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Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature G Medina, D González-Pérez, C Vázquez-Juárez, M Sánchez-Uribe, MA Saavedra and LJ Jara Lupus published online 8 August 2014 DOI: 10.1177/0961203314546018 The online version of this article can be found at: http://lup.sagepub.com/content/early/2014/08/07/0961203314546018
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CASE REPORT
Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature G Medina1,5, D Gonza´lez-Pe´rez2, C Va´zquez-Jua´rez3, M Sa´nchez-Uribe4, MA Saavedra2,5 and LJ Jara3,5 1
Clinical Research Unit, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; 2Rheumatology Department, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; 3Direction of Education and Research, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; 4Pathology Department, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; and 5Universidad Nacional Auto´noma de Me´xico, Mexico City, Mexico
Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to lifethreatening. We report the case of a previously healthy 17-year-old woman with eight months’ history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40 C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients. Lupus (2014) 0, 1–4. Key words: Fulminant vasculitis; visceral vasculitis; systemic lupus erythematosus; active SLE
Introduction Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease of unknown etiology characterized by diversity of clinical manifestations with unpredictable course. Vasculitis is an inflammation of the vessel walls with a broad spectrum of clinical forms due to its capacity to affect vessels of different sizes and sites. Its prognosis may range from mild to life-threatening.1 The prevalence of vasculitis in large series of SLE patients varies between 11% and 20%.2 Cutaneous vasculitis is common in SLE, whereas visceral vasculitis is infrequent.3 Correspondence to: Luis J Jara, Hospital de Especialidades Centro Me´dico la Raza, Seris y Zaachila S/N Col. La Raza CP.02990 Mexico City, Mexico. Email: [email protected] Received 8 January 2014; accepted 8 July 2014
Likewise, involvement of two or more organs, systemic presentation and fatal complications of vasculitis in SLE patients are even more unusual.4 We describe the case of a young woman who developed, in a short period of time, symptoms and signs of active SLE and fulminant and fatal systemic vasculitis.
Case report A 17-year-old woman, previously healthy with unremarkable medical history, without history of medication or drug abuse, presented in August 2008, eight months before death, with arthralgias in proximal interphalangeal, metacarpophalangeal joints and wrists. She was treated by a private general physician with non-steroidal anti-inflammatory
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10.1177/0961203314546018
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(LUP)
[1–4] [PREPRINTER stage]
Fulminant vasculitis in SLE G Medina et al.
2
drugs with partial remission. In December 2008 she developed facial and lower limbs edema, myalgias and arthralgias, with spontaneous remission of the clinical manifestations in a few days. In April 2009, she developed fever, predominantly in the afternoon, of up to 40ºC, hair loss and severe epigastric abdominal pain, liquid stools with mucus and blood, rectal bleeding, hematemesis and blisters in left lower limb. Due to all these manifestations she was hospitalized in a second level hospital. During her hospitalization, she presented new blisters in left lower limb with discoloration, necrotic edges and decreased sensitivity. Urinalysis revealed albuminuria > 500mg/dl, 4–6 hyaline casts and creatinine clearance 26.79 ml/min. Abdominal ultrasound showed hepatomegaly. With active bullous SLE and a rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses. However, she did not improve and after one week of hospitalization, due to her critical condition, she was transferred to a tertiary level hospital. On initial physical examination, her blood pressure was 90/60 mmHg, pulse 156, respiratory frequency 45, conscious, afebrile, dehydrated, pale, with white patches in the oral mucosa, edema of abdominal wall, decreased peristalsis and ecchymosis on both flanks, lower limbs edema, left lower limb with an infected ulcer with necrotic edges approximately 12cm in length and the right one with painless bullous lesion of 18cm in length, and absence of pulses in the right upper extremity. SLE disease activity index (SLEDAI) of 36 points was documented. Supportive measures, hydrocortisone and antibiotics were initiated. The clinical condition deteriorated rapidly with a fall of her blood pressure to 53/36 mmHg. She had acute arterial insufficiency in the lower extremities,
