Transient panhypogammaglobulinaemia and B-lymphocyte deficiency in a patient with neuropsychiatric systemic lupus erythematosus after immunosuppressive therapy

Panhypogammaglobulinaemia is a rare complication of systemic lupus erythematosus (SLE), but its cause and mechanism are unclear. We observed transient panhypogammaglobulinaemia in a patient with neuropsychiatric SLE after treatment with prednisolone and cyclophosphamide. After the patient developed recurrent infections, laboratory findings disclosed panhypogammaglobulinaemia with B-lymphocyte deficiency. The serum immunoglobulin level returned to the normal range after the prednisolone was tapered off. Because lupus patients are susceptible to infections associated with disease exacerbation or immunosuppressive treatment, recurrent infections might be expected during the disease course without need for further evaluation of the immunodeficiency. However, this reversible, probably drug-induced case of hypogammaglobulinaemia highlights the need for immunoglobulin measurements when immunodeficiency is suspected in lupus patients.     

Reversible hypogammaglobulinaemia

In this report we present four patients with reversible hypogammaglobulinaemia who required immunoglobulin substitution for several years. One patient had documented systemic lupus erythematosus (SLE), the other three patients had primary hypogammaglobulinaemia without known cause. Whereas the cessation of azathioprine therapy may have contributed to the recovery in the patient with SLE, the restoration of the immunoglobulin production in the other three patients occurred spontaneously. All four patients were IgA deficient when the hypogammaglobulinaemia was first detected and remained so after IgM and IgG production had recovered. Two of the three patients who also had anti-IgA antibodies started to produce anti-IgA again after stopping the immunoglobulin substitution. We conclude that recovery of hypogammaglobulinaemia is possible but rare. When recovery is suspected, we recommend that immunoglobulin substitution is stopped and the antibody response to vaccination is tested.     

Localization of extrapulmonary tuberculosis in the synovial membrane,skin, and meninges in a patient with systemic lupus erythematosus and IgG deficiency

We report on a 31-year-old female patient with systemic lupus erythematosus (SLE) for 24 years who had a past history of skin tuberculosis (lupus vulgaris), long-term corticosteroid therapy, and IgG deficiency. She presented with monoarthritis and concomitant meningitis from skin tuberculosis after 5 years. The diagnosis of joint and meningeal tuberculosis was defined with clinical symptoms--signs and typical histopathological findings of involved synovium. Clinical improvement was achieved with antituberculous therapy. Cutaneous, articular, and cerebral manifestations of tuberculosis might have been confused with some of the lupus manifestations or lupus activation. It should be kept in mind that tuberculosis may be encountered in SLE due to the nature of the underlying disease and/or its therapy. It is also worth mentioning that, in this patient, tissues involved with extrapulmonary tuberculosis were the primary areas of involvement with SLE.     

Pemphigus vulgaris and systemic lupus erythematosus in a 46-y-old man.

Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many organs in the body. Skin manifestations are frequent and sometimes vesiculobullous lesions may apper such as in bullous lupus erythematosus. SLE may also be exceptionally associated with other blistering diseases such as pemphigus vulgaris (PV). We describe the case of a male diagnosed as having SLE, who one year later developed PV. Only three cases (all in women) of SLE associated with PV have been reported in the literature. Our case is noteworthy in that this exceptional association developed in a middle-aged male.     

Autoimmune pancreatitis as the initial presentation of systemic lupus erythematosus

Pancreatitis is a relatively rare complication in systemic lupus erythematosus (SLE). Here we present a case of SLE associated with autoimmune pancreatitis. A 37-year old woman was admitted to our hospital because of fever, skin rash, proteinuria and abdominal pain. A diagnosis of SLE was made based on her clinical, laboratory and renal histological findings showing diffuse proliferative lupus nephritis. Elevated serum amylase, typical radiographic findings and selective increase in serum IgG4 all suggested that the patient also had autoimmune pancreatitis. Systemic administration of glucocorticoid successfully induced remission of pancreatitis and nephritis along with the reduction of IgG4. Autoimmune pancreatitis is a newly recognized type of pancreatitis, in which IgG4 immune response is thought to participate pathophysiologically. Although the disease has been observed to develop in patients having various connective tissue diseases, our report is the first to describe IgG4-related autoimmune pancreatitis in a patient with SLE.     

