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A B S T R A C T
Background: Wegener's granulomatosis is a very rare long-term systemic
disorder, in which granuloma formation occurs and inflammation of blood
vessels (vasculitis). The cause of disorder is not yet known, but genetic
factors are thought to play an important role. Clinical symptoms are often
similar to other disorders, making diagnosis difficult. However, early
diagnosis is very important in order to provide effective management.
Objective: Diagnosis and management in a rare case of Wegener’s
granulomatosis vasculitis, especially it was found with other comorbidities.
Methods: This case report showed a-38-years-old-man that came to the
emergency room of Dr Moewardi Hospital with complaints of swelling
accompanied by redness and stiff on the face, hands and feet that worsening
since 7 days ago. He also complained of fever fluctuating, nasal congestion
accompanied by clear discharge and sometimes hearing loss in the right
ear. Since the last 3 months, he was often experience similar complaints.
History of diabetes was recognized by the patient for 5 years, but he did not
regularly take medication. Results: In this case, examination of vital signs
within normal limits. Physical examination revealed a saddle nose with clear
discharge, swelling and redness around the face, hands and feet. Laboratory
tests showed hemoglobin 12.9 g/dl, HbA1c 8.4%, sodium level 128 mmol/L,
potassium level 3.1 mmol/L, calcium level 1.12 mmol/L. The Anti -
Neutrophil Cytoplasmic Antibodies (ANCAs) and Anti Nuclear Antibody
(ANA) Indirect Immunofluorescence (IF) method were negative.
Electrocardiogram and chest x-ray examination within normal limits.
Histopathological examination revealed epidermal atrophy and multiple
granulomas of the dermis. The patient underwent treatment for 10 days
with tappering-off dose steroid, immunosuppressants, insulin, calcium,
and potassium preparations therapy. Conclusions: Wegener’s
granulomatosis vasculitis is a rare case. Prompt and accurate diagnosis and
management will prevent poor progression of them, especially it was found
with other comorbidities.