|Year : 2019 | Volume
| Issue : 3 | Page : 85-88
Multiple vasculitic ulcers with lupus nephritis flare in pregnancy
Vijoy Kumar Jha1, KV Padmaprakash2, T Rajkamal3, Ajay Sharma4
1 Department of Nephrology, Command Hospital Air Force, Bangalore, Karnataka, India
2 Department of Medicine and GE Medicine, INHS Kalyani, Vishakhaptanam, Andhapradesh, India
3 Department of Dermatology, INHS Kalyani, Vishakhaptanam, Andhapradesh, India
4 Department of Pathology, INHS Kalyani, Vishakhaptanam, Andhapradesh, India
|Date of Submission||10-Jul-2018|
|Date of Acceptance||07-Aug-2019|
|Date of Web Publication||26-Sep-2019|
Dr. Vijoy Kumar Jha
Physician and Nephrologist, Department of Nephrology, Command Hospital Air Force, Old Air Port Road , Bangalore - 560 007, Karnataka
Source of Support: None, Conflict of Interest: None
Vasculitic ulcer in a case of systemic lupus erythematosus is considered as nonspecific cutaneous manifestations and those presenting with multiorgan involvement in pregnancy carry a higher maternal and fetal risk with disease flares. Presentation of a pregnant patient with severe lupus nephritis (LN) along with multiple cutaneous vasculitic ulcers is very uncommon. Aggressive treatment with immunosuppressants along with supportive treatment of cutaneous ulcerations will not only control the disease flares including LN but also result in complete healing of ulcerations.
Keywords: Antiphospholipid antibody syndrome, direct immunofluorescence, lupus nephritis, pulmonary arterial hypertension, systemic lupus erythematosus
|How to cite this article:|
Jha VK, Padmaprakash K V, Rajkamal T, Sharma A. Multiple vasculitic ulcers with lupus nephritis flare in pregnancy. Curr Med Issues 2019;17:85-8
| Introduction|| |
Pregnancy in women with systemic lupus erythematosus (SLE) carries a higher maternal and fetal risk and also disease flares due to distinguishing physiologic changes related to both pregnancy and disease-related manifestations. Cutaneous vasculitis results from inflammation of small- or medium-sized blood vessels and occurs in a wide variety of clinical settings. We present a case of a young female who was diagnosed SLE with lupus nephritis (LN) for the first time during her mid-trimester pregnancy when she also presented with multiple painful ulcerations over extremities and perineal region.
| Case Report|| |
A 25-year-old female married for the past 3 years with recent-onset amenorrhea of 21-week duration was admitted with a history of high-grade fever and perineal and left thigh swelling with small ulcerations of 7-day duration. She had a history of shortness of breath on exertion of 2-day duration. The patient had one episode of miscarriage at the 32nd week of gestation 2 years ago and had a frequent history of urosepsis since then. Further history revealed that she had nonspecific history of polyarthralgia, hair fall, oral ulcerations, photosensitivity, and rashes for the past 5 years. She was not evaluated in detail earlier and was treated with sulfasalazine, steroids, hydroxychloroquine (HCQ), and methotrexate on and off. Clinically on admission she was febrile, pulse was 102/min and regular, respiratory rate was 28/min, bloo d pressure was 160/100 mm Hg, bilateral lower limb pedal edema was present and there were multiple ulcerations over both thigh /legs and perineal region [Figure 1]. Initial investigations are shown in [Table 1]. A provisional diagnosis of SLE with 2nd-trimester pregnancy, severe pulmonary arterial hypertension (PAH), LN versus preeclampsia, and probable antiphospholipid antibody (APLA) syndrome with possibility of vasculitic ulcer was made. She was initially managed with intravenous (iv) antibiotics, iv methylprednisolone followed by oral steroids, proton-pump inhibitors, HCQ, low-molecular-weight heparin, antihypertensive, and other supportive measures. Skin biopsy was done to rule out the possibility of vasculitic ulcer and was suggestive of fibrin deposits in thickened blood vessel walls with intimal proliferation [Figure 2]. Direct immunofluorescence (DIF) skin biopsy revealed granular staining for IgA, IgG, C1q, C3, and kappa and lambda chains. Repeat obstetric ultrasonography after 1 week of admission revealed single live intrauterine fetus with 21 weeks 1 day gestation age, absent diastolic flow in uterine artery, and intrauterine growth retardation with amniotic fluid index 8. She had complete abortion on day 8 with abortus weight (320 g) and placenta weight (140 gm). Further evaluation after the abortion revealed urine RE/ME-Protein 3+, large number of red blood cell (RBC), 24-h urinary protein – 4.7 g, C3–26.4 (84–168) mg/dl, and C4 – <6.0 (15–50) mg/dl (methods – nephelometry and immunonephelometry). Serum protein - 4.8 g/dl, serum albumin - 1.9 g/dl, Na/K - 140/3.4 meq/L, lactate dehydrogenase - 316 IU/L (100–190), peripheral blood smear – spherocytes, schistocytes, polychromatophilic RBCs noted, APTT-C-28 s, prothrombin time (PT)-29 s, PT-control-13, PT-15.1 s, INR-1.16, serum cryoglobulins – negative, repeat two-dimensional (2D) echo revealed left ventricular ejection fraction – 60%, concentration left ventricular hypertrophy, Mild/Mod MR, RVSP-40 + RAP, minimal pericardial effusion, high-resolution computed tomography chest – normal lung parenchyma, MPA-40 mm, and bilateral minimal pleural effusion (rt > lt). In view of the possibility of lupus flare with LN, she was put on mycophenolate mofetil (MMF) and gradually dose was increased. Renal biopsy was done thereafter which revealed Class IV LN [Figure 3] and [Figure 4]. DIF renal biopsy revealed patchy granular staining for IgA, IgG, C1q, and kappa and lambda chains. Her iv antibiotics were switched to oral antibiotics, and other drugs, including MMF, HCQ, phosphodiesterase inhibitor, and steroids, were continued. She responded to the above treatment with gradual healing of ulcerations and nil proteinuria with normal renal function after 12 weeks of therapy. Her repeat 2D echo revealed a significant reduction in PAH.