Central nervous system systemic lupus erythematosus mimicking progressive multifocal leucoencephalopathy.

The case is reported of a patient with central nervous system systemic lupus erythematosus (SLE) with features of progressive multifocal leucoencephalopathy (PML) seen clinically and by magnetic resonance imaging. A brain biopsy sample showed microinfarcts. The use of magnetic resonance imaging and IgG synthesis rates in evaluating central nervous system lupus, the co-occurrence of SLE and PML, and the differentiation of these entities by magnetic resonance imaging and by histology are considered.     

Systemic lupus erythematosus arising in a patient with epidermodysplasia verruciformis

Few cases have been published relating systemic lupus erythematosus (SLE) and epidermodysplasia verruciformis (EV). We describe a patient with this association. A 22-year-old female with classical skin lesions of EV since childhood together with systemic lupus erythematosus (SLE) which developed 10 years later. According to the knowledge of the authors, this case is the third report of association of EV and SLE and the first report of a patient with EV who developed SLE several years after initiation of EV.     

IgG therapy in systemic lupus erythematosus

Summary Two paediatric patients with systemic lupus erythematosus were treated with immunoglobulin G (IgG). In the first case treatment resulted in regression of the most acute symptoms and a long remission was achieved. In the second patient, who was treated during the chronic stage of the disease, there was no significant effect on the course of the SLE.Abbreviations ESR Erythrocyte sedimentation rate - T Thoracic vertebra - DNA Deoxyribonucleic acid - HLA HLA system (of histocompatibility loci) - IgG Immunoglobulin G - RES Reticuloendothelial system - SLE Systemic lupus erythematosus - CNS Central nervous system     

Chronic intestinal pseudo-obstruction associated with biliary tract dilatation in a patient with systemic lupus erythematosus

We present the case of a 57-year old female patient diagnosed with systemic lupus erythematosus (SLE) along with glomerulonephritis and chronic intestinal pseudo-obstruction (CIPO). Dilatation of bile and pancreatic ducts not associated with malignant or litiasic obstruction is reported. The combination of bile duct associated with CIPO in a patient with lupus has not been previously reported in the literature and it probably suggests a smooth muscle dysmotility.     

Systemic lupus erythematosus and pemphigus vulgaris: association or coincidence

Few cases have been published relating systemic lupus erythematosus (SLE) and pemphigus vulgaris (PV). We describe a patient with this association. A 35-year old woman who started to develop persistent pain and morning stiffness of proximal inter and metacarpo-phalangeal joints. During the following year, the patient recalled the onset of blisters on both legs, face, arms and thorax, as well as erosions appearing on oral mucous membranes. We observed generalized multiple erosions on her trunk and legs, flaccid bullae located on her right thigh and multiple erosions on oral mucous membranes. A skin biopsy reported PV Direct immunofluorescence on the perilesional skin specimen, showed beehive intercellular IgG deposits in the epidermis (+++), suggesting PV; granular discontinuous IgM and C3 deposits in the dermal-epidermal union (+++), suggesting SLE. Direct immunofluorescence of the healthy unexposed skin specimen, reported granular discontinuous IgG deposits in the dermal-epidermal union and beehive intercellular IgG deposits in the lower levels of the epidermis (+++); granular continuous IgM deposits in the dermal-epidermal union (+++). The results of rheumatic studies were obtained as follows: ANA :3 +, Anti-DNA, Anti-Sm, Anti-Ro and Anti-La :4 + . The definite diagnosis was PVand SLE. Treatment with 50 mg of prednisone daily with good evolution.     

