Class IV-G Research Articles

Successful Discontinuation of Glucocorticoid Treatment after Administration of a Calcineurin Inhibitor for Nephrotic Syndrome in a Patient With Lupus Nephritis: A Case Report.

Systemic lupus erythematosus was diagnosed in a patient at 43 years old. When proteinuria recurred at 57 years old, the first kidney biopsy was performed, and class IV-G (A) + V lupus nephritis was diagnosed. The prednisolone dose was increased to 40 mg/day, and cyclosporine A was introduced. After 1 year, proteinuria had decreased to 0.1 g/day. Prednisolone was discontinued three years later, and cyclosporine A was continued. Thereafter, proteinuria did not reoccur. At 67 years old, a second kidney biopsy showed complete remission of lupus nephritis. Cyclosporine A enabled permanent discontinuation of glucocorticoids in a patient with lupus nephritis.

Dissecting the histological features of lupus nephritis highlights new common patterns of injury in class III/IV

ObjectiveThe International Society of Nephrology/Renal Pathology Society classification is the gold standard for the characterisation of lupus nephritis (LN) on renal biopsy, with therapeutic repercussions. Its recent revision simplified the...

Hypotension intracrânienne : une cause rare de céphalées au cours du lupus systémique

IntroductionSpontaneous intracranial hypotension (SIH), a rare cause of headache, may be idiopathic or secondary, in particular to Systemic Lupus Erythematosus (SLE) where it remains exceptionally evoked or documented. Case reportA 36-year-old woman presented with postural headache, recurrent nausea and vomiting. The discovery of a nephrotic syndrome led to the diagnosis of SLE with lupus nephropathy (class IV-G-(A)). A brain MRI showed signs of intracranial hypotension with tonsil ptosis and a left parietal hypersignal, and leading to a diagnosis of neurolupus with SIH. Treatment with prednisone, cyclophosphamide, and then mycophenolate mofetil allowed a rapid complete response of all systemic, renal and neurological manifestations, including the iconographic signs of intracranial hypotension. ConclusionHeadaches are frequent and often unexplained during SLE. Their orthostatic character should, if appropriate, suggests a SIH and lead to perform a brain MRI, even in the absence of other neurological signs.

Eculizumab therapy on a patient with co\u2010existent lupus nephritis and C3 mutation\u2010related atypical haemolytic uremic syndrome: a case report

BackgroundThrombotic microangiopathy (TMA), a rare but serious complication of systemic lupus erythematosus (SLE), is associated with poor outcomes to conventional immunosuppressive therapy. Recently, eculizumab, a humanised monoclonal antibody that blocks the complement factor 5, has been known to effectively treat atypical haemolytic uremic syndrome (aHUS). Here, we report a case of aHUS co-existing with lupus nephritis that was successfully treated with eculizumab.Case presentationA 23-year-old man presented with abdominal pain and diarrhoea. Initial laboratory tests have shown thrombocytopaenia, microangiopathic haemolytic anaemia, and acute kidney injury. Immunologic tests were consistent with SLE. Kidney biopsy have revealed lupus nephritis class IV-G with TMA. Genetic analysis have shown complement C3 gene mutations, which hints the co-existence of lupus nephritis with aHUS, a form of complement-mediated TMA. Although initial treatment with haemodialysis, plasma exchange, and conventional immunosuppressive therapy (steroid and cyclophosphamide) did not appreciably improve kidney function and thrombocytopaenia, the patient was able to respond to eculizumab therapy.ConclusionsDue to the similar features of TMA and SLE, clinical suspicion of aHUS in patients with lupus nephritis is important for early diagnosis and prompt management. Timely administration of eculizumab should be considered as a treatment option for aHUS in lupus nephritis patients to yield optimal therapeutic outcomes.

