An Aggressive Case of Cryoglobulinemia and Membranoproliferative Glomerulonephritis: A Case Report.

This case report describes a 66-year-old female with membranoproliferative glomerulonephritis (MPGN) with pulmonary involvement presumed secondary to Hepatitis C virus (HCV)-associated with mixed cryoglobulinemia. In this condition, pulmonary involvement is uncommon, and aggressive lung involvement can be associated with poor outcomes. Within eight weeks, the patient was hospitalized twice with acute pulmonary presentations and presented at a third hospitalization with dyspnea, chest pain, abdominal pain, and edema. Imaging revealed persistent and historically evolving lung consolidation, as well as a renal biopsy showing MPGN associated with mixed cryoglobulinemia. A lung biopsy revealed inflammation. Bronchoalveolar lavage did not show hemosiderin-laden macrophages and did not grow infectious agents. Serology revealed negative ANCAs and rheumatoid factor positive at 476 IU/ml (upper limit normal 14 IU/ml). Qualitative cryoglobulins were positive at 2 %ppt (reference range: negative %ppt) and Type II mixed cryoglobulinemia with IgM kappa plus polyclonal IgG. The treatment involved steroids and rituximab. The patient's clinical status deteriorated, and she elected to change her resuscitation status to comfort care measures. This case emphasizes that cryoglobulinemia can present with aggressive manifestations on a wide spectrum. Pulmonary manifestations are rare and were evident in this case (although without clear evidence of diffuse alveolar hemorrhage) and led to a complicated disease course and an unfavorable outcome. Overall, this case underscores the complexity of mixed cryoglobulinemia presentations and the challenges of managing severe cases with multi-organ involvement.

Neuro-Behcet's Disease and Its Association With Cerebral Aneurysms and Subarachnoid Hemorrhage: A Case Report.

Behçet's disease is a rare autoimmune condition characterized by systemic vasculitis, an inflammation of blood vessels, with an unknown etiology. It has varied clinical presentations. Herein, we present the case of a 31-year-old male patient with neuro-Behçet disease who presented with subarachnoid hemorrhage and microaneurysms.

A Rare Presentation of Wegener's Granulomatosis With Perinuclear Antineutrophil Cytoplasmic Antibody Positivity.

Wegener's granulomatosis (WG), also termed granulomatosis with polyangiitis, is a vasculitic condition with numerous systemic manifestations. It is mainly a pauci-immune vasculitis which involves small and medium-sized vessels. Herein, we report a case of a 72-year-old female with a background history of type 2 diabetes mellitus and hypertension. She presented to the hospital with recurrent nasal congestion, headache, and intermittent facial pain for two months' duration. At the initial presentation, she was diagnosed with pansinusitis, for which she was treated with antibiotics and was discharged. Two weeks later, she presented with progressively worsening bilateral lower limb numbness and weakness for one week's duration. With serial investigations, she was diagnosed with WG and was treated with intravenous methylprednisolone along with cyclophosphamide, after which she made a significant improvement in her clinical status.

A Young Female Newly Diagnosed With Takayasu's Arteritis Masquerading As Cerebrovascular Stroke.

The condition known as Takayasu's disease or Takayasu's arteritis is a type of vascular inflammation that affects the large and medium arteries. It can lead to a reduction in blood flow to various parts of the body, and it can cause severe complications. Patients with this disease may not have specific symptoms, which can lead to their diagnosis not being confirmed. Takayasu's disease is believed to be a probable cause of stroke in young patients. Although stroke is a common cause of morbidity, it is usually not an initial presentation in Takayasu's disease. In this study, a young female with left-sided hemiparesis was diagnosed with Takayasu's disease after a clinical and angiographic examination.

Eosinophilic Granulomatosis With Polyangiitis Presentation After Nandrolone Withdrawal.

A 41-year-old man was admitted to the Emergency Department with bilateral numbness in lower extremities and left femoral palsy. He also reported constitutional symptoms, vomiting and non-bloody diarrhoea for the last several months. He had a previous history of eosinophilic asthma with erratic follow-up. During admission, eosinophil count was 66% of white blood cells. Sural nerve biopsy revealed vasculitis with eosinophilic infiltration. Further evaluations consisted of colonoscopy and nasal endoscopy that confirmed eosinophil infiltrates on colonic ulcers and nasal polypi. The patient was started on systemic corticosteroids and cyclophosphamide. Among his personal records, he had been taking nandrolone decanoate without medical prescription, and had withdrawn a few years before the first asthma exacerbation.