|Figure 1: Showing skin ulceration with well-demarcated edge and secondary skin changes.|
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|Figure 2: Blood vessels in the dermis showing fibrin deposits (arrowhead) in the thickened blood vessel walls with intimal proliferation and neutrophils (arrows) and nuclear dust (H and E, ×400).|
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|Figure 3: Renal biopsy (MT stain) showing cellular crescents with tufts showing wire loops suggestive of Class IV lupus nephritis (×450).|
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|Figure 4: Renal biopsy (Silver stain) showing cellular crescents with lobular accentuations suggestive of Class IV LN (×400).|
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| Discussion|| |
Inflammatory ulcers are the result of vessel damage mediated by an immunological process that causes inflammation and destruction of both the vessels and the perivascular tissues. It is challenging for the physician to suspect, make diagnosis, and establish the etiology of impairment of cutaneous microcirculation or secondary causes for atypical or nonhealing wounds. Skin ulcers may be the initial sign of ischemia or evolutionary complications of cutaneous vasculitis. The presence of macrocirculatory disturbances or other comorbid conditions as well as long-term use of immunosuppressive drugs can enhance this evolutionary complication in the setting of connective tissue disorder. There is increased risk of hypercoagulability in patients with vasculitic ulcer, suggesting a strong relationship between clotting disorders and ulcerative complication in cutaneous vasculitis. The skin biopsy in this case done just after admission from the edge of ulcer revealed features suggestive of vasculitic ulcers with full-house positivity. In SLE, a careful and continuous study of the antiphospholipid immunity is always necessary and may require anticoagulation. She had possible secondary APLA syndrome in view of positive serology and significant history. In recent times, the prognosis of systemic vasculitis has markedly improved with the combinations of corticosteroids with cytotoxic drugs. Rituximab has been used with very good results in many systemic vasculitis. Lower-limb ulcers due to vasculitis secondary to SLE are extremely resistant to conventional treatment. These ulcers are often complicated with severe local or general infections which require aggressive broad-spectrum antibiotics and other supportive treatment for a long time. Effective use of immunosuppressive therapy, vasodilators, anticoagulants, wound debridement, and skin grafting if required will be appropriate treatment. In the index case, she was given corticosteroids along with MMF as induction therapy to suppress lupus flare and adequate antibiotic cover for secondary infection if any and she responded to these treatments remarkably. Another complex and challenging aspect during pregnancy with SLE is differentiation of preeclampsia and LN. Renal biopsy is a valuable tool for accurate characterization of LN, but due to technical difficulties of this procedure during pregnancy, it is usually avoided. The association of SLE and pregnancy, mainly with active LN, has poorer pregnancy outcomes, with increased frequency of preeclampsia, fetal loss, prematurity, growth restriction, and newborns small for gestational age. This patient had unsuccessful pregnancy with complete abortion, which may have contributed in reducing PAH and diagnosing LN during postabortion period by renal biopsy. This case stresses the fact that on the background of suspicion history of SLE with multiple skin ulcerations, definitive diagnosis of SLE with lupus flares and vasculitic ulcers is to be made. Nonspecific skin lesions such as alopecia, vasculitic lesions, and bullous lesions always indicate disease activity in SLE. Renal biopsy diagnosis of LN Class IV ruled out the possibility of preeclampsia or other glomerular diseases in this case. Treating LN with induction regimen with steroid/MMF not only controlled LN but also systemic flare including vasculitic ulcers.
| Conclusion|| |
Diagnosing and managing a case of SLE in pregnancy is very difficult. Although lupus flares with multiple skin ulcerations due to vasculitis are very uncommon, it should always be kept in mind while managing a patient with LN and other systemic flares. Aggressive management of the lupus flares with immunosuppressant will also control the vasculitis ulcers.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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