A fatal pulmonary complication of lupus in pregnancy

Abstract

Pulmonary vasculitis is an uncommon complication of systemic lupus erythematosus (SLE), and has not been previously documented in pregnancy. This case report describes the rapidly fatal course of a pregnant SLE patient, whose terminal illness was manifested by severe pulmonary hypertension and unexpected autopsy findings of medium and large vessel vasculitis. The relationship of these unusual manifestations to other clinical and serologic features of SLE, as well as novel therapeutic options, are discussed.Pulmonary involvement is reported to occur in up to 50% of patients with systemic lupus erythematows (SLE) at some time during the course of their illness (1). Pleuritis is the most frequent presenting complaint, yet pulmonary parenchymal involvement is uncommon. Pulmonary hypertension has been documented since 1981 (2-5) and is often associated with Raynaud's phenomenon, digital vasculitis, and livedo (3,4). The diagnosis of pulmonary hypertension is frequently delayed, and the development of this complication is not necessarily related to the severity of the overall lupus activity; therefore, a high index of suspicion is required (4). Response to standard therapy is generally unpredictable and often disappointing (4,6).The case presented here is that of a pregnant woman with SLE and pulmonary hypertension, whose course was complicated by the development of severe pulmonary vasculitis. It is impossible to determine with certainty whether this latter complication was directly attributable to the patient's underlying SLE or was somehow precipitated by the terminal phases of her pregnancy. In view of the generally unsatisfactory

Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus

“…PAH has been reported as a common cause of death in Chinese and Korean patients, but not a common cause of death in North American or European cohorts [3], suggesting that ethnicity or access to care may be prognostic factors for survival. Our recent systematic review of the literature [90] identified a number of clinical factors that may be associated with survival; magnitude of the elevation in P pa at diagnosis [78,89,91], thrombosis [89,92,93], thrombocytopenia [94], presence of an anti-cardiolipin antibody [89,92,93], concurrent pregnancy [95,96], infection [77], Raynaud's phenomenon [93,97], plexiform lesion [95] and pulmonary vasculitis [96]. Conversely, lupus central nervous system disease [89], lupus nephritis [89] and lupus disease activity [89,[98][99][100] have not been associated with survival in SLE-PH.…”

Pulmonary Hypertension in Systemic Lupus Erythematosus

“…25,[30][31][32] Findings include acute fibrinoid necrosis and vasculitis, as well as chronic intimal fibrosis, medial hypertrophy, alteration of elastic laminae, periadventitial fibrosis, aneurysmal dilation, and plexiform lesions, which are virtually identical to the alterations seen in patients with IPAH. 9,17,29 These changes occurred in arteries, arterioles, and veins. Occasional cases with thrombotic arteriopathy have also been reported and were found to correlate with a hypercoagulable status, including positive lupus anticoagulant and anticardiolipin antibodies.…”

“…38,39 In addition, when antiphospholipid antibodies bind to the phospholipids on the endothelial surface, there is resultant in-situ thrombosis and the release of soluble mediators and subsequent vascular injury. 9,37 Antiendothelial cell antibodies may also play a key pathogenic role in the development of SLE-PH. Systemic lupus erythematosus is an autoimmune disease characterized by polyclonal B cell activation.…”

“…Pulmonary hypertension has also been associated with pulmonary vasculitis with or without alveolar hemorrhage. [7][8][9] Prevalence, Demographics, and Risk Factors Studies of patients with SLE have found a prevalence of PH ranging from 0.5% to 43%, although the degree of PH is typically modest. [10][11][12][13][14][15][16] The prevalence varied based on the method used for detection, and many patients in these reports either had significant restrictive lung disease or data reported were inadequate to determine the etiology of the PH.…”

Pulmonary Hypertension in Parenchymal Lung Disease

“…Several factors are incriminated for the induction of SLE-PH, such as hypoxic vasoconstriction, pulmonary venous hypertension resulting from left heart disease, antiphospholipid antibody-induced chronic or acute thrombosis, and pulmonary venoocclusive disease (PVOD) [83–88]. There are several pathological similarities in SLE-PH and IPAH, including SMCs hypertrophy, hyperactivation of transcription factors like hypoxia inducible factor-1 alpha and nuclear factor of activated T-lymphocytes, decreased expression of certain voltage-gated potassium channels, and de novo expression of antiapoptotic proteins [89].…”