respiratory failure with apnea, and metabolic acidosis (arterial blood gases: pH: 6.9; pCO2: 37; PO2: 21; HCO3: 7.6; SaO2: 12%), secondary to multiorgan failure requiring endotracheal intubation and advanced life support measures. Laboratory tests revealed pancytopenia: severe anemia (hemoglobin 7.5 g/dl), leukopenia (leukocytes 600 per mm3), neutropenia (neutrophils 251 per mm3), lymphopenia (lymphocytes 250 per mm3), thrombocytopenia (platelets 66,000 per mm3), consumptive coagulopathy (prothrombin time 36.2 s, International Normalized Ratio 4.54, partial thromboplastin time 156 s) and acute renal failure (creatinine 3.13 mg/dl). Six hours after admission, the patient developed cardiac arrest and died. We obtained permission to perform the autopsy and histological images publication, with the following findings: diffuse lupus nephritis, IV-B class (WHO 2003) with activity index 12/24 and chronicity index 1/24 (Figure 1(a) and (b)); myocarditis with transmural and periarterial lymphocytic arteritis (Figure 2); transmural vasculitis with lymphocytic infiltration in arteries (Figure 3); arterioles of the brain (Figure 4), wall of aortic, iliac and renal arteries; acute and chronic pericarditis with pericardial effusion of 300 ml; narrowed splenic arteries (onion skin-like), focal thickening and perivascular fibrosis and serositis of the small intestine and colon. We determined the cause of death as metabolic acidosis due to multi-organ failure, systemic vasculitis and active SLE. Immunologic assay results were: ANA 1:1280, coarse speckled pattern, anti-dsDNA 419 IU/ml (normal value: < 200 IU), hypocomplementemia C3 30.2 mg/dl (normal value: 80–200 mg/dl), C4 6.18 mg/dl (normal value: 10–50 mg/dl) anti-Sm 193 UI/ml, anti-RNP 196 UI/ml.
Figure 1 (a) Glomerulus with mild mesangial hypercellularity with neutrophils exudate, capillary loop thickening with wire loops (white arrow), hyaline thrombus that decreases capillary lumen (black arrow). Diffuse proliferative lupus nephropathy Class IV. HE stain: 40. (b) Glomerulus with thickened capillary loops. Fuchsinophilic deposits are observed in the subepithelial, subendothelial (black arrow) and mesangial loops. Trichromic of Masson stain 40. Lupus Downloaded from lup.sagepub.com by guest on September 10, 2014
XML Template (2014) [5.8.2014–11:36am] //blrnas3/cenpro/ApplicationFiles/Journals/SAGE/3B2/LUPJ/Vol00000/140156/APPFile/SG-LUPJ140156.3d
(LUP)
[1–4] [PREPRINTER stage]
Fulminant vasculitis in SLE G Medina et al.
3
Discussion
Figure 2 Intramyocardial arteritis with inflammatory infiltrates of lymphocytes without transmural necrosis of the intima or media (black arrow). HE stain 20.
Figure 3 Abdominal aorta with arteritis and infiltration of lymphocytes in the intima. (black arrow). HE stain 20.
Figure 4 Cerebral cortex with small arteries with infiltration of lymphocytes (lymphocytic arteritis without necrosis) (black arrow). HE stain 20.
We have described an unusual case of fulminant and fatal vasculitis in a young woman with active SLE of eight months’ duration. For the first four months she had mild SLE but the diagnosis was not suspected. In the following four months she had clinical evidence of systemic involvement (fever, arthritis and edema) but she remained without SLE diagnosis. Two weeks prior to hospitalization, she had sudden development of devastating complications of SLE with very active glomerulonephritis, serositis and skin necrosis. Finally, the clinical diagnosis of active SLE was made in the secondary level hospital, being treated without response. The patient arrived in the tertiary level hospital in this critical condition but she did not respond to intensive treatment and after six hours the patient died in the emergency room. The results of immunological studies confirmed the diagnosis of SLE, thus fulfilling the American College of Rheumatology classification criteria for SLE.5 Autopsy findings revealed diffuse systemic vasculitis and active glomerulonephritis. However, the abruptness of the clinical presentation and the fulminant course did not allow the implementation of other intensive treatment measures. Vasculitis in the setting of SLE may present different clinical courses. The broad spectrum of symptoms ranges from mild forms affecting cutaneous vessels to catastrophic forms with vasculitis within the internal organs and development of complications. SLE vasculitis is a secondary inflammatory process affecting arteries, veins and capillaries of various organs. It has been demonstrated that visceral vasculitis in SLE is rare and it was reported to occur in 6% to 18% of patients with SLE. It usually coincides with systemic flares and frequently follows or is associated with cutaneous vasculitis.3,6 Epidemiological data about vasculitis as a cause of death in SLE are scarce. RamosCasals et al.1 studied 670 SLE patients, of whom 76 presented vasculitis. Of these, 14 had visceral involvement in only one organ; there was no information about mortality. Jindal et al.,4 in an autopsy study, reported fatal complications of SLE in 25 patients; of these, nine cases presented vasculitis in a single organ and three patients in two or more organs with systemic vasculitis contributing to morbidity in one case. Zonana-Nacach et al.7 studied disease activity, damage and survival in 41 Mexican patients with SLE; of these, 16 died and vasculitis was reported in two patients. Another report described a SLE patient with active necrotizing Lupus
Downloaded from lup.sagepub.com by guest on September 10, 2014
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(LUP)
[1–4] [PREPRINTER stage]
Fulminant vasculitis in SLE G Medina et al.
