Case Presentation: A 14 y.o. male with acne presented to dermatology for follow up. Having failed multiple acne treatments he was started on isotretinoin. Approximately 6 weeks later he developed fatigue, non-purulent conjunctivitis, retrosternal chest pain associated with dyspnea, joint pains, and bilateral lower extremity muscle aches. He was evaluated by cardiology with normal cardiac workup but was found to have elevated ESR, CRP, and RF. Isotretinoin was stopped after which his symptoms improved. However, shortly thereafter he developed fatigue, generalized weakness, fever, pallor, cough, recurrence of chest pain, flank pain, tea colored urine, and recurrence of leg pain and so presented to our ED.In the ED he was mildly tachycardic with noted pallor. Labs were remarkable for significant anemia, and elevated Creatinine (Cr), CK, and CRP. UA showed trace protein and large amount of blood. A renal US showed bilaterally enlarged kidneys. He was admitted to our hospital for further management.
Various subspecialties were consulted including Pulmonology, Nephrology, and Rheumatology. He received a CT of the chest which revealed large areas of ground glass attenuation consistent with pulmonary hemorrhage. His Cr continued to trend up with repeat UA showing RBC casts, this led to a renal biopsy which showed necrotizing/crescentic glomerulonephritis without significant immune complex deposition. Further lab testing revealed an elevated anti-proteinase 3 (anti-PR3).
Based on the history of lung and renal involvement, positive antineutrophil cytoplasmic antibodies (ANCA), and results of the renal biopsy a diagnosis of Granulomatosis with Polyangitis (GPA) was made. He was started on treatment with steroids and rituximab after which symptoms gradually improved.
Discussion: GPA is one of the ANCA associated small vessel vasculitidies. Primary systemic vasculitidies are rare in children, but GPA is one of the most common vasculitidies seen in the pediatric population. GPA has a variable presentation but most commonly presents with constitutional symptoms and some variation of ENT, pulmonary, renal, cutaneous and ophthalmic involvement. It is diagnosed based on clinical findings, positive ANCA (in particular anti-PR3), and confirmed with a biopsy of an affected organ. The treatment consists of immunosuppressive therapy.
Risk factors for the development of GPA are not well known, but there have been multiple drugs associated with the development of GPA. Isotretinoin is one of them and was the likely trigger in the case above.
Conclusions: GPA has significant morbidity and mortality if left untreated, and should be considered in any patient with clinical findings suggestive of GPA and recent use of one of the known offending drugs. Treatment can be initiated based on the clinical findings and positive ANCA. Biopsy should be performed as soon as possible to confirm the diagnosis, but should not delay treatment in serious cases.