EP16 Challenges in treating new onset systemic lupus erythematosus with lupus nephritis and COVID-19 infection overlap

Case report - IntroductionCOVID-19 infection caused by a novel coronavirus SARS-coV-2 has made the diagnosis and the treatment of inflammatory diseases incredibly challenging. On the one hand, because of its pro-inflammatory state, that may aggravate or trigger flares in autoimmune diseases such as systemic lupus erythematosus (SLE). On the other hand, the risk of an immunosuppressive therapy during the active phase SARS-coV-2 infection that may lead to catastrophic outcomes. We report a case of a 24-year-old female newly diagnosed with SLE during COVID-19 pandemic who developed COVID-19 infection during her induction treatment for lupus nephritis.Case report - Case descriptionA 24-year-old Nepali female, with no past medical history of note, presented to her regional hospital with a history of flu-like symptoms few days ago, peripheral oedema, acute kidney injury with proteinuria and hypertension. Further investigations showed a high titre of double-stranded DNA antibodies, anti-cardiolipin IgM and B2 microglobulin positive and low C3. She also developed a haemolytic anaemia and thrombocytopenia during her admission. She received pulsed steroid therapy and was started on mycophenolate mofetil (MMF) for a probable lupus nephritis awaiting the results of biopsy, which showed later a lupus nephritis Class IV-G with active lesions. She then developed symptoms of COVID-19 infection and had a positive PCR leading to an interruption of her induction therapy. She was recruited to the RECOVERY trial on the lopinavir-ritonavir arm and made a good recovery.Case report - DiscussionIt is well known that viruses can trigger or aggravate auto-immune response in patients predisposed genetically. However, the role of SARS-coV-2 is not elucidated yet. The EULAR COVID-19 registry showed that rheumatoid arthritis and SLE were the most prevalent rheumatic diseases, and there was an increased risk in those who are on moderate to high dose corticosteroids. In patients with SLE and COVID-19 infection, it is agreed by all the national and international rheumatology societies to interrupt their immunosuppressive therapy until the symptoms resolve, especially those with renal involvement or an active disease. Which is the case in our patient. Luckily, she resumed her MMF a month later after a negative PCR and her renal function has continued to improve.Case report - Key learning pointsLupus nephritis is a major risk factor for overall morbidity and mortality in SLE. It requires an early immunosuppressive treatment to induce remission.Randomized clinical trials showed that MMF is at least equally effective as cyclophosphamide in inducing remission and that it has been associated with a reduced risk of infection and amenorrhea. It seems to be a suitable alternative in women of childbearing age. In patients with concomitant COVID-19 disease, immunosuppressive therapy should be paused until the symptoms improve.

Lupus Nephritis Class IV-Global Is Associated with a Higher Risk of End-Stage Renal Disease than Class IV-Segmental

Introduction: The International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of lupus nephritis (LN) divides class IV into segmental and global (IV-S and IV-G) based on evidence suggesting different renal outcomes. However, subsequent studies have shown conflicting results. Objective: This study was performed to compare long-term renal outcomes between the IV-S and IV-G classes. Methods: This is a retrospective cohort study of adult patients with biopsy-proven class IV LN using the ISN/RPS classification. The primary end point was end-stage renal disease (ESRD). Results: Among the 89 patients, rapidly progressive glomerulonephritis was twice as frequent in the IV-G group (60 vs. 29%; p = 0.005) than that in the IV-S group. Moreover, the IV-G group had a higher rate of biopsy with cellular and fibrocellular crescents (70.9 vs. 47.1%, p = 0.024) and more crescentic glomerulonephritis (34.5 vs. 5.8%, p = 0.007) than the IV-S group. After a mean follow-up of 57 months, the IV-G group had a greater risk of ESRD (RR 3.9; 95% CI 1.2–12.2, p = 0.006) than the IV-S group. Multivariate analysis indicated that class IV-G was an independent predictor of ESRD. Conclusions: Patients with class IV-G have a higher risk of ESRD than patients with class IV-S.

ANCA positivity at the time of renal biopsy is associated with chronicity index of lupus nephritis.

