"Benign" hypergammaglobulinemic purpura is not benign in pregnancy.

A woman presented at 25 weeks gestation in her first pregnancy with severe preeclampsia and an intrauterine death. It later emerged that she had Waldenstrom's benign hypergammaglobulinemic purpura. We discuss the implications of this diagnosis in pregnancy and explore possible management options during subsequent pregnancies.

[A case of disseminated nontuberculous mycobacteriosis during purpura associated with hypergammaglobulinemia and hepatitis type C].

A 74-year-old woman with hepatitis due to hepatitis C virus followed up using oral predonisolone (3 mg/day) for two years because of hypergammaglobulinemia-associated purpura reported fever and lumbago in February 2005. Upon admission in June, she was found in chest-computed tomography to have atelectasia in the right middle lung lobe and a nodule with a cavity in the right lower lobe. She tested positive for tuberculous glycolipid antibody. Gallium scintigraphy showed an abnormal accumulation in the lower lumbar vertebra. Magnetic resonance imaging showed abnormal enhancement at L4, L5, and their intervertebral disc. Mycobacterium intracellulare (M. intracellulare) was detected in blood culture, bronchoalveolar lavage, and a biopsy specimen from the intervertebral disc, yielding a diagnosis of disseminated nontuberculous mycobacteriosis (NTM) due to M. intracellulare. She was treated with clarithromycin (CAM), ethambutol (EB), and rifampicin (RFP), but EB and RFP were discontinued due to of the fever they induced. She was then treated with a combination of CAM, levofloxacin, and streptomycin and followed up as an out patient. Based on case reports of disseminated NTM infection in Japan, the prognosis is poor and a protocol must be established for its treatment.

Combination therapy of prednisolone and mizoribine improves cryoglobulinemic vasculitis with purpura and skin ulcers.

Conventional treatment is not standardized for hepatitis C virus-negative cryoglobulinemia, but corticosteroids, immunosuppressive agents, and plasma exchange typically improved the symptoms. Mizoribine is an immunosuppressive agent that was developed in Japan and has been found to inhibit the proliferation of lymphocytes, especially B cells. We have encountered an elder patient who had hepatitis C virus-negative, type II cryoglobulinemic vasculitis with leg purpura and skin ulcers. Her symptoms improved and cryoglobulin disappeared by the combination therapy of prednisolone and mizoribine. We speculate the action mechanism of this therapy is due to immunosuppressive effects including up-regulation of the efficacy of prednisolone by mizoribine.

Juvenile-onset hypergammaglobulinemic purpura and fetal congenital heart block.

Waldenström's hypergammaglobulinemic purpura (HGP) is a rare chronic disorder characterized by recurrent purpura on the legs, a polyclonal increase in serum gamma-globulin, an elevated erythrocyte sedimentation rate and a positive rheumatoid factor. A 30-year-old primigravid woman with 14 years of HGP was found to have fetal bradycardia at 25 weeks' gestation. Laboratory investigations demonstrated positive anti-Ro/SSA and anti-La/SSB antibodies in the maternal serum. Cesarean delivery was performed at 39 weeks, and a 2750-g female infant was born with complete atrioventricular block. Fortunately, the neonatal period has been uneventful without need for pace-making. Maternal HGP exacerbated just after delivery, but resolved within 1 week without treatment. Physicians should be aware of the possible presence of neonatal lupus-related anti-Ro/SSA and anti-La/SSB autoantibodies in patients with HGP. Screening for these autoantibodies is important and could be used as a marker to identify and manage high-risk pregnancies.

Neurocisticercosis. Una enfermedad emergente / Neurocysticercosis. An emergent disease

La neurocisticercosis es una afectación del sistema nervioso central por las larvas de la Taenia solium. Aunque en nuestro país su diagnóstico era excepcional, en los últimos años se ha observado un notable incremento en el número de casos diagnosticados, debido al fenómeno de la inmigración desde países donde la enfermedad es endémica. La forma de presentación más frecuente de la neurocisticercosis es la crisis epiléptica, seguida de la cefalea. Para el diagnóstico de sospecha debemos valorar los datos epidemiológicos y la clínica y confirmarlo mediante los estudios de neuroimagen e inmunológicos. El tratamiento de elección debe ser farmacológico, principalmente con albendazol, y reservar la cirugía para los casos en el que el primero falla. Las medidas higiénico-sanitarias y el tratamiento de los pacientes con teniasis son de suma importancia. La neurocisticercosis ha dejado de ser uno de esos diagnósticos excepcionales y dado el previsible aumento de su incidencia en nuestro medio, los profesionales sanitarios debemos conocer dicha enfermedad e incluirla en niveles más altos de los algoritmos de diagnóstico diferencial (AU)