4
cerebral vasculitis in whom autopsy findings revealed systemic vasculitis in small arteries of the gallbladder, colon, cervix and vasa vasorum of aorta.8 Our patient developed, in a short period of time, severe vasculitis in five organs, three of them compromising the patient’s life. Previous reports showed that patients with vasculitis had longer disease duration and younger age at onset of SLE. On the other hand, visceral vasculitis was associated with increased mortality regarding age of onset and nephropathy.9 In the present case the patient was very young and SLE attributed symptoms were of relatively recent onset. Visceral vasculitis and very active lupus nephritis contributed to mortality. According to the literature, as in this case, low serum complement level is indicative of increased complement deposition in vascular walls and other tissues, being a useful marker in the diagnosis of systemic vasculitis and active SLE. The presence of anti-Sm antibodies was previously associated with a much higher incidence of both cutaneous and visceral vasculitis, especially when it co-existed with anti-dsDNA antibodies, as happened in this case.6,10 Therefore, the patient had all the immunological manifestations that explain her fulminant and fatal clinical course. In this case it is very difficult to identify the factors that contributed to its fatal course. In this sense, the factors were delay in the diagnosis of SLE and late referral for prompt care by a rheumatologist. It is likely that the class IV glomerulonephritis clinical manifestations had started a few months before the presentation of fulminant vasculitis. In support of this point the patient had edema four months previous to admission, high creatinine levels and proteinuria on admission, and the histologic analysis demonstrated high activity index. In relation to treatment, other immunosuppressants, plasmapheresis and biological therapy were considered but, unfortunately, the devastating situation did not allow other treatments besides methylprednisolone pulses.
Conclusion In this exceptional case of systemic vasculitis, the fulminant course was the result of SLE high disease
activity and late diagnosis and treatment. This case suggests that visceral vasculitis of two or more organs has a poor prognosis. Early SLE diagnosis, and opportune referral to a rheumatologist for intensive treatment, can improve the outlook in these patients.
Funding This research received no specific grant from any funding agency in the public, commercial, or notfor-profit sectors.
Conflict of interest statement The authors have no conflicts of interest to declare.
References 1 Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in systemic lupus erythematosus: Prevalence and clinical characteristics in 670 patients. Medicine (Baltimore) 2006; 85: 95–104. 2 Vitali C, Bencivelli W, Isenberg DA, et al. Disease activity in systemic lupus erythematosus: Report of the Consensus Study Group of the European Workshop for Rheumatology Research I. A descriptive analysis of 704 European lupus patients. European Consensus Study Group for Disease Activity in SLE. Clin Exp Rheumatol 1992; 10: 527–539. 3 Toubi E, Kessel A, Bamberger E, Golan TD. Systemic lupus erythematosus vasculitis: A current therapeutic overview. Curr Treat Options Cardiovasc Med 2004; 6: 87–97. 4 Jindal B, Joshi K, Radotra BD, Banerjee AK. Fatal complications of systemic lupus erythematosus – an autopsy study from north India. Indian J Pathol Microbiol 2000; 43: 311–317. 5 Petri M, Orbai AM, Alarco´n GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012; 64: 2677–2686. 6 Cies´ lik P, Hrycek A, Kiucin´ski P. Vasculopathy and vasculitis in systemic lupus erythematosus. Pol Arch Med Wewn 2008; 118: 57–63. 7 Zonana-Nacach A, Yan˜ez P, Jime´nez-Balderas FJ, CamargoCoronel A. Disease activity, damage and survival in Mexican patients with acute severe systemic lupus erythematosus. Lupus 2007; 16: 997–1000. 8 Goel D, Reddy SR, Sundaram C, Prayaga AK, Rajasekhar L, Narsimulu G. Active necrotizing cerebral vasculitis in systemic lupus erythematosus. Neuropathology 2007; 27: 561–565. 9 Drenkard C, Villa AR, Reyes E, Abello M, Alarco´n-Segovia D. Vasculitis in systemic lupus erythematosus. Lupus 1997; 6: 235–242. 10 Cuellar MC, Espinoza LR. Laboratory testing in the evaluation and diagnosis of vasculitis. Curr Rheumatol Rep 2000; 2: 417–422.
