Scurvy-induced pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that results in precapillary pulmonary hypertension. PAH is caused by a group of clinical conditions involving multiple organ systems. Several cases have been reported in the literature demonstrating an association between vitamin C deficiency and PAH. Low endothelial nitric oxide levels in the pulmonary vasculature, combined with the inappropriate activation of hypoxia-inducible transcription factors, seen in patients with ascorbic acid deficiency, are believed to be the main contributors to the pathogenesis of pulmonary vasculopathy and the exaggerated pulmonary vasoconstrictive response seen in patients with scurvy-induced PAH. Vitamin C supplementation is considered the definitive treatment.

Vitamin C deficiency can lead to pulmonary hypertension: a systematic review of case reports

“…Of the 32 case reports, 26 reported cardiac features of PH: RV enlargement in 24 cases and septal flattening in 14 cases. Pericardial effusion was reported in 4 cases [ 79 , 90 , 99 , 103 ]. The P2 sound is common in PH [ 33 , 107 ] and was reported in 6 cases.…”

“…Of the 19 patients for whom a time point of observed benefit from vitamin C on symptoms or signs was reported, 12 described that there was substantial benefit within 4 days. In this calculation, we have made the interpretation that “rapidly” [ 76 ], “in a few days” [ 79 , 99 ], and “progressively” [ 77 ] indicate within 4 days.…”

“…According to current recommendation group 1 PAH classification is based on three criteria: mPAP > 20 mmHg, pulmonary wedge pressure < 15 mmHg and PVR > 2 WU [ 33 ]. Only 5 of the 10 proposed PAH cases demonstrated these criteria [ 78 , 79 , 82 , 86 , 96 ]. However, we noted 3 further cases satisfying the criteria [ 89 – 91 ].…”

“…Hospital stay usually includes a number of medical treatments, but the other treatments did not lead to sufficient improvement in the included cases. Within hospitals, several patients were administered standard PAH drugs such as prostacyclins [ 90 , 96 , 102 , 105 ], phosphodiesterase 5 inhibitors [ 78 , 79 , 82 , 89 , 90 , 96 , 98 , 102 , 103 , 105 ], endothelin receptor antagonist [ 87 , 103 , 105 ], inhaled NO [ 82 , 98 , 103 ], calcium channel blockers [ 93 , 94 , 100 ], phosphodiesterase 3 inhibitors [ 82 , 91 , 95 , 105 ], and nonspecified pulmonary vasodilator [ 100 ].…”