Púrpura hipergammaglobulinémica asociada con cirrosis por hepatitis crónica por virus C / Hypergammaglobulinemic purpura associated with chronic hepatitis C and cirrhosis

Se presenta un paciente de 60 años, argentino, con antecedentes de púrpura hipergammaglobulinémica, afectando miembros inferiores y abdomen, desde hace 19 años, con vasculitis leucocitoclástica, factor reumatoideo positivo y crioglobulinemia, asociada con cirrosis por hepatitis crónica por virus C. Es posible que pacientes diagnosticados como púrpura crioglobulinémica estén afectados de hipergammaglobulinas séricas y ambas púrpuras representen una misma enfermedad

Púrpura hipergammaglobulinémica asociada con cirrosis por hepatitis crónica por virus C / Hypergammaglobulinemic purpura associated with chronic hepatitis C and cirrhosis

Se presenta un paciente de 60 años, argentino, con antecedentes de púrpura hipergammaglobulinémica, afectando miembros inferiores y abdomen, desde hace 19 años, con vasculitis leucocitoclástica, factor reumatoideo positivo y crioglobulinemia, asociada con cirrosis por hepatitis crónica por virus C. Es posible que pacientes diagnosticados como púrpura crioglobulinémica estén afectados de hipergammaglobulinas séricas y ambas púrpuras representen una misma enfermedad (AU)

Hypergammaglobulinaemic purpura associated with IgG subclass imbalance and recurrent infection.

We describe a child who initially presented with recurrent infections. He subsequently developed hypergammaglobulinemic purpura but responded well to corticosteroids and hydroxychloroquine.

Benign hypergammaglobulinaemic purpura of Waldenström associated with lymphoid interstitial pneumonitis.

A 31-year-old female is described who developed benign hypergammaglobulinaemic purpura and lymphoid interstitial pneumonitis concomitantly. High titre anti-nuclear antibodies were also noted. Several years previously, the patient had developed myasthenia gravis and multiple sclerosis. The present case is an example of multiple medical disorders characterized by immune dysregulation and represents the first reported associated of hypergammaglobulinaemic purpura with lymphoid interstitial pneumonitis.

Primary hypergammaglobulinemic purpura associated with IgG2 deficiency. A case report.

Hypergammaglobulinemic purpura is a rare disease in children. We report a case of a 12 year-old girl with a history of frequent infections. We found the presence of IgG2 deficiency despite polyclonal hypergammaglobulinemia. An IgG subclass determination should be obtained in every child with polyclonal hypergammaglobulinemia and features of immunodeficiency.

Hypergammaglobulinemic purpura in systemic autoimmune rheumatic diseases: predictive value of anti-Ro(SSA) and anti-La(SSB) antibodies and treatment with indomethacin and hydroxychloroquine.

OBJECTIVE: To define the clinical manifestations, autoantibody associations, optimal treatment, and prognosis of hypergammaglobulinemic purpura associated with systemic autoimmune rheumatic diseases. METHODS: Of 303 consecutive patients with systemic autoimmune rheumatic diseases evaluated over 5 years, 17 French Canadian patients with hypergammaglobulinemic purpura with systemic lupus erythematosus (SLE) (n = 12) or another systemic autoimmune rheumatic disease (n = 5) were identified and followed prospectively. Mild secondary Sjörgren's syndrome developed in 9 (53%) patients. RESULTS: Sixteen (94.1%) patients were women. Attacks of hypergammaglobulinemic purpura occurred in the pretibial (76.5%) or perimalleolar (70.5%) areas or the dorsal aspect of the feet (52.9%). Triggering factors included walking, prolonged standing, and alcohol intake. The mean duration of attacks was 6.1 days. Systemic manifestations consistent with a flare of the underlying systemic autoimmune rheumatic diseases accompanied hypergammaglobulinemic purpura attacks in 15 (88%) patients. Arthralgias (n = 13, 86.6%), arthritis (n = 9, 69.2%), and periarthritis were characterstically localized adjacent to the purpura. Anti-Ro antibodies were expressed in all (100%) patients with hypergammaglobulinemic purpura with SLE, but in only 11 (28.9%) of 38 consecutive patients with SLE without hypergammaglobulinemic purpura (P < 0.000001, odds ratio 84, 95% confidence interval 4.6, 1525). The positive predictive values for hypergammaglobulinemic purpura in SLE were: anti-Ro plus anti-La 73%, anti-La 57%, and anti-Ro 52%. The negative predictive value of anti-Ro was 100%. Although 11 (92%) patients with SLE with anti-Ro expressed anti-52 kDa Ro [4 (36.3%) of whom also expressed anti-60 kDa Ro], this frequency was not greater than in anti-Ro positive patients with SLE without hypergammaglobulinemic purpura. The effects of indomethacin or hydroxychloroquine were assessed over 6 months in 8 patients with recurrent incapacitating hypergammaglobulinemic purpura. Complete (n = 4) or partial (n = 4) remission of hypergammaglobulinemic purpura occurred. In 5 additional patients with severe hypergammaglobulinemic purpura, attacks stopped with prednisone 25 to 60 mg daily. The mean duration of hypergammaglobulinemic purpura followup was 5.4 years (range 1-6 years). At last followup, hypergammaglobulinemic purpura had resolved in 11 (64.7%) patients despite persistently abnormal serology. CONCLUSION: In the absence of anti-Ro antibodies, a presumptive diagnosis of hypergammaglobulinemic purpura secondary to SLE should be questioned. Prednisone should be used only in severe hypergammaglobulinemic purpura. Indomethacin and hydroxychloroquine are of value in the treatment of milder hypergammaglobulinemic purpura.