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Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature G Medina, D González-Pérez, C Vázquez-Juárez, M Sánchez-Uribe, MA Saavedra and LJ Jara Lupus published online 8 August 2014 DOI: 10.1177/0961203314546018 The online version of this article can be found at: http://lup.sagepub.com/content/early/2014/08/07/0961203314546018
Published by: http://www.sagepublications.com
Additional services and information for Lupus can be found at: Email Alerts: http://lup.sagepub.com/cgi/alerts Subscriptions: http://lup.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav
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CASE REPORT
Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature G Medina1,5, D Gonza´lez-Pe´rez2, C Va´zquez-Jua´rez3, M Sa´nchez-Uribe4, MA Saavedra2,5 and LJ Jara3,5 1
Clinical Research Unit, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; 2Rheumatology Department, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; 3Direction of Education and Research, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; 4Pathology Department, Hospital de Especialidades Centro Me´dico La Raza IMSS, Mexico City, Mexico; and 5Universidad Nacional Auto´noma de Me´xico, Mexico City, Mexico
Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to lifethreatening. We report the case of a previously healthy 17-year-old woman with eight months’ history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40 C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients. Lupus (2014) 0, 1–4. Key words: Fulminant vasculitis; visceral vasculitis; systemic lupus erythematosus; active SLE
Introduction Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease of unknown etiology characterized by diversity of clinical manifestations with unpredictable course. Vasculitis is an inflammation of the vessel walls with a broad spectrum of clinical forms due to its capacity to affect vessels of different sizes and sites. Its prognosis may range from mild to life-threatening.1 The prevalence of vasculitis in large series of SLE patients varies between 11% and 20%.2 Cutaneous vasculitis is common in SLE, whereas visceral vasculitis is infrequent.3 Correspondence to: Luis J Jara, Hospital de Especialidades Centro Me´dico la Raza, Seris y Zaachila S/N Col. La Raza CP.02990 Mexico City, Mexico. Email: [email protected] Received 8 January 2014; accepted 8 July 2014
Likewise, involvement of two or more organs, systemic presentation and fatal complications of vasculitis in SLE patients are even more unusual.4 We describe the case of a young woman who developed, in a short period of time, symptoms and signs of active SLE and fulminant and fatal systemic vasculitis.
Case report A 17-year-old woman, previously healthy with unremarkable medical history, without history of medication or drug abuse, presented in August 2008, eight months before death, with arthralgias in proximal interphalangeal, metacarpophalangeal joints and wrists. She was treated by a private general physician with non-steroidal anti-inflammatory
! The Author(s), 2014. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav
Downloaded from lup.sagepub.com by guest on September 10, 2014
10.1177/0961203314546018
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(LUP)
[1–4] [PREPRINTER stage]
Fulminant vasculitis in SLE G Medina et al.