We investigated the association of antineutrophil cytoplasmic antibody (ANCA) positivity with lupus nephritis (LN) activity, histological features and prognosis in Korean patients with biopsy-proven LN having the results of both myeloperoxidase (MPO-ANCA) and proteinase 3 (PR3)-ANCA. We retrospectively reviewed the medical records of 91 LN patients having the results of ANCA. We divided patients with LN into the two groups according to the ANCA positivity. We collected clinical and laboratory data at kidney biopsy and histological features such as LN class including class I, II, III, IV-S, IV-G and V, and activity and chronicity index. We evaluated prognosis of LN during the follow-up by death and kidney failure. Twelve of 91 patients (13.2%) had ANCA at kidney biopsy. There were no differences in demographic data, comorbidities, reasons for kidney biopsy and laboratory data at kidney biopsy between patients with and without ANCA. In 12 LN patients with ANCA, Class III was the most frequently observed LN class (41.7%), while in 79 LN patients without ANCA, class IV-G was the most often detected LN class (35.4%). There were no meaningful differences in classes of LN between the two groups. On the other hand, patients with ANCA exhibited the higher median chronicity index than those without (2.5 vs. 1.0, P = 0.028), unlike activity index. ANCA positivity exhibited no association with death or kidney failure during the follow-up. ANCA positivity at kidney biopsy is associated with chronicity index of LN.

Lupus nephritis with massive subendothelial deposits in a patient with autoimmune hepatitis-related cirrhosis

A 72-year-old Japanese woman with a history of autoimmune hepatitis-associated liver cirrhosis and portosystemic shunt was admitted to our hospital for evaluation of renal dysfunction, nephrotic range proteinuria, a high titer of anti-double-stranded DNA antibody, and hypocomplementemia. Renal biopsy showed massive subendothelial deposits (wire loop lesions), and class IV-G (A/C) lupus nephritis was diagnosed. Immunosuppressants, such as steroids (including intravenous methylprednisolone pulse therapy) and mycophenolate mofetil, could not prevent renal dysfunction. It was assumed that circulating immune deposits produced in the gastrointestinal tract entered the systemic circulation from the portal vein via the portosystemic shunt without hepatic clearance, resulting in massive subendothelial and mesangial accumulation compared to lupus nephritis patients without a shunt and causing renal injury.

294 Mycophenolate Mofetil Induction Therapy for Crescentic Lupus Nephritis Class IV-G: A Promising Outcome

294 Mycophenolate Mofetil Induction Therapy for Crescentic Lupus Nephritis Class IV-G: A Promising Outcome

Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices

We present a consensus report pertaining to the improved clarity of definitions and classification of glomerular lesions in lupus nephritis that derived from a meeting of 18 members of an international nephropathology working group in Leiden, Netherlands, in 2016. Here we report detailed recommendations on issues for which we can propose adjustments based on existing evidence and current consensus opinion (phase 1). New definitions are provided for mesangial hypercellularity and for cellular, fibrocellular, and fibrous crescents. The term "endocapillary proliferation" is eliminated and the definition of endocapillary hypercellularity considered in some detail. We also eliminate the class IV-S and IV-G subdivisions of class IV lupus nephritis. The active and chronic designations for class III/IV lesions are replaced by a proposal for activity and chronicity indices that should be applied to all classes. In the activity index, we include fibrinoid necrosis as a specific descriptor. We also make recommendations on issues for which there are limited data at present and that can best be addressed in future studies (phase 2). We propose to proceed to these investigations, with clinicopathologic studies and tests of interobserver reproducibility to evaluate the applications of the proposed definitions and to classify lupus nephritis lesions.