New onset systemic lupus erythematosus with pheochromocytoma

We describe a patient with positive antinuclear and anti-Smith antibodies, proteinuria, and thrombocytopenia suggesting systemic lupus erythematosus (SLE). During hospitalization, the patient developed labile hypertension, tachycardia, and intermittent fever. A computer tomography scan of the abdomen showed an extraadrenal mass, which was confirmed as a pheochromocytoma. After removal of the pheochromocytoma, the patient's symptoms resolved and her serology normalized. Previous case reports describe SLE patients with adrenal pheochromocytomas that presented many years after the diagnosis of lupus. This is a novel case of pheochromocytoma discovered at the onset of SLE, with resolution of SLE manifestations shortly after its removal.     

Drug induced systemic lupus erythematosus due to ophthalmic timolol.

We report a case of systemic lupus erythematosus (SLE) apparently induced by topical use of ophthalmic timolol maleate, a beta adrenergic blocking agent. The patient developed fever, malaise, pleurisy and recurrent sterile pleural effusions while taking no medication other than timolol. Antinuclear antibodies in a homogenous pattern, and markedly elevated histone antibodies (IgG anti-(H2A-H2B)-DNA) were present while antibodies to native DNA were absent. After discontinuation of the timolol, his symptoms improved promptly and the pleural effusions resolved. To our knowledge, this is the first report of timolol induced SLE.     

Lupus anticoagulant,Factor V Leiden,and methylenetetrahydrofolate reductase gene mutation in a lupus patient with cerebral venous thrombosis

We describe the case of a young Lebanese woman with systemic lupus erythematosus (SLE) and a positive lupus anticoagulant (LAC) who developed right internal jugular vein and sigmoid sinus thrombosis. Coagulation studies showed that in addition to the LAC the patient was heterozygous for the factor V (FV) Leiden mutation, and C677T mutation of the methylenetetrahydrofolate reductase gene. The high prevalence of FV Leiden in the eastern Mediterranean region suggests that we should probably screen our SLE patients in this area, especially those with anticardiolipin antibodies and/or LAC who have no history of thrombosis, for this and other thrombophilia markers. The detection of such abnormalities may have major practical consequences for the long-term management of these patients to prevent further thrombotic episodes.Abbreviations APS Antiphospholipid syndrome - CVT Cerebral venous thrombosis - LAC Lupus anticoagulant - DVT Deep vein thrombosis - SLE Systemic lupus erythematosus - SVC Superior vena cava     

Pancreatitis leading to thrombotic thrombocytopenic purpura in systemic lupus erythematosus: a case report and review of literature

Pancreatitis is a well-established but unusual complication of thrombotic thrombocytopenic purpura (TTP). It is also an unusual complication of systemic lupus erythematosus (SLE). However, TTP occurring as a consequence of acute pancreatitis in a patient with SLE has never been reported. We report a 24-year-old African American woman with active systemic lupus (SLE) who developed thrombotic thrombocytopenic purpura (TTP) following an episode of acute pancreatitis. The TTP was manifested by low-grade fever, microangiopathic hemolytic anemia, renal insufficiency, altered mental status, seizures and thrombocytopenia. The patient was initially treated with pulse corticosteroids with inadequate response and subsequently with daily plasmaphresis, leading to full remission. This case represents first report of pancreatitis leading to TTP in a patient with systemic lupus erythematosus.     

Proliferative lupus nephritis in a patient with systemic lupus erythematosus and longstanding secondary amyloid nephropathy

Secondary amyloidosis is an unusual complication of systemic lupus erythematosus (SLE). We report the case of a 60-year-old woman with SLE and secondary amyloidosis who developed class III proliferative lupus nephritis 13 years after the onset of amyloid nephropathy. The patient was treated with mycophenolate mofetil (1.5 g/day) with a significant improvement in proteinuria.     

Anti-DNA antibody synthesis after bone marrow transplantation: implications for IgG subclass restrictivity and pathogenicity of autoantibodies

Two patients developed antinuclear antibodies (ANA) including antibodies directed against double-stranded DNA (dsDNA) after bone marrow transplantation. In one of the patients very high levels of IgM, IgG as well as IgA anti-dsDNA were found during 6 months in the absence of symptoms of systemic lupus erythematosus (SLE). The other patient made IgM anti-dsDNA antibodies for a period of more than 3 years, also in the absence of SLE symptoms. The ANA were restricted to the IgG1 and IgG3 isotypes as is the case in SLE but did not express idiotypes commonly found in this autoimmune disease. The occurrence of these antibodies may reflect a non-antigen induced expansion of the existing donor B-lymphocyte repertoire.     