2
drugs with partial remission. In December 2008 she developed facial and lower limbs edema, myalgias and arthralgias, with spontaneous remission of the clinical manifestations in a few days. In April 2009, she developed fever, predominantly in the afternoon, of up to 40ºC, hair loss and severe epigastric abdominal pain, liquid stools with mucus and blood, rectal bleeding, hematemesis and blisters in left lower limb. Due to all these manifestations she was hospitalized in a second level hospital. During her hospitalization, she presented new blisters in left lower limb with discoloration, necrotic edges and decreased sensitivity. Urinalysis revealed albuminuria > 500mg/dl, 4–6 hyaline casts and creatinine clearance 26.79 ml/min. Abdominal ultrasound showed hepatomegaly. With active bullous SLE and a rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses. However, she did not improve and after one week of hospitalization, due to her critical condition, she was transferred to a tertiary level hospital. On initial physical examination, her blood pressure was 90/60 mmHg, pulse 156, respiratory frequency 45, conscious, afebrile, dehydrated, pale, with white patches in the oral mucosa, edema of abdominal wall, decreased peristalsis and ecchymosis on both flanks, lower limbs edema, left lower limb with an infected ulcer with necrotic edges approximately 12cm in length and the right one with painless bullous lesion of 18cm in length, and absence of pulses in the right upper extremity. SLE disease activity index (SLEDAI) of 36 points was documented. Supportive measures, hydrocortisone and antibiotics were initiated. The clinical condition deteriorated rapidly with a fall of her blood pressure to 53/36 mmHg. She had acute arterial insufficiency in the lower extremities,
respiratory failure with apnea, and metabolic acidosis (arterial blood gases: pH: 6.9; pCO2: 37; PO2: 21; HCO3: 7.6; SaO2: 12%), secondary to multiorgan failure requiring endotracheal intubation and advanced life support measures. Laboratory tests revealed pancytopenia: severe anemia (hemoglobin 7.5 g/dl), leukopenia (leukocytes 600 per mm3), neutropenia (neutrophils 251 per mm3), lymphopenia (lymphocytes 250 per mm3), thrombocytopenia (platelets 66,000 per mm3), consumptive coagulopathy (prothrombin time 36.2 s, International Normalized Ratio 4.54, partial thromboplastin time 156 s) and acute renal failure (creatinine 3.13 mg/dl). Six hours after admission, the patient developed cardiac arrest and died. We obtained permission to perform the autopsy and histological images publication, with the following findings: diffuse lupus nephritis, IV-B class (WHO 2003) with activity index 12/24 and chronicity index 1/24 (Figure 1(a) and (b)); myocarditis with transmural and periarterial lymphocytic arteritis (Figure 2); transmural vasculitis with lymphocytic infiltration in arteries (Figure 3); arterioles of the brain (Figure 4), wall of aortic, iliac and renal arteries; acute and chronic pericarditis with pericardial effusion of 300 ml; narrowed splenic arteries (onion skin-like), focal thickening and perivascular fibrosis and serositis of the small intestine and colon. We determined the cause of death as metabolic acidosis due to multi-organ failure, systemic vasculitis and active SLE. Immunologic assay results were: ANA 1:1280, coarse speckled pattern, anti-dsDNA 419 IU/ml (normal value: < 200 IU), hypocomplementemia C3 30.2 mg/dl (normal value: 80–200 mg/dl), C4 6.18 mg/dl (normal value: 10–50 mg/dl) anti-Sm 193 UI/ml, anti-RNP 196 UI/ml.
Figure 1 (a) Glomerulus with mild mesangial hypercellularity with neutrophils exudate, capillary loop thickening with wire loops (white arrow), hyaline thrombus that decreases capillary lumen (black arrow). Diffuse proliferative lupus nephropathy Class IV. HE stain: 40. (b) Glomerulus with thickened capillary loops. Fuchsinophilic deposits are observed in the subepithelial, subendothelial (black arrow) and mesangial loops. Trichromic of Masson stain 40. Lupus Downloaded from lup.sagepub.com by guest on September 10, 2014
XML Template (2014) [5.8.2014–11:36am] //blrnas3/cenpro/ApplicationFiles/Journals/SAGE/3B2/LUPJ/Vol00000/140156/APPFile/SG-LUPJ140156.3d
(LUP)
[1–4] [PREPRINTER stage]
Fulminant vasculitis in SLE G Medina et al.
3
Discussion
Figure 2 Intramyocardial arteritis with inflammatory infiltrates of lymphocytes without transmural necrosis of the intima or media (black arrow). HE stain 20.
Figure 3 Abdominal aorta with arteritis and infiltration of lymphocytes in the intima. (black arrow). HE stain 20.
Figure 4 Cerebral cortex with small arteries with infiltration of lymphocytes (lymphocytic arteritis without necrosis) (black arrow). HE stain 20.