Lupus in fabula - hipertenzija mlađeg muškarca i sistemski lupus eritematozus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that predominantly affects women during the reproductive period (the ratio of female to male is 9 to 1). Although SLE can affect any organ, glomerulonephritis mediated by immune complexes lead to a sudden deterioration of the disease. The literature data indicate the role of chronic inflammation in the pathogenesis of arterial hypertension (HTA) in patients with SLE. This is a case study of a young man with a newly diagnosed HTA, with lupus nephritis behind. The data were obtained retrospectively, by analyzing the patient's medical record. A 40-year-old patient, with a two-year history of irregular and unsuccessful treatment of HTA with ACE inhibitor, saw his doctor for frequent headaches and elevated arterial blood pressure (TA) during self-measurement. By physical examination an auscultatively accelerated cardiac action was found, TA was 170/100 mmHg, and the ECG registered sinus tachycardia (about 100 bpm), while the rest of the finding was neat. Anamnestic data revealed that patient was recreational athlete, a non-smoker, and there was no positive family history of cardiovascular disease. Laboratory report of blood and urine was without significant deviation. Beta blocker and thiazide diuretic were used in the therapy. A month later lower leg edema appeared, followed by highly elevated TA values (up to 200/110 mmHg) and maculopapular rash. The patient was sent to the internist, and then hospitalized at the department of allergology. During the hospitalization, additional diagnostics was made. Laboratory finding reported sudden renal impairment and anemia (creatinine 144 mmol/L, urea 15.8 mmol/L, proteinuria 9.4 g/day, hemoglobin 106 g/L), and immunological analyzes indicated the positivity of SLE specific antibodies (ANA 1:160; anti-dsDNA 1:320). The patient was moved into the nephrology department where the treatment was initiated by the bolus of methylprednisolone (3x500mg). Under the control of ultrasound, a biopsy of the kidneys was performed, and the pathohistological finding showed lupus nephritis of class IV-G (A / C), activity index 17 and chronicity index 4, of the total of eight analyzed glomeruli one was globally sclerotic and in 3 glomeruli found crescent. Cyclophosphamide bolus therapy was started, and patient was released in a well general condition with prescribed corticosteroid and antihypertensive therapy - ACE inhibitor, calcium-channel blocker, Henle's loop diuretic, and beta blocker. Understanding the pathophysiology of HTA in this chronic autoimmune disease is of great clinical significance for patients suffering from SLE, as the cardiovascular disease is still the main cause of mortality with these patients.

Do ANCAs make the difference in lupus nephritis?

There has been much debate as to whether the distinction between lupus nephritis class IV-S and class IV-G is clinically relevant and whether antineutrophil cytoplasmic antibodies are pathomechanistically involved because they have been associated with the more necrotic and pauci-inflammatory phenotype of lupus nephritis that is more often seen in class IV-S. Recent data show that antineutrophil cytoplasmic antibody positivity indeed influences the histological pattern of lupus nephritis and is associated with worse baseline renal function and more active lupus serology.

Clinical and histological features of lupus nephritis in Japan: A cross-sectional analysis of the Japan Renal Biopsy Registry (J-RBR).

The clinical and histological features of lupus nephritis (LN) are highly variable, depending on race and ethnicity. The Japan Renal Biopsy Registry (J-RBR) is a nationwide registry of renal biopsies. Here, we report a cross-sectional analysis of Japanese LN using the J-RBR database. Out of 18 463 patients registered in the J-RBR, 331 LN patients, who received renal biopsy for the first time, were extracted and their clinical features were analyzed according to the ISN/RPS 2003 classification. The median age of the 331 LN patients was 37 years (women, 81.3%). The frequencies of each of the ISN/RPS Classes were as follows: I, 1.2%; II, 7.9%; III (±V), 25.1%; IV-S (±V), 13.0%; IV-G (±V), 31.1%; V, 20.8%; and VI, 0.9%. The level of proteinuria and the prevalence of nephrotic syndrome were highest for Class IV-G (±V). When Classes I, II, and VI were excluded from the analysis, Class IV-G (±V) was significantly associated with a lower eGFR and severer haematuria than the other classes. This nationwide study revealed that Class IV-G (±V) was the most prevalent form of LN in Japan and was associated with a severe clinical renal presentation.

