gms | German Medical Science

Leading symptom during disease manifestation: We present a case of a previously healthy 17-year old male who presented with fever for 6 days, mild cough, arthritis (elbows, knees, ankle), hematuria, petechial hemorrhages in fingers and lower extremities.

Diagnostics and therapy: SARS-COV2 PCR was positive. High inflammatory parameters, pulmonary infiltrates on X-ray and clinical improvement to antibiotics suggested pneumonia. However, the patient developed hemoptysis and life- threatening diffuse alveolar hemorrhage (DAH), seen on bronchoscopy. PR3-ANCAs were highly positive, eosinophils were max. 1,35 *109/l. Diagnosis of SARS-COV2-triggered granulomatosis with polyangiitis (GPA) was made. Despite of intensive treatment with methylprednisolone pulses, cyclophosphamide, rituximab and avacopan the patient needed intensive care with mechanical ventilation and blood transfusions. Renal biopsy was conducted due to increasing creatinine and showed pauci-immune necrotizing glomerulonephritis. The patient slowly stabilized. He was dismissed with prednisolone, avacopan, angiotensin-II-receptor antagonist and thiazid diuretics.

Discussion: GPA is an ANCA associated vasculitis with incident rates in children and adolescents from 0.4 to 6.4/1million inhabitants per year. Our patient does fulfill the EULAR/PRINTO/PRES classification criteria [1] for GPA, with (clinical) small vessel vasculitis, ANCA positivity, lung and kidney involvement. The high eosinophilic count is remarkable, but criteria are in favor of GPA, not of EGPA (no obstructive airway disease, no nasal polyps). Diffuse alveolar hemorrhage (DAH) is a possible complication of GPA. The typical trias of clinical symptoms are anemia, hemoptysis (60% of patients) and respiratory failure, on Xray diffuse pulmonary infiltrates can be seen [2]. Bronchoscopy should be urgently performed to make the diagnosis, since DAH in GPA patients requires immediate immunosuppressive treatment. Mortality rate of DAH is reported as high as 20 to 50% in adult GPA patients. Additionally, our patient had an acute SARS COV2 infection, which has been reported as trigger for ANCA associated vasculitis before [3], [4].

Conclusion:

1.

SARS COV2 infection can be a trigger for granulomatosis with polyangiitis (GPA).

2.

Chest Xray can´t differentiate between infectious or hemorrhagic infiltrates. CT is helpful, but bronchoscopy is needed to make the diagnosis.

3.

Diffuse alveolar hemorrhage (DAH) is a life- threatening complication of GPA. The typical clinical trias is anemia, hemoptysis and respiratory failure. It requires immediate immunosuppressive therapy.