A recent study has found that thrombosis may be the first manifestation of granulomatosis with polyangiitis disease in children following the very first report of its presentation in a 13 year old child.
Thrombosis
Thrombosis can happen within the veins or the arteries. However, between the two, arterial thrombosis is much more common in children. Venous thrombosis, while much less common in adolescents, can lead to much more severe effects. Thrombosis is a rare indication of GPA.
Granulomatosis with polyangiitis (GPA), which used to be called Wegener’s granulomatosis, is a rare type of chronic vasculitis. It typically does not lead to thrombosis.
The Study
This study analyzed a clinical case where a young girl who was 13 years old presented in the hospital with pain and swelling in her left arm. Her left arm was 5cm larger than her right. For the past 3 months she had dealt with fatigue, coughs, and bloody noses.
The child was diagnosed with GPA.
A Doppler as well as compression ultrasonography were utilized. These showed that she had thrombosis in the left brachial and axillary vein. Further testing showed –
- Alveolar hemorrhage
- Pansinusitis
- Hematuria
- Positive cANCA
- Proteinuria
Her coagulopathy tests were normal.
The patient was started on IV enoxaparin as well as pulse methylprednisolone. Then, she was given IV cyclophosphamide. She was discharged after 5 days and moved to oral enoxaparin and prednisolone.
A one week check up showed that her axillary and left brachial veins were normal. However, she continued on treatment for 3 months.
Researchers explain that thrombosis is likely the first indication of GPA in children as it occurred in this case before inflammation was controlled in the first stage of GPA. Future research is needed, however this study provides important insights that will be essential to consider moving forward.
You can read more about this investigation here.
