TOPIC: Wegener Granulomatosis, a syndrome of systemic Vasculitis

Authors: Dr. Sandeep jain1, Dr. Sabyasachi Chowdhury2, Dr. Nilotpal Bhattacherjee1, Dr. Prasenjit Ghata1
1
Post Graduate Trainee, Department of Respiratory Medicine, Medical College and Hospitals, Kolkata.
2
Assistant Professor, Department of Respiratory Medicine, Medical College and Hospitals, Kolkata.
Email: jainsandeepdr@gmail.com
Contact number: +91 9836111241

ABSTRACT:
Background:
Wegener’s granulomatosis, known as granulomatosis with polyangitis (GPA), involves autoimmune
attack by abnormal circulating antibody, called ANCA (Anti-neutophil cytoplasmic antibody) against
small and medium sized vessels.GPA, a rare systemic disorder, can involve Kidneys (RPGN, often leading
to CKD),ears(Conductive hearing loss),eyes(scleritis,conjunctivitis),trachea(subglottal stenosis),arthritis,
skin(subcutaneous nodules,purpura,cutaneous vasculitis),nervous system (sensory neuropathy) and
lungs(pulmonary nodules,infiltrates sometimes mimicking pneumonia,cavitary lesions,pulmonary
haemorrhage and rarely bronchial stenosis).
Exact cause of this disease is unknown, though it is presumed that ANCA(typically that react with
Proteinase3) are responsible for the inflammation. Infectious causes and genetic basis has also been
implicated.

Strong clinical and radiological suspicion and presence of ANCA can help in the diagnosis. Biopsies may
also be obtained, that would reveal leucocytic vasculitis with necrosis and granuloma.

Apart from supportive care, immunosuppressants form the mainstay of treatment(Corticosteroids and
Rituximab/Cyclophosphamide/methotrexate depending on severity).

Case Summary:

A 27 year old, Non-Diabetic, Non-hypertensive, Euthyroid, immune-competent hindu female presented

with complains of dry cough (insidious in onset, gradually progressive, no diurnal or postural variation),
left sided chest pain and exertional shortness of breath (MMRC 2) for 2 weeks and streaky hemoptysis
for last 2 days. No past history of tuberculosis, but she has been having difficulty in hearing in both the
ears and has been having mild pain in multiple joints for the past few months. Chest ChestXray revealed
multiple nodular shadows. Patient was provisionally diagnosed to be having multilobar pneumonia with
anemia. CT scans revealed bilateral CSOM, and nodular opacities in both lungs (few cavitating) with left
pleural effusion, and cysts in both the kidneys. FNAC and tru-cut biopsy from lung lesion were
inconclusive. ANA profile was positive for Anti-PR3 antibody and RA factor was positive.
Patient lost to followup.
Conclusion:
In India, where tuberculosis is endemic, pulmonary findings may mimic Tuberculosis. A detailed history,
clinical examination and strong clinical suspicion along with radiological and biochemical markers form
the mainstay for diagnosis of Wegener’s granulomatosis.

KEY WORDS: Wegener’s Granulomatosis, ANCA, Proteinase-3, Vasculitis