polyangiitis, which was formerly known as Wegener’s granulomatosis, is a rare disease of the small and medium-sized blood vessels. It usually affects the respiratory system and the kidneys, although other organs may also be involved.

It affects both men and women equally with a ratio of 3 in 100,000 people.

The initial symptoms are usually attributable to the upper respiratory tract including nasal congestion, nose bleeds, shortness of breath, cough with hemoptysis (blood in sputum).  Other clinical features may include:

Arthralgia (joint pain)
Malaise
Fever and night sweats
Numbness
Loss of appetite and weight loss

Although the symptoms progress gradually if not treated in time, the disease may get aggressive enough to cause tissue and organ damage.

No single test can definitely diagnose granulomatosis with polyangiitis.

The clinical features, physical examination, and investigations, all play a role in reaching a diagnosis. Apart from serum tests and imaging, a biopsy from the affected tissue may also aid in confirming the diagnosis.

Renal biopsy specimen shows focal glomerulonephritis, characteristic of granulomatosis with polyangiitis.
Image Source: Medscape©

The most common serological test is the ANCA (anti-neutrophil cytoplasmic antibody) test, an antibody that is not specific for GPA but helps support the clinical picture.

A combination of corticosteroids and cytotoxic agents is the mainstay of treatment.

Usually, up to 75% of patients respond well to this treatment regimen. However, up to 50% of the patients may relapse even after complete recovery. It is imperative to treat the patient before the disease gets worse because a severe GPA has a poor prognosis with an average survival of 2 years.

Surgical interventions may be required in cases where tissues undergo necrosis, fibrosis or bony deformities. For patients with end-stage renal disease, renal transplantation is an option.

After renal transplantation, will the disease affect the transplanted kidney?
No, usually glomerulonephritis does not affect the transplanted kidney.

References

Christopher L Tracy, M. (2019, October 09). Granulomatosis with Polyangiitis (Wegener Granulomatosis). Retrieved from Medscape: https://emedicine.medscape.com/article/332622-overview

Federica Bello, M. a. (2020, March 05). Nasal Deformity in Granulomatosis with Polyangiitis. Retrieved from The New England Journal of Medicine: https://www.nejm.org/doi/full/10.1056/NEJMicm1909658

Vaneet Sandhu, M. (2019, March). Granulomatosis with Polyangiitis (Wegener’s). Retrieved from American College of Rheumatology: https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Granulomatosis-with-Polyangitis-Wegners

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Dr. Arsia Hanif has been a meritorious Healthcare professional with a proven track record throughout her academic life securing first position in her MCAT examination and then, in 2017, she successfully completed her Bachelors of Medicine and Surgery from Dow University of Health Sciences. She has had the opportunity to apply her theoretical knowledge to the real-life scenarios, as a House Officer (HO) serving at Civil Hospital. Whilst working at the Civil Hospital, she discovered that nothing satisfies her more than helping other humans in need and since then has made a commitment to implement her expertise in the field of medicine to cure the sick and regain the state of health and well-being. Being a Doctor is exactly what you’d think it’s like. She is the colleague at work that everyone wants to know but nobody wants to be. If you want to get something done, you approach her – everyone knows that! She is currently studying with Medical Council of Canada and aspires to be a leading Neurologist someday. Alongside, she has taken up medical writing to exercise her skills of delivering comprehensible version of the otherwise difficult medical literature. Her breaks comprise either of swimming, volunteering services at a Medical Camp or spending time with family.

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