111 A LUPUS NEPHRITIS PATIENT WITH BREATHLESSNESS FOLLOWING PLASMA EXCHANGE

Category: Other Presenter: Dr WAN HASNUL HALIMI WAN HASSAN Keywords: SLE, lupus nephritis, plasma exchange, fluid overload A 17 years old girl, diagnosed with Lupus nephritis since 2014. She had Lupus Nephritis class III , in partial remission following treatment with t.mycophenolate mofetil (MMF). She was transferred to high dependency ward requiring non invasive ventilation after 4th cycle of plasmapharesis. This is due to fluid overload and her condition improves after hemodialysis and ultrafiltration. She initially presented with symptoms of facial puffiness and leg swelling for 2 weeks. She was admitted was started on high dose prednisolone (60mg OD) and t.MMF 1g BD. Progressive worsening renal function was noted (urea 29/creat 190; baseline renal function was normal urea/creat) Renal biopsy results shows Crescentric Lupus Nephritis, ISN/RPS class IV-G (A/C). activity index 15/24, chronicity index 4/12. (57% cellular crescent, 15% global sclerosis). IV cyclophosphamide induction and plasma exchange was initiated. She completed total of 10 plasmapheresis cycle and is currently still ongoing 3rd cyclophosphamide induction. Her renal profile shows improvement (urea 15.7/creatinine 116). Plasma Exchange is a recognized modality of treatment in more severe and/or resistant lupus nephritis, moreover for crescentic glomerulonephritis. The complication rate of is not high. The Swedish registry reported no fatalities during 20,485 procedures, and an overall adverse incidence rate of only 4.3% of all exchanges (0.9% for severe adverse events) of which 27% were paresthesias, 19% transient hypotension, 13% urticaria, and 8% nausea. An overall complication rate of 1.4% has been reported in more than 15,000 treatments in patients receiving albumin, and 20% in patients receiving FFP. There's increases the risk of bleeding as a result of depletion of coagulation factors in patients receiving albumin as sole replacement colloid. A study in Japan looking at 53 kidney transplant recipients receiving plasma exchange for desensitization, recorded the were no severe adverse event, milder events were pruritus 53%, numbness 40% and mild dyspnea 5%. The incidence of numbness during plasma exchange was less in patients receiving combined hemodialysis. In our case, fluid overload was noted as a major complication requiring non invasive ventilation and hemodialysis. This is due to the large volume exchange and associated renal impairment. Although uncommon, fluid overload and other complication related to large volume plasma exchange is needed to be taken under account in a renal impaired patient.

Acute Kidney Injury, Recurrent Seizures, and Thrombocytopenia in a Young Patient with Lupus Nephritis: A Diagnostic Dilemma.

Introduction. Posterior reversible encephalopathy syndrome (PRES) is a constellation of clinical and radiologic findings. Fluctuations in blood pressure, seizures, and reversible brain MRI findings mainly in posterior cerebral white matter are the main manifestations. PRES has been associated with multiple conditions such as autoimmune disorders, pregnancy, organ transplant, and thrombotic microangiopathy (TMA). Case Presentation. A 22-year-old woman with history of Systemic Lupus Erythematous complicated with chronic kidney disease secondary to lupus nephritis class IV presented with recurrent seizures and uncontrolled hypertension. She was found to have acute kidney injury and thrombocytopenia. Repeat kidney biopsy showed diffuse endocapillary and extracapillary proliferative and membranous lupus nephritis (ISN-RPS class IV-G+V) and endothelial swelling secondary to severe hypertension but no evidence of TMA. Brain MRI showed reversible left frontal and parietal lesions that resolved after controlling the blood pressure, making PRES the diagnosis. Conclusion. PRES is an important entity that must be recognized and treated early due to the potential reversibility in the early stages. Physicians must have high suspicion for these unusual presentations. We present a case where performing kidney biopsy clinched the diagnosis in our patient with multiple confounding factors.