IgG2 deficiency in primary Sjögren's syndrome and hypergammaglobulinemic purpura.

Total IgG and IgG subclasses were studied in 34 patients with primary Sjögren's syndrome and 4 with hypergammaglobulinemic purpura. Total IgG was elevated in 30/34 patients with Sjögren's syndrome. IgG1 increase was responsible for the main part of total IgG increase, contrasting with low levels of IgG2. The difference in IgG1/IgG2 ratio between 38 patients as a group and 40 normal controls was statistically highly significant, but was not seen in all patients. Six patients had markedly low levels of IgG2, but only two had severe repeated respiratory infections. These observations probably reflect selective autoantibody restriction to the IgG1 subclass. We conclude that patients with Sjögren's syndrome may be IgG2 subclass deficient despite elevated levels of total IgG, but also that such deficiency in most instances does not cause a tendency to infections. IgG subclass analysis may be of value to characterize polyclonal IgG increase, since IgG1 subclass predominance often indicates autoimmune disease.

Waldenström hypergammaglobulinemic purpura and pregnancy.

BACKGROUND: Waldenström hypergammaglobulinemic purpura is characterized by hypergammaglobulinemia, recurring purpura, and an elevated erythrocyte sedimentation rate. It is a rare disease and, to our knowledge, there have been no previous reports of its presence during pregnancy. We report a patient with this disease whose pregnancy was complicated by severe fetal growth restriction (FGR) and acute fetal distress. CASE: A 24-year-old primigravid woman with a history of Waldenström hypergammaglobulinemic purpura and renal insufficiency developed FGR at 32 weeks' gestation. Cesarean delivery was performed at 33.5 weeks because of acute fetal distress, and a 1305-g male infant was delivered. Neonatal outcome was successful. No deterioration of the woman's medical condition occurred during or after her pregnancy. CONCLUSION: Successful pregnancy outcome is possible in women with Waldenström hypergammaglobulinemic purpura. In view of the risk of FGR, close monitoring of fetal growth and well-being is recommended in women with this condition.

[Distal tubular renal acidosis associated with hyperglobulinemic purpura and Sjõgren's syndrome]. / Acidosis tubular renal distal asociada a púrpura hipergammaglobulinémica y síndrome de Sjögren.

A 35-year-old female with a diagnosis of Waldenström's benign hypergammaglobulinemic purpura developed bilateral renal stones during the course of her disease. Evaluation disclosed type I distal tubular renal acidosis. In addition, the patient developed Sjögren's syndrome seven years later. The etiology and pathogenesis of renal tubular acidosis secondary to autoimmune disease are reviewed, and the exceptional occurrence of the three conditions in a single patient is commented.

Hypergammaglobulinemic purpura in patients with Sjögren's syndrome: a report of nine cases and a review of the Japanese literature.

Nine patients with hypergammaglobulinemic purpura associated with primary Sjögren's syndrome were presented. All patients were female (mean age 45.6) and showed recurrent purpura on the lower extremities and typical findings of Sjögren's syndrome with high gammaglobulin and IgG levels, rheumatoid factors, anti-SSA/SSB antibodies (5/5) and anti-nuclear antibodies (6/9). Vasculitis was seen in 6 patients, 4 were of the mononuclear cell type and 2 of the neutrophilic cell type. Six patients had intermediate complexes between 7S and 19S shown by serum ultracentrifugation. Immunofluorescent staining showed immunoglobulin deposition along the blood vessel walls of the skin in these patients. These data suggest that hypergammaglobulinemic purpura in Sjögren's syndrome is vasculitis caused by immunologic processes.

Benign hypergammaglobulinemic purpura in a patient with ulcerative colitis and peripheric arthritis.

A 32-year-old patient is described presenting with ulcerative colitis and oligoarthritis who later developed benign hypergammaglobulinemic purpura. This diagnosis was confirmed by clinical investigation, immunological findings as well as by histological and immunofluorescent examination. He was successfully treated with steroids and azathioprin. The relevant literature is reviewed.