Long-term membranous glomerulonephritis as the presenting manifestation of systemic lupus erythematosus in a patient with human immunodeficiency virus infection

The coexistence of human immunodeficiency virus (HIV) infection and systemic lupus erythematosus (SLE) is unusual, but the occurrence of SLE after HIV infection is even less common. Both conditions share similar clinical features including constitutional symptoms, facial rash, oral ulcers, alopecia, arthralgias, arthritis, seizures, cytopenias, glomerulonephritis, and antinuclear and antiphospholipid antibodies. This clinical overlap makes the diagnosis of SLE in a patient with pre-existing HIV infection difficult. Furthermore, immune complex glomerulonephritis with features resembling lupus nephritis has been described in HIV-positive patients. We present the case of a 45-year-old Hispanic woman with long-standing HIV infection who developed membranous glomerulonephritis with histological features of lupus nephritis. Five years after onset of renal disease she developed clinically evident SLE.     

Ruptured renal microaneurysms complicated with a retroperitoneal abscess for a patient with systemic lupus erythematosus

Renal artery aneurysm is extremely rare among patients with systemic lupus erythematosus.(SLE). Herein, we report on a 22-year-old male lupus patient who presented with acute abdominal pain, anemia and subsequent hypertension. Abdominal computed tomography revealed a peri-renal hematoma over the right kidney. A renal angiography revealed bilateral renal microaneurysms. The patient subsequently developed a right-side retroperitoneal abscess 4 weeks after hematoma formation and received an emergent laparotomy with drainage. Subsequent culture ofthe abscess-derived fluid revealed the presence of Proteus mirabilis and Escherichia coli. Following appropriate antipyretic and immunosuppressive drugs therapy, the patient recovered successfully. To the best of our knowledge, this is the first report of SLE associated with a retro-peritoneal abscess probably secondary to a ruptured renal microaneurysm.     

Disseminated intravascular coagulation in lupus erythematosus with acute liver damage.

A case of disseminated intravascular coagulation (DIC) in a patient with systemic lupus erythematosus (SLE) with acute liver dysfunction is described. A 37-year-old man with SLE developed acute DIC and marked liver damage after fracture of the right clavicle and pharyngitis. Treatment with high-dose steroids, heparin, antithrombin III, gabexate mesilate, and antibiotics resulted in prompt improvement. The recovery of an SLE patient after acute DIC and marked liver damage is considered very rare. We report here such a case and discuss the previous reports.     

Thrombotic microangiopathy in patients with phosphatidylserine dependent antiprothrombin antibodies and antiphospholipid syndrome

Thrombotic microangiopathy (TMA) is a rare disorder characterized by microvascular thrombosis. TMA has been reported in patients with antiphospholipid antibodies and/or antiphospholipid syndrome but its pathogenesis is not clarified. We present two patients with TMA associated with IgG phosphatidylserine dependent antiprothrombin antibodies (aPS/PT). CASE 1: A 44-year-old Japanese female with systemic lupus erythematosus (SLE) and positive lupus anticoagulant (LA) was started on ticlopidine after having stroke. Four weeks later she developed TMA. IgG/M/A anticardiolipin antibodies (aCL) were negative, but strong positive IgG aPS/PT were detected. CASE 2: A 32-year-old Russian female with SLE was admitted because of hypertension, renal insufficiency and proteinuria at 14 weeks of pregnancy. She developed TMA after surgical abortion. IgG aPS/PT and LA were strongly positive but IgG/M/A aCL were negative. Neither case had von Willebrand factor cleaving protease (ADAMTS-13), suggesting that TMA in those patients was associated with thrombophilia rather than insufficient ADAMTS-13. Both patients were successfully treated with a series of plasma exchange.