Secondary glomerular diseases

typical case reportsmorphology

Doc. MUDr. Zdeňka Vernerová, CSc., MUDr. Martin Havrda

Case 1

Man, 60 years, complaints:

• Edema of the legs.

• History of diabetes mellitus with complications:– Retinopathy– Polyneuropathy

Man, 60 years, physical examination:

• Generalised edema.

• Severe hypertension common.

• Signs of chronic diabetes:– Muscular atrophy– Fatigue, poor physical performance– Folliculitis, mycotic cutaneous infections

Man, 60 years, DIAGNOSIS?

• Diabetic nephropathy.

Man, 60 years, confirmation of the diagnosis?

• Diabetic vascular abnormalities of the retina.

• EMG signs of polyneuropathy.

• Nephrotic syndrome.

• Loss of GFR common.

• Renal biopsy.

Man, 60 years, follow-up:

• Complex approach to treatment:– Tight diabetes control, physical activity, diet– Tight control of hypertension– ACE-inhibitors

• Early detection and early treatment → better prognosis– Microalbuminuric phase– Normal renal function

• Poor prognosis in advanced cases– Vascular complicatiations (Stroke, IHD, diabetic foot)

Case 2

Female, 21 years, complaints:

• 6 months of treatment for migrating arthralgias– Small joints of the hands– Wrists, knees

• Fever up to 38 deg. Celsius

• Polymorphic skin rash– Red appearance of both cheeks– Red skin rash on sun exposed areas

• Dyspepsia, nausea, vomiting

Female, 21 years, physical examination:

• Erythema of the face (butterfly-shaped)

• Erythema of sun-exposed areas

• Edema of some joints

• Unilateral signs of pleuritis (dull percussion, decreased breathing sounds)

• Fleeting pericardial friction rub

• Small aphtous ulcers in the mouth

• Splenomegaly

Female, 21 years, laboratory tests:

• ESR 90/120 mm• CRP 15 mg/l• WBC´s 2,7 ... 90% neutrophils• Hgb 70 g/l• Trc 90• Urea 15 mmol/l, creatinine 180 umol/l• Urinalysis - RBC´s 40/ul• Proteinuria 1,7 g/24h

Female, 21 years, DIAGNOSIS?

• SYSTEMIC LUPUS ERYTHEMATODES

• Lupus nephropathy

Female, 21 years, confirmation of the diagnosis?

• Multisystem involvement

• ARA criteria

• ANA positive, dsDNA positive, ENA positive, Anti-Ro positive, (ACLA)

• ↑ IgG

• ↓ C3, ↓ C4

• Renal biopsy – type I,II,III,IV,V,VI

Classification of lupus nephritis

I. Minimal mesangial LN

II. Mesangial proliferative LN

III. Focal LN

IV. Diffuse LN

V. Membranous LN

VI. Advanced sclerosing LN

aIf  4 of these criteria, well documented, are present at any time in a patient's history, the diagnosis is likely to be SLE. Specificity is  95%: sensitivity is  75%.Note: ECG, electrocardiography; dsDNA, double-stranded DNA; ANA, antinuclear antibodies.

Table 300–2. Classification Criteria for the Diagnosis of SLEa

Malar rash Fixed erythema, flat or raised, over the malar eminences

Discoid rash Erythematous circular raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur

Photosensitivity Exposure to ultraviolet light causes rash

Oral ulcers Includes oral and nasopharyngeal ulcers, observed by physician

Arthritis Nonerosive arthritis of two or more peripheral joints, with tenderness, swelling, or effusion

Serositis Pleuritis or pericarditis documented by ECG or rub or evidence of effusion

Renal disorder Proteinuria >0.5 g/d or   3+, or cellular casts

Neurologic disorder

Seizures or psychosis without other causes

Hematologic disorder

Hemolytic anemia or leukopenia (<4000/   L) or lymphopenia (<1500/   L) or thrombocytopenia (<100,000/   L) in the absence of offending drugs

Immunologic disorder

Anti-dsDNA, anti-Sm, and/or anti-phospholipid

Antinuclear antibodies

An abnormal titer of ANA by immunofluorescence or an equivalent assay at any point in time in the absence of drugs known to induce ANAs

Diagnostic criteria of SLEAmerican Rheumatology Association

1. Malar rash2. Discoid rash3. Photosensitivity4. Oral ulcers5. Arthritis6. Serositis7. Renal disorder8. Neurologic disorder9. Haematologic disorder10. Immunologic disorder (Anti-dsDNA, AntiSm, ACLA)11. Antinuclear antibodies

Female, 21 years, follow-up:

• Treatment depends on clinical and laboratory assessment and on histologic classification of the disease

• Prolipherative GN– Immune-supressive treatment

• Other forms– Antimalarials, steroids

• Prognosis– Variable, good with systematic treatment– Long (life-long) treatment

Case 3

Female, 65 years, complaints:

• Strong back pain

• Edema of the legs

• Fatigue

• Weight loss

Female, 65 years, physical examination:

• Pale skin

• Tender percussion on vertebral bodies

• Edema

Female, 65 years, DIAGNOSIS?

• MULTIPLE MYELOMA with renal involvement.– Amyloidosis– Myeloma kidney– Light chain deposit disease

Female, 65 years, confirmation of the diagnosis?

• X-ray of the bones

• Immunoelectrophoresis of serum

• Bone marrow aspiration (biopsy)

• Renal biopsy

Female, 65 years, follow-up:

• Hematologic treatment of myeloma (steroids, cytotoxic drugs, autologous bone marrow transplantation...).

• Poor prognosis of amyloidosis.

• In other types of involvement, stabilisation or improvement of renal function is possible.

Case 4

Female, 60 years, complaints:

• In cold environment – tingeling and dyscoloration of the fingertips (Raynaud phenomenon).

• Painful cutaneous defects.• Edema of the legs and face.• Arthralgias.• History of hepatitis.

Female, 60 years, physical examination:

• Generalised edema.• Hypertension.• Hepatomegaly, other signs of cirrhosis lacking.• Palpable purpura.• Cutaneous ulcers may appear.

Female, 60 years, DIAGNOSIS?

• Cryoglobulinemia secondary to chronic hepatitis C, renal involvement.

MembranoprolipherativeGN

Type 1

Membranoprolipherative GN

Type 2

Dense deposit disease.

Female, 60 years, confirmation of the diagnosis:

• Glomerular hematuria, red blood cell casts• Nephrotic syndrome• Loss of GFR may appear• Cryoglobulins in serum• ↓ C3, ↓ C4• AntiHCV positive, HCV RNA positive.• Renal biopsy – usually membranoprolipherative

GN.

Female, 60 years, follow-up:

• Treatment of primary disease (hepatitidy)– Interferone α

• Good prognosis, if hepatitis may be controlled– Symptomatic and supportive treatment– Treatment of hypertension