Granulomatosis with Polyangitis and Lung involvment (Wegener’s Disease)

Shankavi Mohanraj

Medical Officer, Kauvery Hospital, Tennur

 

Background

Granulomatosis with polyangiitis (GPA) is one of the Antineutrophil Cytoplasmic Antibody (ANCA) associated small vessel vasculitides involving various organs such as nasal septum, sinuses, upper respiratory tract, lungs and kidneys. It is a rare, multi-systemic, life threatening autoimmune inflammatory condition of unknown etiology. Its clinical signs vary, but its pulmonary manifestations are common. This disease poses major challenges in management. Therefore, early and accurate diagnosis, and aggressive treatment are essential to improve the outcomes for the disease In this article,  I present a case of GPA, with c-ANCA positive, and with lung involvement. Its differential diagnosis and treatment are also discussed.

Case Presentation

A 63-years-aged female was admitted with complaints of fever on and off for fifteen days, along with bilateral shoulder pain for 10 days. History of cold for one week, nausea, fatigueness and decreased food intake for one month and inability to walk due to pain present.

Patient was known to have hypothyroidism but was not on treatment.

On examination, she was conscious, oriented, with mild tachypnea and tachycardia. She was febrile, with 100°F temperature.

Granulomatosis1

Fig. 1

. Purpuric rashes on her lower extremities for 15 days.

Ultrasound evaluation of both shoulders were done: she had painful range of motion around neck and shoulders. The scan reported Bilateral supraspinatus tendinitis with acromioclavicular arthritis.

Echocardiogram:

EF-60%, Mild mitral regurgitation, mild aortic regurgitation, Mild tricuspid regurgitation, Mild PAH, No intracavitary  masses, thrombus, vegetations. Trace pericardial effusion.

On 2nd day of admission patient’s breathlessness aggravated and desaturation was present along with hemoptysis. Patient was supported with O2, and CT chest was taken.

Granulomatosis2

The above CT Chest was taken three days before admission

Granulomatosis3

CT Chest taken after admission showed patchy peribroncho vascular consolidation both lungs, with subpleural ground glass opacities in Right Upper Lobe.

Laboratory tests

WBC 7000
Hb 9.2
HEMATOCRIT 29.5
PLATELET 406000
ESR 120
CRP +++
ANA NEGATIVE
Rhd Factor +++
C-ANCA +++
URINE ALBUMIN +
PUS CELLS 10-15
RBC 20-25

The diagnosis of GPA was made, based on the patient’s history, her physical examination, significant C-ANCA and CT chest lesions.

Patient was treated with intravenous methyl prednisolone (1 g/day) followed by intravenous rituximab (600 mg weekly once), intravenous antibiotics, bronchodilators, analgesics and other supportive drugs.

Treatment was continued with oral prednisolone. The patient’s condition improved. Her arthralgia, arthritis, cough decreased and her hypoxia improved. Her hemoptysis and hematuria decreased. She was discharged on day 17 on oral prednisolone, antibiotic and other supportive drugs.

Discussion

GPA consists of necrotizing granulomatous inflammation of upper and lower respiratory tracts, rapidly progressive glomerulonephritis, necrotizing vasculitis involving lungs and a variety of systemic organs and tissues. Most frequently manifested as parenchymal lung disease resulting in multiple nodules, lesions, masses as seen in our patient. The patient’s progressive multisystem complaints along with elevated ESR, anemia, hemoptysis strongly suggested  the differential diagnosis of bronchogenic carcinoma and pulmonary TB. After excluding them we included GPA, microscopic polyangiitis, churg-strauss syndrome and SLE.

This patient’s clinical course, manifested typical GPA signs and symptoms, her strongly positive C-ANCA were considered diagnostic of GPA. The combination of high-dose steroid and immunosuppressant is the mainstay of treatment. Broad spectrum antibiotics were found to be beneficial in patient with GPA in remission by decreasing relapse by the elimination of the microbe responsible and hence ending the initiation of the harmful stimulus.

Conclusion

GPA should be critically considered as diagnosis when a presentation involves multiple system including joint, kidney, lungs. Life saving treatment is based on prompt and aggressive administration of pulse steroids, high dose of immunosuppressant in repeat intervals and antibiotics.