Clinical History:
A 37-year-old woman came with a history of intermittent frontal headache a year ago with mild hyperthermia, fatigue, and general asthenia with skeletal pain with a history of recurrent nasal congestion, the patient was initially treated as chronic sinusitis and a tension headache, but the response was minimal. After 4 months, she developed a nasal shape malformation in which several biopsies were taken from it and the pharynx. The biopsy result was nonguiding (unspecified lymphatic hyperplasia), and the patient received an empirical treatment for tuberculosis with little response to treatment.
Figure 4- Nasal Recession
During the treatment period, the patient developed a cough-producing yellow sputum with hemoptysis in addition to frequent nosebleeds, and upon her review of the Martyr Muhammad Wasim Moaz Hospital, which is supported by the Independent Doctors Assosication IDA. The patient was admitted to the hospital by the resident doctor in Internal Medicine, Saleh Al-Yousef.
Clinical examination:
Clinical examination showed severe pallor of the skin and conjunctiva with damage to the nasal septum (saddle nose).
- Chest examination: coarseness of respiratory sounds with spreading wheezing in both lungs.
- Cardiac examination: within normal
- Limb examination: palpable purpura in the lower extremities, which later developed and extended to the upper extremities (wrist and back of the hand).
Figure5Purpura on legs and feet
An ENT consultation was performed, and the diagnosis was chronic sinusitis.
Laboratory and radiological tests:
A simple chest scan showed the presence of a small nodule in the vicinity of the left lung, so a CT of the chest was performed after consulting the supervising doctor, Dr. Issa Al-Khalil. The CT showed a density in the upper lobe of the left lung with an irregular background of edges measuring 25 mm
CT scan showing density in the upper lobe Unstructured left lung 25 mm
Laboratory tests:
- Creatinine: 1.9
- CRP: 102
- ESR: 100
- HGB: 5.5
- LDH: 281
- INR: 1.55
The supervising college specialist, Dr. Khaled Haj Naasan, was consulted, and a urinary echograph was performed which was normal, and a urine analysis was requested, which showed the presence of hematuria and proteinuria. So, the trend was towards small vessel inflammation, and therefore some immunological tests were requested, which were negative
- ANA negative
- C-ANCA negative
- P-ANCA negative
- Anti-GBM negative
- Natural C3 and C4 .
Therefore, the resident doctor, Mohammed Sheikh Yusuf, under the supervision of the kidney specialist, performed a biopsy of the kidney guided by echography.
Management:
Corticosteroids (methylprednisolone IV) were started with intravenous cyclophosphamide and after two weeks the biopsy resulted in multi-granulomatous vasculitis.
The patient went to the kidney clinic and found excellent improvement, hemoptysis and hematuria stopped, and blood creatine returned to 0.9 mg/dl.
Discussion:
Poly granulomatous vasculitis, formerly known as Wagner Granulomatosis, is a rare idiopathic disease that affects people between the ages of 40 and 60 and causes inflammation of the blood vessels of the nose, sinuses, lungs, and kidneys.
Signs and symptoms of the disease can appear suddenly or over several months. The first warning signs usually include sinus inflammation manifesting as sinusitis and may lead to nasal Recession, or it may affect the lungs, which manifests as hemoptysis, as in this patient. The condition often worsens quickly, affecting some important organs that supply it with blood, such as the kidneys, which determine the patient’s prognosis and manifest as rapidly progressive glomerulonephritis, as in this patient.
This vasculitis is an inflammation of the small vessels so it is possible to see cutaneous purpura and we may also see companion polyneuritis and articular pain.
A high sedimentation rate and anemia are common manifestations of the disease and an ANCA test can be ordered, which is positive in only 30% of cases.
The disease is definitely diagnosed by biopsy of some of the affected organs, such as the kidneys and lungs, except for the nose and sinuses, which are not indicated.
Treatment:
The condition may be cured in a few months with early diagnosis and the use of appropriate treatment. Treatment includes corticosteroids and immunosuppressive drugs such as cyclophosphamide, azathioprine, and mycophenolate mofetil. Rituximab is also another treatment option, and the duration of treatment is long at this time and may reach two years to prevent relapse.
References:
- https://www.nhs.uk/conditions/granulomatosis-with-polyangiitis/
- Harrison’s Principles of Internal Medicine, 20e, Chapter 356.2: Granulomatosis with Polyangiitis (Wegener’s)