The Challenges of Diagnosis and Management of Wegener's Granulomatosis with Negative ANCA.

ABSTRAK Penyakit Granulomatosis Wegener merupakan suatu penyakit autoimun yang mengenai dinding pembuluh darah terutama pembuluh darah kecil dan sedang akibat adanya reaksi komplek imun. Sampai saat ini etiologi penyakit Granulomatosis Wegener belum diketahui secara pasti. Diagnosis Granulosis Wegener salah satunya adalah terdeteksinya antibodi cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), akan tetapi pemeriksaan ANCA negatif atau tidak ditemukan pada kasWegener's granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved.us ini sangat jarang terjadi. Artikel ini membahas mengenai kasus laki-laki 33 tahun datang dengan keluhan luka pada kedua tungkai kaki yang sulit sembuh disertai nyeri pada sendi, demam pada malam hari, penurunan berat badan, rambut rontok dan mimisan berulang yang tidak diketahui penyebabnya. Pada pemeriksaan fisik ditemukan saddle nose, terdapat bercak kehitaman dari kedua pangkal paha kanan dan kiri sampai ke punggung kedua kaki.  Pada regio cruris dan dorsum pedis didapatkan adanya ulkus multiple, ukuran bervariasi, irregular. Hasil pemeriksaan laboratorium menunjukkan Hb 8,7 gr/dl, LED 130 mm/jam, Hapusan darah tepi: anemia hipokromik-mikrositik suspek et causa proses kronis bersamaan dengan proses perdarahan. ANA-IF pola speckled 1:320, ANCA tes hasil negatif (-). Hasil pemeriksaan urin rutin makroskopis darah +4, dan mikroskopis ditemukan leukosituria 2-10 LPB, eritrosituria 8-21 LPB. Hasil USG doppler pada regio ekstremitas inferior sinistra menunjukkan kesan: swelling jaringan lunak pedis sinistra dengan arteritis perifer pada area lesi kutis. Hasil pemeriksaan Patologi Anatomi menunjukkan peradangan kronis non spesifik et regio cruris dan pedis. Tatalaksana yang dilakukan debridement luka oleh spesialis bedah, transfusi pack red cell (PRC), metylprednisolon  mg, azathioprine, cefixime. Hasilnya mimisan sudah tidak dirasakan, nyeri sendi berkurang tetapi masih ada, dan demam membaik. Kata Kunci: Granulomatosis wegener, Vaskulitis autoimun, ANCA negatif  ABSTRACT Wegener's granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved. Keywords: Wegener's granulomatosis, Autoimmune vasculitis, negative ANCA.

Deficiency of the CD155-CD96 immune checkpoint controls IL-9 production in giant cell arteritis.

Loss of function of inhibitory immune checkpoints, unleashing pathogenic immune responses, is a potential risk factor for autoimmune disease. Here, we report that patients with the autoimmune vasculitis giant cell arteritis (GCA) have a defective CD155-CD96 immune checkpoint. Macrophages from patients with GCA retain the checkpoint ligand CD155 in the endoplasmic reticulum (ER) and fail to bring it to the cell surface. CD155low antigen-presenting cells induce expansion of CD4+CD96+ T cells, which become tissue invasive, accumulate in the blood vessel wall, and release the effector cytokine interleukin-9 (IL-9). In a humanized mouse model of GCA, recombinant human IL-9 causes vessel wall destruction, whereas anti-IL-9 antibodies efficiently suppress innate and adaptive immunity in the vasculitic lesions. Thus, defective surface translocation of CD155 creates antigen-presenting cells that deviate T cell differentiation toward Th9 lineage commitment and results in the expansion of vasculitogenic effector T cells.

Autoimmune Vasculitis Causing Acute Bilateral Lower Limb Paralysis.

Autoimmune vasculitis is an autoimmune disease that causes various systemic symptoms, such as fever, fatigue, joint pain, and night sweats. Its clinical course depends on the severity of the inflammation, which can cause acute clinical progression of symptoms. Moreover, when the inflammation of the arteries occurs in the deeper parts of the body, a biopsy may be difficult to perform. Here, we report a case of autoimmune vasculitis in an elderly man who visited our hospital with a chief complaint of muscle pain and fever triggered by a rapid paralysis of both lower limbs. Autoimmune vasculitis can cause a variety of systemic symptoms depending on the size of involved arteries, and its clinical course depends on the severity of the inflammation. Prompt diagnosis and simultaneous treatment of symptoms, excluding other likely diseases, prevent the development of severe and long-term complications of autoimmune vasculitis.

