Richards J. Acute post-streptococcal glomerulonephritis. W V Med J ; Rodriguez-Iturbe B, Batsford S. Pathogenesis of poststreptococcal glomerulonephritis a century after Clemens von Pirquet. Kidney Int ;
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Language: English Portuguese. Rapidly progressive glomerulonephritis RPGN is a renal disease with an extensive differential diagnosis. This paper reports the case of a year-old female patient diagnosed with Hansen's disease with acute progressive renal impairment after developing lower limb pyoderma.
The association between Hansen's and kidney disease has been well documented, with glomerulonephritis GN ranked as the most common form of renal involvement. Post-infectious glomerulonephritis PIGN in adults has been associated with a number of pathogens occurring in diverse sites.
The patient described in this case report had RPGN and biopsy findings suggestive of PIGN with C3 and IgA detected on immunofluorescence and kidney injury secondary to recent infection by Staphylococcus , a well-documented manifestation of renal impairment in patients with Hansen's disease.
A previously healthy year-old female without known comorbidities was admitted to the General Practice Clinic of Hospital Geral Dr.
She complained of weakness, paresthesia, and a burning sensation in her lower limbs she had been feeling for three years along with macular hyperchromic lesions on the soles of her feet.
The patient went to a dermatologist nine months prior to admission and was diagnosed with contact eczema. She was prescribed topical corticosteroids and a moisturizing agent. One month before hospitalization the patient had pain, hyperemia, and bullous lesions on her right foot, which ruptured spontaneously letting out a serous secretion.
She improved after taking unspecified medication. Five days prior to admission the patient developed oliguria, lower limb edema, and abdominal pain - mainly in the hypogastrium - along with nausea and hyporexia. She went to an Emergency Unit and was found to have a serum creatinine SCr level of Her heart was normal on auscultation while crackles were heard bilaterally on the bases of her lungs.
She had a flaccid distended abdomen on account of fat accumulation and complained of pain on her hypogastrium upon palpation. No evidence of visceromegaly was found. Examination of the upper limbs revealed the interosseous muscles of her right hand were atrophied. Neurological examination showed she had predominantly distal paresis of the lower limbs grade IV on the left and III on the right leg , grade IV paresis of the upper limbs, and anesthesia on the soles of her feet.
Serum protein electrophoresis showed polyclonal increases of alphaglobulin and gamma globulins. Ultrasound examination of the kidneys and urinary tract showed normal-sized kidneys with irregular contours RK: 9. Fat pad biopsy was negative for amyloidosis. Electroneuromyography revealed distal mixed axonal demyelinating sensorimotor polyneuropathy with a predominant axonal component and preferential involvement of the right leg, producing severe impairment of the lower limbs and moderate to mild dysfunction of the upper limbs, as seen in cases of infectious neuropathies including Hansen's disease , uremia, and vasculitis.
The patient was started on hemodialysis three times a week. Neurological assessment showed she had multibacillary Hansen's disease positive for bacilli agglomerates. The patient was prescribed polychemotherapy with rifampicin, dapsone, and clofazimine. The pathology specimen was satisfactory and featured 22 glomeruli and two medium-caliber vessels.
Ten glomeruli had global sclerosis and three had fibro-cellular crescents Figure 2. The other glomeruli had mild mesangial proliferation Figure 3 ; findings such as polymorphonuclear infiltration and subepithelial or mesangial deposits humps were not seen. Mild to moderate interstitial fibrosis Figure 4 , acute tubular necrosis, and benign nephrosclerosis were also described. The patient described in this case report suffered from significant loss of renal function within less than three months and had evidence of glomerular injury, hematuria, and proteinuria, which combined yielded a diagnosis of rapidly progressive glomerulonephritis, later confirmed by its pathological correspondent crescentic glomerulonephritis.
RPGN is caused by three disease groups: 1 Goodpasture syndrome or anti-glomerular basement membrane antibody disease; 2 pauci-immune glomerulonephritis; and 3 immune complex glomerulonephritis. Goodpasture syndrome stems from the presence of antibodies targeting the alpha-3 chain of type IV collagen of the GBM, and may manifest as a lung-kidney syndrome marked by linear deposition of IgG on the GBM confirmed by biopsy.
Pauci-immune glomerulonephritis is characterized by the presence of antineutrophil cytoplasmic antibodies ANCA , with p-ANCA anti-myeloperoxidase occurring more commonly in Churg-Strauss syndrome eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis, while c-ANCA anti-proteinase 3 is seen in Wegener's granulomatosis granulomatosis with polyangiitis. The patient was diagnosed with Hansen's disease after she was found to be positive for bacilli agglomerates, a trait used to categorize the disease as multibacillary according to the World Health Organization.