Tubulointerstitial lesions in lupus nephritis: International multicentre study in a large cohort of patients with repeat biopsy

The glomerulocentric International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification is the gold standard for the evaluation of lupus nephritis, while tubulointerstitial (TIN) parameters are often under-recognized in pathological reports. Renal biopsies from 142 patients who underwent repeat biopsy (RB) were evaluated for the following histological parameters: (i) inflammatory interstitial infiltrates; (ii) interstitial fibrosis; (iii) tubulitis; and (iv) tubular atrophy. The inter-relationships between the four TIN variables were explored by multivariate analysis. A linear mixed model was used to investigate the potential impact of TIN variables on eGFR and proteinuria at the two biopsy occasions. The study showed that moderate-severe lesions were not so frequent at the reference biopsy, but more extensively represented upon RB. A strong association was found between the two inflammatory indices and between those related to chronic damage, while the relationship with the ISN/RPS classification was present at RB. If class IV-G was the most related with TIN (especially at RB), the existence of primary TIN in class II patients was also confirmed. Finally, our results support the hypothesis that tubulitis is an independent predictive factor for eGFR. We recommend that the standard histological evaluation of SLE nephritis also includes TIN features.

Therapeutic effect of double-filtration plasmapheresis combined with methylprednisolone to treat diffuse proliferative lupus nephritis.

The efficacy of double-filtration plasmapheresis (DFPP), combined with methylprednisolone, to treat diffuse proliferative lupus nephritis (LN) was studied. Twenty-four patients who were admitted to the hospital and diagnosed with diffuse proliferative LN (LN Class IV-G(A)) through renal biopsy from 2011 to 2013 were recruited as the study subjects. The patients' clinical manifestations were nephritic syndrome and/or renal insufficiency. The pathological features were glomerular diffuse proliferative lesions. The patients were divided into two groups: the treatment group and the control group, with 12 patients in each group. The patients in the treatment group were first treated with DFPP combined with methylprednisolone (0.8-1.0 mg/kg/day); subsequently, they were put on methylprednisolone therapy only. The patients in the control group were first put on methylprednisolone pulse therapy (500-1,000 mg) for 3 days; subsequently, they were treated with methylprednisolone (0.8-1.0 mg/kg/day) combined with mycophenolate mofetil (1.5 g/day). The patients were observed for 24 months. Levels of hemoglobin, platelet, albumin, serum creatinine, 24-h urinary protein, serum C3 , antinuclear antibody (ANA), anti-dsDNA, and anti-Smith were measured at 0, 3, 6, 12, and 24 months. Complete remission and recurrence standards were established. The total dosages of methylprednisolone were calculated. Repeated renal biopsy was performed on several patients. There was no statistical significance in the baseline conditions of the treatment and the control groups. For the treatment group, no plasmapheresis-related complications occurred. The two groups showed no significant difference in complete remission. The patients' edema and serous effusion resolved, urine volume, serum creatinine, and albumin levels returned to normal, urine protein decreased in treatment group more rapidly than the patients in the control group. The mean dose of methylprednisolone received in the treatment group was lower than in the control group. The complement C3 levels in the treatment group were significantly higher than in the control group. The recurrence rate in the treatment group was lower than in the control group. Repeated renal biopsies on several patients in the treatment group indicated that their pathology improved significantly, changing from LN (IV) to LN(II-III). Appropriate application of DFPP combined with glucocorticoid therapy could accelerate the remission of diffuse proliferative LN, reduce overall glucocorticoid dosage, prevent recurrence, and maintain C3 level in a higher level. J. Clin. Apheresis 31:375-380, 2016. © 2015 Wiley Periodicals, Inc.