Tolosa-Hunt Syndrome and Hemorrhagic Encephalitis Presenting in a Patient after COVID-19 Vaccination Followed by COVID-19 Infection.

The presence of neurological symptoms within the clinical range of COVID-19 disease infection has increased. This paper presents the situation of a 45-year-old man having the medical antecedent diabetes mellitus, who presented to the emergency department with fever, headache, and respiratory symptoms, nine days following vaccination with the Ad26.COV2-S COVID-19 vaccine. The patient tested positive for SARS-CoV-2 based on nasal polymerase chain reaction (RT-PCR). Two weeks after the presentation, he developed Tolosa-Hunt Syndrome, an autoimmune phenomenon, with painful left ophthalmoplegia. Significant improvement was seen in terms of his discomfort; however, ptosis and ocular mobility improved only moderately after treatment with intravenous methylprednisolone, and the patient was discharged on a new insulin regimen. The patient returned after four weeks and the neurological exam results showed significant signs of right hemiparesis, mixed aphasia, incomplete left ophthalmoplegia, severe headache, and agitation; after a few days, the patient experienced a depressed level of consciousness and coma. The patient's clinical condition worsened and, unfortunately, he died. MRI brain images revealed multiple ischemic strokes, meningitis, infectious vasculitis, and hemorrhagic encephalitis, which are all serious complications of COVID-19.

Granulomatosis With Polyangiitis Presenting as Complete Hearing Loss.

Granulomatosis with Polyangiitis is a rare autoimmune vasculitis that is classically characterized by effects on the upper respiratory tract, lungs, and kidneys. Delay in diagnosis is often attributed to variable and sequential presentation of symptoms rather than concurrent symptomatology. It is important to recognize the wide range of initial presenting symptoms as early diagnosis and treatment is critical in preventing potentially irreversible damage resulting from delayed diagnosis. We present a case of a 29-year-old male with history of mixed sensorineural-conductive hearing loss presumed to be secondary to chronic otitis media who presented to the emergency department with complaint of hematemesis with a subsequent diagnosis of granulomatosis with polyangiitis.

A Rare Case of Susac's Syndrome Masquerading as Progressive-Relapsing Multiple Sclerosis.

Susac's syndrome (SS) is a rare, autoimmune-mediated vasculitis involving central nervous system (CNS) microvasculature, which typically targets the brain, retina, and cochlea. The disease pathology in these regions produces the characteristic triad of encephalopathy, visual loss, and hearing loss. Unfortunately, less than 20% of cases present as the full triad, often making diagnosis challenging. Diagnosis is also confounded by the similarity in the clinical presentation of multiple sclerosis (MS), with an overlap also seen in laboratory studies and radiographic imaging. In this report, we present a case of SS in a young and previously healthy adult male that was initially mistaken as MS. We review the characteristics of SS and highlight the key differences between the two diseases that can be used by diagnosing physicians. Lastly, we describe the treatment strategies involved in SS compared to MS.

Unusual presentation of CREST retinal vasculitis.

PURPOSE: To report a case of CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) syndrome-induced retinal vasculitis in the setting of ocular inflammation soon after recent micropulse cyclophotocoagulation (mTS-CPC). OBSERVATIONS: Our patient developed CREST associated retinal vasculitis in both eyes (right > left) eight days after receiving mTS-CPC in her left eye. There was initial concern for sympathetic ophthalmia due to the resulting bilateral inflammation. The patient was treated with prednisone with resolution of her symptoms. CONCLUSIONS: This is the first case of CREST retinal vasculitis that appears to be directly triggered by inflammation caused by mTS-CPC.

Elucidating the Intriguing Association Between Systemic Lupus Erythematosus and Cardiovascular Disease.

