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2015 ; 4
(1
): 48-54
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Pulmonary hemorrhage associated with Henoch-Schöenlein purpura in an adult
patient primary diagnosed of IgA nephropathy
#MMPMID28509268
Tani T
; Arima R
; Kaneko T
; Hayashi H
; Tsuruta K
; Shimizu A
; Tsuruoka S
CEN Case Rep
2015[May]; 4
(1
): 48-54
PMID28509268
show ga
We describe a case of IgA nephropathy (IgAN) followed by pulmonary hemorrhage
associated with Henoch-Schöenlein purpura (HSP) in an adult female. The patient
had a history of renal insufficiency and persistent hematuria and proteinuria,
without any extra-renal involvement. She was diagnosed with IgAN 7 years before
the onset of HSP and had received immunosuppressive therapy for 6 years. One year
after discontinuing oral prednisolone and mizoribine, she suffered a pulmonary
hemorrhage. She presented with exacerbated urinary findings, and palpable
purpura, resulting in the diagnosis of HSP. Intravenous pulse methylprednisolone
followed by oral prednisolone (1 mg/kg/day) and a monthly intravenous
cyclophosphamide pulse resolved the pulmonary hemorrhage. In a review of 36 HSP
patients complicated with pulmonary hemorrhage, 27.8 % of the patients perished
[Rajagopala et al., Semin Arthritis Rheum 42:391-400, 1]. While the most
efficient therapeutic strategies for these patients have yet to be determined, we
speculate that an aggressive therapy of pulse methylprednisolone combined with
immunosuppression agents is likely to bring about the best outcome in cases with
pathological conditions similar to our patient's. On the other hand,
discontinuance of immunosuppressive therapy might have resulted in the
aggravation of the disease, hence we should examine patients carefully not to
miss the cue.