The clinico-pathological features of lupus nephritis and the significance of ISN/RPS-2003 Class IV lesions

Background: The ISN/RPS-2003 classification of lupus nephritis should emphasize clinically relevant lesions and encourage uniformity and reproducibility in histopathological reporting. Objective: To describe the clinico-pathological features of lupus nephritis and discuss the significance of the ISN/RPS-2003 class IV-G and IV-S lesions. Method: The histopathological features and corresponding clinical data of 75 patients with lupus nephritis were analysed using the International Society of Nephrology/Renal Pathology Society ISN/RPS - 2003 classification. This was a retrospective descriptive study carried out over a period of two and a half years at the Department of Pathology, Faculty of Medicine, Colombo. Results and conclusions: Lupus nephritis was commoner in females (88%, 66/75), with 52%, (36/75) in the 21-30 year age group. ANA positivity (93%, 70/75) was the commonest ACR (American College of Rheumatology - 1997) criterion to clinically diagnose SLE. Asymptomatic sub-nephrotic proteinuria was found in 47% (35/75), nephrotic syndrome in 21% (16/75) and hypertension in 17% ( 13/75). Endocapillary proliferation 84% (63/75) and wire-loop lesions 51% (38/75) were found to be the commonest histological features. 79% (59/75) had ISN/RPS class IV lupus nephritis (diffuse lupus nephritis) with the majority 93% (55/75) belonging to class IV-G (predominantly diffuse global lesions) and the remainder to class IV-S (predominantly segmental lesions). The pathogenesis of class IV-G lesions is thought to be immune complex mediated where as class 1V-S lesions are thought to show injury analogous to systemic vasculitides, unrelated to immunologic injury. Data on the prognostic outcomes of the two groups is conflicting because class IV-G is morphologically heterogenous, with two prognostically and pathogenetically distinct subcategories. Journal of Diagnostic Pathology 2014; 9(2): 14-22

Clinicopathological correlation in asian patients with biopsy-proven lupus nephritis.

A total of 244 patients with lupus nephritis (219 women (89.8%) with a female to male ratio of 9 : 1) were included in the study. Clinical and laboratory findings at renal biopsy are clinically valuable in identifying different renal classifications of lupus pathology, activity, and chronicity index. Patients with class IVG had significantly higher proportions of microscopic hematuria, proteinuria, hypertension, impaired renal function, anemia, hypoalbuminuria, and positive anti-DNA antibody. All of these findings correlated well with high activity index and chronicity index of lupus pathology. Considering these correlations may help to determine the clinicopathologic status of lupus patients.

Pulmonary hemorrhage as an unusual initial manifestation of systemic lupus erythematosus

Pulmonary hemorrhage as the initial manifestation of systemic lupus erythematosus (SLE) has been rarely reported in children. We present the case of a 10-year-old girl who was admitted to Kangbuk Samsung Hospital with hemoptysis. She had a 5-day history of cough with dyspnea. On physical exam, breath sound was significantly decreased combined with rales on both lung fields. Blood tests revealed pancytopenia, decreased complement levels (C3, 21.28 mg/dL; C4, 3.10 mg/dL), positive antinuclear antibody (>1:640) and anti-double-stranded DNA antibody (262.5 IU/mL). Chest computed tomography revealed patchy ground glass opacity on both lung fields. She had proteinuria and diffuse lupus nephritis (International Society of Nephrology/Renal Pathology Society class IV-G(A)) confirmed by renal biopsy. High-dose methylprednisolone pulse therapy (30 mg/kg/day) was given for 3 days and then switched to a maintenance dose (1 mg/kg/day). Initially hemoptysis resolved after administration of methylprednisolone, but recurred on the 14th day of treatment. She was then treated with cyclophosphamide pulse therapy and hemoptysis subsided without recurrence. She was discharged on the 31st day of admission. She continued to receive monthly cyclophosphamide pulse therapy until the occurrence of leukopenia and then her regimen was switched to mycophenolate and hydroxychloroquine. SLE continues to be well controlled after 18 months of treatment. Recognition of pulmonary hemorrhage as a possible initial manifestation of SLE is crucial for early diagnosis. SLE was successfully treated with good outcome. (Allergy Asthma Respir Dis 2015;3:370-374)