Systemic lupus erythematosus (SLE) patients have demonstrated a higher risk of developing cardiovascular disease (CVD), resulting in it being one of the leading causes of death in SLE patients. SLE itself acts as a sole risk factor influencing the prevalence and progression of CVD. However, conventional risk factors, such as age, hypertension, smoking, and obesity, play a crucial role as well. Therefore, this systematic review attempts to unravel the association of CVD in SLE patients while evaluating the role of conventional risk factors. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed to search the PubMed database starting from March 2021 systematically. Original studies that evaluated the prevalence and progression of CVD in SLE patients were extracted by two reviewers independently. Quality in Prognostic Studies (QUIPS) tool was used to assess the risk of bias. Most studies have a moderate to low risk of bias. Among 3,653 studies identified by our search, 10 studies were included in the review. Strong epidemiologic evidence of SLE patients having an increased relative risk of CVD compared to controls was found. Traditional CVD risk factors, such as age, hypertension, obesity, and smoking, influence the prevalence of CVD among SLE patients. Several SLE-specific factors such disease activity, duration, and certain medications also acted as influencing factors. However, the relative risk of CVD was still higher in SLE patients after adjustment of certain risk factors. One study found that the odds of having a Coronary Artery Calcification (CAC) score greater than zero in women with SLE aged less than or equal to 45 years was 12.6 times higher than women in the Coronary Artery Risk Development in Young Adults (CARDIA) cohort (95% CI 5.2 to 30.7) (participants of CARDIA cohort acted as control). This finding was made after age, hypertension, total cholesterol levels, and aspirin use were adjusted, and the study was restricted to women. Although conventional risk factors increase CVD prevalence, SLE itself also dramatically increases the prevalence of CVD. Therefore, we recommend that SLE should be treated as a "CVD risk equivalent." SLE patients should be managed more extensively with greater emphasis given to cardiac health for better clinical outcomes.

Unique Presentation of Microscopic Polyangiitis: Hearing and Vision Loss, Dysphagia, and Renal Dysfunction.

Microscopic polyangiitis (MPA) is an autoimmune small-vessel vasculitis often positive for perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), or anti-myeloperoxidase (MPO), that classically affects the lungs, kidneys, and skin. Several atypical presentations of MPA involving other organs have also been reported in the literature. We report a unique case of a patient who presented with rare presentations of MPA: hearing and vision loss, dysphagia, renal dysfunction. Despite the atypical nature of her symptoms, her p-ANCA serology was positive and kidney biopsy was consistent with MPA. Regardless of the bizarre nature of a patient's symptoms, we highlight the importance of considering MPA as a differential diagnosis in the setting of positive p-ANCA serology.

Takayasu arteritis - epidemiology, pathogenesis, diagnosis and treatment.

Takayasu disease belongs to the group of autoimmune vasculitis which most often affects the aorta and its branches. It is rare, and it mainly affects young women. Recent epidemiologic studies suggest that Takayasu arteritis is being increasingly recognized in Europe. The first symptoms are non-specific and an early diagnosis is difficult and requires clinical awareness and suspicion. Patients with Takayasu arteritis often present increased inflammatory markers, including C-reactive protein and erythrocyte sedimentation rate, but systemic inflammatory response does not always show a positive correlation with inflammatory activity in the vessel wall. Therefore, imaging studies play a principal role in diagnosis and control of the disease. Glucocorticoids remain the most effective and serve as a cornerstone first line treatment. Immunosuppressive drugs play an important role as well, and biological therapy is increasingly being included in the treatment. This article describes the epidemiology, pathophysiology, diagnostics and treatment of this rare disease, so as to alert clinicians because disease left untreated can lead to narrowing and even closure of vital blood vessels. The most common Takayasu arteritis complications include pulmonary thrombosis, aortic regurgitation, congestive heart failure, cerebrovascular events, vision degeneration or blindness, and hearing problems.

A rare case of mitral valve dissection and aortic-left ventricular tunnel associated with possible autoimmune vasculitis.

We report a very rare case of mitral valve dissection and aortic-left ventricular tunnel caused by possible autoimmune vasculitis. We suspected Behcet's disease in this patient. There was no obvious clinical evidence of infective endocarditis. Echocardiography is the diagnostic tool of choice to recognize valvular dysfunction, related pathology, and possible complications. The patient may require immunosuppressive therapy due to the high likelihood of recurrence in the perioperation period.

Testing anti-neutrophil cytoplasmic antibodies (ANCA): analysis of the European EASI survey on the daily practice of the French laboratories.