Hansen's disease is a chronic granulomatous infection caused by Mycobacterium leprae, a highly infectious pathogen that produces low morbidity. Several urinary alterations have also been described proteinuria in 4. Immunohistochemistry methods have identified granular deposits of IgG and C3, while IgA, IgM, and fibrin in the glomerular mesangium and capillaries have been reported less frequently. Patients with lower limb ulcers and altered sensitivity are more susceptible to secondary infection and, therefore, have a greater chance of developing post-infectious glomerulonephritis.
The treatment of Hansen's disease with polychemotherapy and of lepra reactions with prednisone and thalidomide seems to improve renal function, particularly in patients with erythema nodosum leprosum. In the past, most of the cases of post-infectious glomerulonephritis PIGN were seen in children after skin or respiratory infection by Streptococcus. Prevalence in adults - the elderly and individuals on immunosuppressants in particular - is well documented and is on the rise. It is more prevalent in males and manifests, as in children, in the form of nephritic syndrome hematuria, proteinuria, hypertension, and renal failure usually weeks after infection sometimes infection along with kidney injury is suspected because the symptoms of infection are milder or less specific in elderly or diabetic individuals.
Contrary to children, who rarely need dialysis, nearly half of the elderly patients are prescribed hemodialysis on account of uremic or congestive symptoms. In lab tests, it is characterized by complement consumption C3 predominantly. Kidney biopsy is required for most adults suspected for PIGN to confirm the diagnosis and rule out glomerulonephritis with similar clinical presentation and for individuals in need of specific immunosuppressant therapy.
PIGN is characterized by neutrophil-rich diffuse proliferative exudative glomerulonephritis. Crescents may form, but are less frequent in cases of pauci-immune GN. Immunofluorescence detects mainly the presence of C3 and possibly IgA in specific cases. Electron-dense subepithelial deposits "humps" may be seen in electron microscopy images if pathology tests are not conclusive after correlation with clinical signs. Satoskar et al.
Treatment is based on the eradication of infection antibiotics and surgery and the management of nephritic syndrome diet, antihypertensive medication, and diuretics. The role of immunosuppressants in PIGN is unclear, and this class of medications is not generally indicated. The patient described in this case report had glomerulonephritis with complement consumption C3 and biopsy findings suggestive of advanced chronic post-infectious GN with C3 and IgA labeling on immunofluorescence, in addition to fibro-cellular crescents, glomerular sclerosis, and interstitial fibrosis.
In this stage of the disease the characteristic subepithelial humps are less visible and involvement is essentially mesangial. The patient had signs suggestive of skin infection on her right foot before the onset of the renal symptoms associated with IgA labeling on immunofluorescence. Therefore, she may have had PIGN by Staphylococcus , a well-documented manifestation of renal disease in patients with Hansen's disease. The patient is still on dialysis and is currently weaning from glucocorticoids.
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Correspondence to: Yuri de Deus Mont'alverne Parente. E-mail: moc. Received Mar 3; Accepted Jun Copyright notice. This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Open in a separate window. Figure 1. Hyperchromic lesions with altered sensitivity more evident on the soles of the feet. Table 1 Lab Tests on Admission. Figure 2. Light microscopy image of a kidney biopsy fragment - periodic acid silver methenamine stain x magnification - showing a glomerulus with a fibro-cellular crescent. Figure 3.
Light microscopy image of a kidney biopsy fragment - hematoxylin and eosin stain x magnification - showing a glomerulus with mesangial proliferation. Figure 4. Light microscopy image of a kidney biopsy fragment - Masson's trichrome stain x magnification - showing mild interstitial fibrosis. Figure 5. Immunofluorescence of kidney biopsy fragment showing strong labeling for C3 x magnification.
Figure 6. Immunofluorescence of kidney biopsy fragment showing strong labeling for IgA x magnification. Case Records of the Massachusetts General Hospital. Case N Engl J Med. Na overlapping etiology of rapidly progressive glomerulonephritis. Am J Kidney Dis. Moroni G, Ponticelli C. Rapidly progressive crescentic glomerulonephritis: Early treatment is a must.
Autoimmun Rev. Jennette JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int. Arthritis Rheumatol. Diagn Tratamento. Leprosy nephropathy: a review of clinical and histopathological features.
Glomerulonefrite rapidamente progressiva
Numerose malattie possono provocare una glomerulonefrite rapidamente progressiva. Esse si possono suddividere in tre gruppi:. Al microscopio elettronico si osservano spesso depositi immuni tra l'epitelio e la membrana basale, che in tutti i casi appare colpita da distruzioni focali. All' immunofluorescenza si possono trovare aspetti diversi a seconda dell'eziologia: nei casi postinfettivi si osservano depositi immuni granulari, nelle forme da anticorpi anti-MBG sindrome di Goodpasture i depositi sono lineari, mentre nei casi idiopatici o associati a vasculite i depositi sono spesso assenti. A questa si associa inevitabilmente un rapido incremento della creatinina sierica, che indica una grave compromissione nella funzione di filtrazione del rene.