We have described an unusual case of fulminant and fatal vasculitis in a young woman with active SLE of eight months’ duration. For the first four months she had mild SLE but the diagnosis was not suspected. In the following four months she had clinical evidence of systemic involvement (fever, arthritis and edema) but she remained without SLE diagnosis. Two weeks prior to hospitalization, she had sudden development of devastating complications of SLE with very active glomerulonephritis, serositis and skin necrosis. Finally, the clinical diagnosis of active SLE was made in the secondary level hospital, being treated without response. The patient arrived in the tertiary level hospital in this critical condition but she did not respond to intensive treatment and after six hours the patient died in the emergency room. The results of immunological studies confirmed the diagnosis of SLE, thus fulfilling the American College of Rheumatology classification criteria for SLE.5 Autopsy findings revealed diffuse systemic vasculitis and active glomerulonephritis. However, the abruptness of the clinical presentation and the fulminant course did not allow the implementation of other intensive treatment measures. Vasculitis in the setting of SLE may present different clinical courses. The broad spectrum of symptoms ranges from mild forms affecting cutaneous vessels to catastrophic forms with vasculitis within the internal organs and development of complications. SLE vasculitis is a secondary inflammatory process affecting arteries, veins and capillaries of various organs. It has been demonstrated that visceral vasculitis in SLE is rare and it was reported to occur in 6% to 18% of patients with SLE. It usually coincides with systemic flares and frequently follows or is associated with cutaneous vasculitis.3,6 Epidemiological data about vasculitis as a cause of death in SLE are scarce. RamosCasals et al.1 studied 670 SLE patients, of whom 76 presented vasculitis. Of these, 14 had visceral involvement in only one organ; there was no information about mortality. Jindal et al.,4 in an autopsy study, reported fatal complications of SLE in 25 patients; of these, nine cases presented vasculitis in a single organ and three patients in two or more organs with systemic vasculitis contributing to morbidity in one case. Zonana-Nacach et al.7 studied disease activity, damage and survival in 41 Mexican patients with SLE; of these, 16 died and vasculitis was reported in two patients. Another report described a SLE patient with active necrotizing Lupus
Downloaded from lup.sagepub.com by guest on September 10, 2014
XML Template (2014) [5.8.2014–11:36am] //blrnas3/cenpro/ApplicationFiles/Journals/SAGE/3B2/LUPJ/Vol00000/140156/APPFile/SG-LUPJ140156.3d
(LUP)
[1–4] [PREPRINTER stage]
Fulminant vasculitis in SLE G Medina et al.
4
cerebral vasculitis in whom autopsy findings revealed systemic vasculitis in small arteries of the gallbladder, colon, cervix and vasa vasorum of aorta.8 Our patient developed, in a short period of time, severe vasculitis in five organs, three of them compromising the patient’s life. Previous reports showed that patients with vasculitis had longer disease duration and younger age at onset of SLE. On the other hand, visceral vasculitis was associated with increased mortality regarding age of onset and nephropathy.9 In the present case the patient was very young and SLE attributed symptoms were of relatively recent onset. Visceral vasculitis and very active lupus nephritis contributed to mortality. According to the literature, as in this case, low serum complement level is indicative of increased complement deposition in vascular walls and other tissues, being a useful marker in the diagnosis of systemic vasculitis and active SLE. The presence of anti-Sm antibodies was previously associated with a much higher incidence of both cutaneous and visceral vasculitis, especially when it co-existed with anti-dsDNA antibodies, as happened in this case.6,10 Therefore, the patient had all the immunological manifestations that explain her fulminant and fatal clinical course. In this case it is very difficult to identify the factors that contributed to its fatal course. In this sense, the factors were delay in the diagnosis of SLE and late referral for prompt care by a rheumatologist. It is likely that the class IV glomerulonephritis clinical manifestations had started a few months before the presentation of fulminant vasculitis. In support of this point the patient had edema four months previous to admission, high creatinine levels and proteinuria on admission, and the histologic analysis demonstrated high activity index. In relation to treatment, other immunosuppressants, plasmapheresis and biological therapy were considered but, unfortunately, the devastating situation did not allow other treatments besides methylprednisolone pulses.
Conclusion In this exceptional case of systemic vasculitis, the fulminant course was the result of SLE high disease
activity and late diagnosis and treatment. This case suggests that visceral vasculitis of two or more organs has a poor prognosis. Early SLE diagnosis, and opportune referral to a rheumatologist for intensive treatment, can improve the outlook in these patients.
Funding This research received no specific grant from any funding agency in the public, commercial, or notfor-profit sectors.
Conflict of interest statement The authors have no conflicts of interest to declare.
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