Anti-neutrophil cytoplasmic antibodies (ANCA) are mainly searched for the diagnosis of autoimmune vasculitis. They may be found also in other conditions with chronic inflammation. Testing ANCA is based on two main technics: indirect immunofluorescence (IFI) and immunochemical technics to identify the antigenic specificity of the autoantibodies. There is heterogeneity among the laboratories' daily practice. An international group called EASI (European autoimmunity standardisation initiative), composed of 15 countries, comprising France, works to harmonize the practices of the biological diagnosis of the autoimmune diseases. It elaborated a survey consisting of 54 questions related to the analytic parameters of the technics, the algorithms for their use and their biological interpretation; and submitted it to European laboratories. We propose an analysis of the answers obtained from 36 French laboratories specialized in autoimmunity. We compare them to the ones obtained from the other countries and discussed them according to the international recommendations. The analysis reveals a predominant use of IFI as a first step with variable strategies for the identification of the antigenic specificity of the autoantibodies. Overall, the practices are chiefly conformed to the recommendations for the diagnosis of vasculitis, but they are less consensual when the ANCA are performed in other clinical situations.

Periodic Acid-Schiff Staining Parallels the Immunoreactivity Seen By Direct Immunofluorescence in Autoimmune Skin Diseases.

BACKGROUND: In many countries and laboratories, techniques such as direct immunofluorescence (DIF) are not available for the diagnosis of skin diseases. Thus, these laboratories are limited in the full diagnoses of autoimmune skin diseases, vasculitis, and rheumatologic diseases. In our experience with these diseases and the patient's skin biopsies, we have noted a positive correlation between periodic acid-Schiff (PAS) staining and immunofluorescence patterns; however, these were just empiric observations. In the current study, we aim to confirm these observations, given the concept that the majority of autoantibodies are glycoproteins and should thus be recognized by PAS staining. AIMS: To compare direct immunofluorescent and PAS staining, in multiple autoimmune diseases that are known to exhibit specific direct immunofluorescent patterns. MATERIALS AND METHODS: We studied multiple autoimmune skin diseases: Five cases of bullous pemphigoid, five cases of pemphigus vulgaris, ten cases of cutaneous lupus, ten cases of autoimmune vasculitis, ten cases of lichen planus (LP), and five cases of cutaneous drug reactions (including one case of erythema multiforme). In addition, we utilized 45 normal skin control specimens from plastic surgery reductions. RESULTS: We found a 98% positive correlation between DIF and PAS staining patterns over all the disease samples. CONCLUSION: We recommend that laboratories without access to DIF always perform PAS staining in addition to hematoxylin and eosin (H&E) staining, for a review of the reactivity pattern.

Toe Amputation After Minor Surgery in a Patient with Behçet's Disease: A Case Report.

Behçet's disease is a systemic autoimmune vasculitis. Although various clinical findings can be observed depending on the pathologic features caused by the blood vessels involved, the classic triad of the disease includes oral aphthae, genital ulcers, and uveitis. Although complications involving the aorta or the vena cava inferior can prove fatal, thrombophlebitis in the superficial veins of the lower extremities are more commonly observed. Some patients can remain asymptomatic for a long period after the diagnosis. In patients with positive pathergy test findings, trauma can trigger the inflammatory cascade. This case report presents a patient with vasculitis that occurred subsequent to minor surgery and led to amputation of the great toe in a female patient with a 14-year old history of Behçet's disease.

Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches.

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. The disease predominantly affects small-calibre blood vessels and is associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). Microscopic polyangiitis was considered to be a disease entity by Savage et al. in 1985. Microscopic polyangiitis has a reported low incidence and a slight male predominance. The aetiology of MPA remains unknown. There is, however, increased evidence that MPA is an autoimmune disease in which ANCAs, particularly those reacting with MPO, are pathogenic. MPA belongs to the systemic vasculitides, indicating that multiple organs can be affected. The major organs involved in MPA are the kidneys and the lungs. As expected for an illness that affects multiple organ systems, patients with MPA can present with a myriad of different symptoms. Ear, nose and throat (ENT) manifestations are not considered to be clinical symptoms of MPA, but in the majority of populations described, ENT involvement was found in surprisingly high percentages. MPA is part of the ANCA-associated vasculitides, which are characterized by necrotizing vasculitis of small vessels. Diagnosis is mainly established by clinical manifestations, computed tomography (TC), ANCA antibody detection and renal and pulmonary biopsy. The introduction of aggressive immunosuppressive treatment has substantially improved the prognosis. The standardized therapeutic regimen is based on cyclophosphamide and corticosteroids. Using this regimen, remission can be achieved in most of the patients. Rituximab may represent an important alternative to cyclophosphamide for patients who may not respond adequately to antimetabolite therapies.