A Diagnosis Missed for Several Years- Wegener’s Granulomatosis

Abstract

Wegener’s granulomatosis is a form of systemic vasculitis of small to medium sized vessels and affects upper respiratory tract, lungs and kidneys along with various organs. It causes necrotizing granulomatous inflammation of the affected parts and presents with positive antineutrophil cytoplasmic antibodies in more severe forms. Being a systemic disease with the potential to affect any organ-systems with a wide range of clinical presentations, it is associated with a risk of delay in diagnosis with resultant setback in institution of appropriate treatment. Confusion may arise due to an extent of histological similarity between Wegener’s granulomatosis and the more prevalent tuberculosis, both causing granulomatous inflammation of the affected parts. Here, we present two cases of this rare disorder where the diagnosis was missed for several years in the beginning causing a delay in institution of specific therapy which led to the development of complications.DOI: http://dx.doi.org/10.3126/kumj.v9i3.6309 Kathmandu Univ Med J 2011;9(3):218-21

Granulomatosis with Polyangiitis Presenting as Pyrexia of Unknown Origin, Leukocytosis, and Microangiopathic Haemolytic Anemia

“…Fever suggestive of an infectious aetiology is not uncommon in GPA. Several case reports describe fever and pulmonary symptoms with abnormal chest imaging studies (often initially interpreted to be pneumonia), as in our patient's diagnosis from her prior admission [ 6 – 8 ]. Tuberculosis was suspected in our patient on both hospitalizations due to exposure risks in Haiti and in her role as a healthcare worker, along with the presence of a positive tuberculin skin test.…”

Clinical characteristics and outcome of vasculitides

“…In Western Nepal, only one case of vasculitis was observed in a series of 365 rheumatic cases. 3 Except few case reports and small series 4,5 on vasculitis, there is lack of data on clinical presentation and outcome locally. This study aims to assess clinical characteristics and outcome of vasculitic diseases from a tertiary care teaching hospital in Kathmandu, Nepal.…”